BRAIN TUMOR TYPES
IN CHILDREN
JASSIN M. JOURIA, MD
DR. JASSIN M. JOURIA IS A MEDICAL DOCTOR, PROFESSOR OF ACADEMIC MEDICINE, AND MEDICAL AUTHOR. HE GRADUATED FROM ROSS UNIVERSITY SCHOOL OF MEDICINE AND HAS COMPLETED HIS CLINICAL CLERKSHIP TRAINING IN VARIOUS TEACHING HOSPITALS THROUGHOUT NEW YORK, INCLUDING KING’S COUNTY HOSPITAL CENTER AND BROOKDALE MEDICAL CENTER, AMONG OTHERS. DR. JOURIA HAS PASSED ALL USMLE MEDICAL BOARD EXAMS, AND HAS SERVED AS A TEST PREP TUTOR AND INSTRUCTOR FOR KAPLAN. HE HAS DEVELOPED SEVERAL MEDICAL COURSES AND CURRICULA FOR A VARIETY OF EDUCATIONAL INSTITUTIONS. DR. JOURIA HAS ALSO SERVED ON MULTIPLE LEVELS IN THE ACADEMIC FIELD INCLUDING FACULTY MEMBER AND DEPARTMENT CHAIR. DR. JOURIA CONTINUES TO SERVES AS A SUBJECT MATTER EXPERT FOR SEVERAL CONTINUING EDUCATION ORGANIZATIONS COVERING MULTIPLE BASIC MEDICAL SCIENCES. HE HAS ALSO DEVELOPED SEVERAL CONTINUING MEDICAL EDUCATION COURSES COVERING VARIOUS TOPICS IN CLINICAL MEDICINE. RECENTLY, DR. JOURIA HAS BEEN CONTRACTED BY THE UNIVERSITY OF MIAMI/JACKSON MEMORIAL HOSPITAL’S DEPARTMENT OF SURGERY TO DEVELOP AN E-MODULE TRAINING SERIES FOR TRAUMA PATIENT MANAGEMENT. DR. JOURIA IS CURRENTLY AUTHORING AN ACADEMIC TEXTBOOK ON HUMAN ANATOMY & PHYSIOLOGY.
Abstract
The field of brain tumor research, diagnosis, and treatment is rapidly evolving. Over 120 types of brain tumors have been identified to date, and that number continues to increase. As the information available about brain tumors expands, so does the ability to target screening and therapies to provide patients with optimal outcomes. It is essential that health clinicians understand brain tumor surgery and treatment options in order to communicate it to their patients and to develop a care plan focused on a positive outcome while respecting the patient's needs and desires. 1 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Policy Statement
This activity has been planned and implemented in accordance with the policies of NurseCe4Less.com and the continuing nursing education requirements of the American Nurses Credentialing Center's Commission on Accreditation for registered nurses. It is the policy of NurseCe4Less.com to ensure objectivity, transparency, and best practice in clinical education for all continuing nursing education (CNE) activities.
Continuing Education Credit Designation
This educational activity is credited for 3.5 hours. Nurses may only claim credit commensurate with the credit awarded for completion of this course activity.
Statement of Learning Need
Health clinicians need to be able to differentiate between malignant and benign tumors and to understand the commonly used classification system identifying tumor types. Once the type of tumor and its prognosis are identified, the important responsibility of explaining treatment options to the patient will help to guide the patient and family members to make informed choices that is right for them.
Course Purpose
To provide health clinicians with knowledge of brain tumor types and surgical interventions in order to educate patients and their families, and to improve collaboration between all members of the health team. 2 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Target Audience
Advanced Practice Registered Nurses and Registered Nurses (Interdisciplinary Health Team Members, including Vocational Nurses and Medical Assistants may obtain a Certificate of Completion)
Course Author & Planning Team Conflict of Interest Disclosures
Jassin M. Jouria, MD, William S. Cook, PhD, Douglas Lawrence, MA, Susan DePasquale, MSN, FPMHNP-BC – all have no disclosures
Acknowledgement of Commercial Support
There is no commercial support for this course.
Please take time to complete a self-assessment of knowledge, on page 4, of sample questions before reading the article.
The opportunity to complete a self-assessment of knowledge learned will be provided at the end of the course.
3 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
1. ______are the most frequent type of solid tumor in children under 15 years of age.
a. Leukemias b. Brain and spinal cord tumors c. Non-malignant peripheral nerve tumors d. Lymphomas
2. Brain and spinal cord tumors
a. are a single, type cancer and are histologically similar. b. have similar patterns of incidence. c. are of several, distinct histological types. d. are related because they have a common etiology.
3. True or False: The meninges are three membranes that completely cover the brain and spinal cord.
a. True b. False
4. ______are the core components of the brain, spinal cord, and peripheral nerves, and the human brain has approximately 100 billion of them.
a. Neurons b. Cranial nerves c. Synapses d. Glial cells
5. The International Classification of Childhood Cancer (ICCC) groups childhood tumors mainly on the basis of
a. age of onset. b. malignant and non-malignant types. c. secondary versus primary cancers. d. morphology (shape and structure of the cancer cells).
4 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Introduction
Brain and spinal cord tumors are the most frequent type of solid tumor in children under 15 years of age and second only to leukemias among all malignancies. In most populations, brain and spinal tumors represent upwards of 20 per cent of all childhood cancers; however, these tumors cannot be considered together as a single entity as they are of several distinct histological types with their own patterns of incidence, and cannot be assumed to have a common etiology.
Anatomy Of The Brain
This section will first review the normal anatomy of the brain before continuing forward with a discussion of the common types of brain tumors and treatment in children. The brain and spinal cord together form the central nervous system (CNS). The CNS controls personality, thoughts, memory, intelligence, speech and understanding, emotions, senses, and basic body functions, as well as how individuals function in their environment. Many factors influence how a child will experience and report symptoms associated with a brain tumor, such as the age and ability of the child to communicate symptoms. Clinicians may have to depend on the observation of parents or teachers to determine how the child diagnosed with a brain tumor may be affected.
Brain Structure
The brain is made up of three main components: the forebrain, midbrain, and hindbrain. The forebrain is comprised of the cerebrum, thalamus, and hypothalamus, which is part of a complex system of nerves and networks in the brain called the limbic system. The
5 nursece4less.com nursece4less.com nursece4less.com nursece4less.com midbrain consists of the tectum and tegmentum. The cerebellum, pons and medulla are in the area of the brain known as the hindbrain.
Cerebrum
The largest area of the brain is the cerebrum, which consists of the left and right cerebral hemispheres. Generally speaking, the right cerebral hemisphere controls the left side of the body and the left cerebral hemisphere controls the right side of the body. Research has shown that the right cerebral hemisphere is responsible for such functions as innovation, intuition, and creativity. The left cerebral hemisphere is associated with analytic thought, logic, and language.1
The cerebrum is comprised of four main lobes: frontal, parietal, temporal, and occipital. Each lobe controls a specific group of activities.2
• Frontal — Movement, intelligence, reasoning, behavior, memory, personality, planning, decision making, judgment, initiative, inhibition, mood.
• Parietal — Intelligence, reasoning, telling right from left, language, reading, and sensation. 6 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
• Temporal — Speech, behavior, memory, hearing, vision, smell, and emotions.
• Occipital — Vision.
Cerebellum
The cerebellum is the second largest area of the brain. It is made up of two hemispheres, or halves, as well as a middle section. The cerebellum is connected to the brain stem.
Corpus Callosum
The corpus callosum is made of nerve fibers, deep in the brain, that connect the two halves of the cerebral hemispheres.
Brain Stem
The brain stem is the bottom-most portion of the brain, connecting the cerebrum with the spinal cord. The midbrain, pons, medulla oblongata, and reticular formation are all part of the brain stem.
Midbrain
The midbrain is the short portion of the brain stem between the pons and the cerebral hemispheres. The top of the midbrain is called the tectum. The third and fourth cranial nerves originate in the midbrain.
Pons
The pons, a part of the brain stem, contains the origins of the fifth, sixth, seventh, and eighth cranial nerves.
7 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Medulla Oblongata
The medulla oblongata, a part of the brain stem, connects the brain with the spinal cord. It contains the origins of the ninth, tenth, eleventh, and twelfth cranial nerves.
Reticular Formation
The reticular formation is the central core of the brain stem. It connects with all parts of the brain and brain stem.
Ventricles
The ventricles (passageways in the brain) are connected cavities; which are the lateral, third, and fourth ventricles. There are two lateral ventricles, one in each cerebral hemisphere. The third ventricle is beneath the corpus callosum and surrounded by the thalamus. The fourth ventricle is an expansion of the central canal of the medulla oblongata. These ventricles contain cerebrospinal fluid (CSF); and, the choroid plexus produces this clear, watery fluid. Cerebrospinal fluid flows through the ventricles and the subarachnoid space as it bathes and cushions the brain and spinal cord.
Cranial Nerves
There are 12 pairs of cranial nerves. The cranial nerves are the 1) olfactory, 2) optic, 3) oculomotor, 4) trochlear, 5) trigeminal, 6) abducens, 7) facial, 8) vestibular cochlear, 9) glossopharyngeal, 10) vagus, 11) accessory, and 12) hypoglossal.
8 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Optic Chiasm
The optic chiasm is the area under the hypothalamus where each of the two optic nerves crosses over to the opposite side, forming an X shape.
Pituitary Gland
The pituitary gland is attached to, and receives messages from, the hypothalamus. The pituitary gland is made up of two lobes — the anterior and the posterior. Several hormones are produced by the pituitary gland, including prolactin, corticotropin, and growth hormone.
Hypothalamus
The hypothalamus makes up part of the wall of the third ventricle and is the base of the optic chiasm.
Pineal Gland
The pineal gland lies below the corpus callosum. It produces the hormone melatonin. Melatonin is believed to control the biological rhythm of the body.
Spinal Cord
The spinal cord is made up of neurons and their extensions (i.e., nerve fibers). It begins in the medulla oblongata and continues through the hollow center of the vertebrae. The spinal cord is covered by meninges. Cerebrospinal fluid flows through the meninges.
9 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Meninges
The meninges are three membranes that completely cover the brain and spinal cord. Cerebrospinal fluid flows in the space between two of the membranes. A tumor called meningioma can originate from the meninges.
Glial Tissue (Neuroglia)
Glia is the supportive tissue of the brain. The cells, which make up this tissue, are called glial cells. The most common glial cells are astrocytes and oligodendrocytes. Ependymal cells are another form of glia. The largest percentage of brain tumors originates from the glia.
Tentorium
The tentorium is a flap of meninges separating the cerebral hemispheres from the structures of the posterior fossa.
Posterior Fossa/Infratentorium
The tentorium separates the posterior fossa from the cerebral hemispheres. The area below the tentorium is called the infratentorium, or the posterior fossa. This area within the skull contains the cerebellum and the brain stem.
Supratentorium
The supratentorium is the area above the tentorium containing the cerebral hemispheres.
10 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Neuron Anatomy
The human body is made up of trillions of cells. Cells of the central nervous system (CNS) are called neurons. Neurons are electrically excitable cells in the CNS that process and transmit information. They communicate with each other via chemical and electrical synapses, in a process known as synaptic transmission. Neurons are the core components of the brain, spinal cord, and peripheral nerves, and the human brain has approximately 100 billion of them.
Neurons are the oldest and longest cells in the body. Unlike many of the other types of cells in the body, an individual has many of the same neurons for his or her whole life. Neurons come in all shapes and sizes. Some of the smallest neurons have cell bodies that are just 4 microns wide. The largest ones have cell bodies that are 100 microns wide. Some of them, such as corticospinal neurons or primary afferent neurons, can be several feet long.1-3
Tumor Types Commonly Found In Children
In the International Classification of Childhood Cancer (ICCC), which groups childhood tumors mainly on the basis of morphology, diagnostic group III, CNS and Miscellaneous Intracranial and Intraspinal Neoplasms, contains six subgroups, namely (a) ependymomas (including choroid plexus tumors), (b) astrocytomas, (c) primitive neuroectodermal tumors (PNETs), (d) other gliomas, (e) other specified neoplasms (including pituitary adenomas, craniopharyngiomas, pineal parenchymal tumors, gangliogliomas, and meningiomas), and (f) unspecified neoplasms. The nonmalignant tumors are concentrated in subgroups (a), (e), and (f). A further ICCC subgroup, Xa, comprises intracranial and intraspinal 11 nursece4less.com nursece4less.com nursece4less.com nursece4less.com germ-cell tumors and also includes a sizeable proportion of non- malignant tumors. Non-malignant tumors of blood vessels and peripheral nerves, including hemangiomas, hemangioblastomas, and schwannomas, are not included in the ICCC.4
Many cancer registries, while generally restricting their coverage to malignant neoplasms, also include benign and unspecified intracranial and intraspinal tumors. Others include only malignant tumors of these sites. Total incidence of malignant CNS tumors falls steadily from age 0–4 years to a minimum at age 15–19 years, before rising steadily through adulthood. In the U.S., it has been estimated that the inclusion of nonmalignant tumors increases the incidence among children under 15 years of age by 22 per cent overall; for age groups 0–4 years and 5–9 years, the increase is 17 per cent, but for age 10– 14 years it is 31 percent. Among the mainly white populations of western industrialized countries, total incidence is nearly always in the range 25–40 per million; the male to female ratio is typically 1.2 to one.
The highest incidence rates are observed in the Nordic countries. Japan and Israeli Jews have comparable incidence rates above 25 per million. In developing countries, brain and spinal tumors are usually outnumbered not only by leukemias but also by lymphomas, and the recorded incidence of brain and spinal tumors is often no more than 15 per million. The true incidence is not necessarily that low, since there is almost certainly considerable cases under diagnosis and under- ascertainment in areas that are not served by neurological facilities. Registries that depend substantially on pathology departments for notification of cases will suffer a further deficit if autopsies are rarely
12 nursece4less.com nursece4less.com nursece4less.com nursece4less.com performed. In the developing countries of Asia, but not in Africa or Latin America, there is a larger excess of boys affected than in industrialized countries, with a male/female ratio of around 1.4 to one.5,6
Comparison of incidence rates within the same country suggests that risk may vary between ethnic groups. In the U.S., incidence among blacks is lower than among whites, most markedly so in early childhood. Incidence among Hispanic children in Los Angeles is slightly lower than among non-Hispanic whites, and this difference is more marked in California as a whole.4
Ependymomas (including choroid plexus tumors) hardly ever have an incidence above four per million. In most reports, they account for 10– 15 per cent of CNS tumors of specified type. In the German Childhood Cancer Registry, 60% of ependymomas of a known site were infratentorial.7
Ependymoma is more than twice as common at age 0–4 years as it is in the next decade of life. The male/female ratio is around 1.2–1.3 to one. Astrocytomas collectively are by far the most common childhood CNS tumors, usually accounting for 40–55 per cent of those of specified type. They cover a wide spectrum of degrees of malignancy, ranging from the often slow-growing pilocytic or juvenile astrocytoma to the highly malignant glioblastoma multiforme, but low-grade tumors predominate. Among astrocytomas of a known site in the German registry, 53 per cent were supratentorial. The highest incidence rates were recorded in predominantly white populations in North America, Europe, and Oceania. In California, incidence among Hispanic children
13 nursece4less.com nursece4less.com nursece4less.com nursece4less.com is about half that among non-Hispanic whites of both sexes and in all three of the five-year age groups. The incidence in Osaka, Japan, is lower, but this registry has a high proportion of tumors of unspecified type, and the relative frequency of astrocytoma out of all specified tumors is similar to that in western populations, suggesting that this may also be true of incidence. Elsewhere in the world, incidence is lower, but the relative frequency as a proportion of all CNS tumors is similar to that in western countries. Incidence is fairly constant throughout childhood, and the male to female ratio is usually 1–1.1 to one.8
Primitive neuroectodermal tumor (PNETs) are the second most frequent subgroup in many populations, typically accounting for 20–30 per cent of all specified CNS tumors. In the German registry, 73 per cent of PNETs were infratentorial, and nearly all of these would have been cerebellar medulloblastomas. The highest incidence rates, around 12 per million, have been recorded in two indigenous Pacific populations, the Hawaiians of Hawaii and the Maori of New Zealand. In mainly white populations, the incidence is commonly five to nine per million, and the similarity of relative frequencies across other registries suggests that there is little international or ethnic variation in underlying risk outside the Pacific region. The incidence is highest at age 1– 4 years, slightly lower among infants and at age 5 – 9 years, but falls to around half its peak by age 10 – 14 years. The male to female ratio is 1.6 – 1.7 to one. Other gliomas tend to have a similar incidence and relative frequency to ependymomas. Most of these tumors were not specified further in the literature, but there were also substantial numbers of oligodendrogliomas.9
14 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
The incidence of other specified tumors (other than germ-cell) is nearly always below three per million. As many of these tumors are nonmalignant, some of the differences in rates between registries must be due to variations in ascertainment of nonmalignant tumors. The most frequent histological type is craniopharyngioma, which has an incidence of one to two per million in children in the USA. This is followed by pineal parenchymal tumors and meningiomas. Childhood germ-cell tumors of any site are most common in eastern Asian populations. The highest incidence of intracranial and intraspinal germ- cell tumors is found in Japan and among Singapore Chinese. The next highest rate is in Denmark, but over half of the cases there are non- malignant. Two rare tumors that have been consistently diagnosed and registered only fairly recently are dysembryoplastic neuroepithelial tumor (DNT) and atypical teratoid rhabdoid tumor (AT/RT).4
The main variations in age-specific incidence during the first 20 years of life have been described, but the contrast with the pattern of incidence at older ages is much greater. Although CNS tumors are outnumbered only by leukemias among children and adolescents in many regions of the world, they are less common than in any other age group. Infratentorial tumors are at least as frequent as supratentorial tumors in children, but they account for only a small minority of CNS neoplasms among adults. Whereas low-grade astrocytomas and PNETs are the most numerous CNS tumors in children, glioblastoma and meningioma are the predominant types in adults.
Compared with most other types of childhood cancer, improvements in survival from CNS tumors have been modest, although some
15 nursece4less.com nursece4less.com nursece4less.com nursece4less.com subtypes, notably astrocytoma, have long had a relatively good prognosis. The following sections highlight and describe in detail the most common types of tumors found in children.
Astrocytomas
Astrocytomas are the most common type of glioma. This type of glioma develops from glial cells called astrocytes, most often in the cerebrum (the large, outer part of the brain), but also in the cerebellum (the lower, back part of the brain). About half of childhood brain tumors are astrocytomas. They are most common in children between the ages of 5 and 8.
They can be slow growing (low grade, Grade I or II) or fast growing (high grade, Grade III or IV). Most astrocytomas in children (80%) are low grade. Sometimes they spread to the spine. There are four primary types of astrocytomas in children, and these are discussed below.10-12
Juvenile Pilocytic Astrocytoma (Grade I)
This slow-growing tumor is the most common brain tumor found in children. Juvenile pilocytic astrocytoma is usually cystic (fluid-filled) and develops in the cerebellum. Surgical removal is often the only treatment necessary.
Fibrillary Astrocytoma (Grade II)
This brain tumor infiltrates into the surrounding normal brain tissue, making surgical removal more difficult. A fibrillary astrocytoma may
16 nursece4less.com nursece4less.com nursece4less.com nursece4less.com cause seizures.
Anaplastic Astrocytoma (Grade III)
This brain tumor is malignant. An anaplastic astrocytoma can produce symptoms such as weakness, unsteady walking and a loss of sensation.
Glioblastoma Multiforme (Grade IV)
This is the most malignant type of astrocytoma. It grows rapidly, increasing pressure in the brain.
Brain Stem Glioma
Brain stem gliomas are tumors found in the brain stem. The brainstem is a very delicate location where many pathways from the brain to the spinal cord travel. Tumors in this location can be very challenging to treat. The majority of the tumors is located in the middle of the brainstem and cannot be surgically removed. A minority of brainstem tumors is more favorably located and can be treated with surgery.
Brain stem gliomas occur almost exclusively in children; the group most often affected is the school-age child. The child usually does not have increased intracranial pressure, but may have problems with double vision, movement of the face or one side of the body, or difficulty with walking and coordination. Symptoms of brain tumors in the brainstem (base of brain) may include:13-15
• seizures
• endocrine problems (diabetes and/or hormone regulation)
• visual changes or double vision
17 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
• headaches
• paralysis of nerves/muscles of the face, or half of the body
• respiratory changes
• increased intracranial pressure (ICP)
• clumsy, uncoordinated walk
• hearing loss
• personality changes
Choroid Plexus Tumors (CPTs)
Choroid plexus tumors (CPTs) are rare tumors that occur most commonly in children under the age of two. About 90% of choroid plexus tumors are benign. These tumors are found in the choroid plexus — the part of the brain within the spaces in the brain called ventricles that makes cerebrospinal fluid, which surrounds and cushions the brain and the spinal cord. As they grow, choroid plexus tumors can cause hydrocephalus, a build-up of cerebrospinal fluid, commonly known as water on the brain. This results in increased pressure on the brain and enlargement of the skull.16
Choroid plexus tumors begin in the brain within areas called ventricles that are filled with cerebrospinal fluid. As CPTs grow, they block the flow of cerebrospinal fluid. This can increase pressure on the brain and cause the skull to get bigger. A CPT may be noncancerous (benign) or cancerous.17
• Choroid plexus papilloma (CPP) and atypical choroid plexus papilloma are both noncancerous and account for the majority of the choroid plexus tumors. These tumors grow slowly and rarely spread to other parts of the brain or spinal cord.
18 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
• Choroid plexus carcinoma (CPC), the cancerous form, grows faster and are much more likely to spread through the cerebrospinal fluid and invade nearby tissue.
Overall, choroid plexus tumors are rare and represent only about 3% of brain tumors in children. They are most common in infants but can occur at any age. Choroid plexus tumors account for 10 to 20 percent of brain tumors diagnosed in children from birth to one year of age. Choroid plexus papillomas account for about 80% of all choroid plexus tumors. Choroid plexus carcinomas account for 10% to 20% of childhood choroid plexus tumors.18
Choroid plexus tumor symptoms vary depending on the tumor’s location and size and the patient’s age. If a child has a CPT, symptoms may include the following:
• Headaches
• Nausea and vomiting (that are worse in the morning and improve as the day goes on)
• Feeling irritable and decreased energy
An infant or toddler with CPT may have trouble feeding or walking. The child’s fontanel (the “soft spot” at the top of the skull) may also become fuller.19
Treatment
This section discusses the treatment options of surgery as well as chemotherapy and radiation for pediatric patients diagnosed with a type of brain tumor discussed above.16-18,20
19 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Surgery is the most common form of CPT treatment except in patients who should not have surgery because of their age or health or because of the tumor’s location. The goal of surgery is to remove as much of the tumor as possible. Most patients with choroid plexus papilloma will need no further treatment after the tumor is removed completely. Other patients may have a shunt (tube) inserted to drain extra fluid.
Children with choroid plexus carcinoma undergo surgery to remove as much of the cancer as possible. These patients often need more treatment with chemotherapy and radiation to kill cancer cells that remain after surgery.
Chemotherapy (“chemo”) uses powerful medication to kill cancer cells or stop them from dividing, growing and making more cancer cells. Chemo may be injected into the bloodstream, so that it can travel throughout the body. Some chemo may be given by mouth. Combination therapy uses more than one type of chemo at a time.
Radiation therapy uses high-energy X-rays or other types of radiation to kill cancer cells or to stop them from growing. External radiation uses machines outside the body to deliver the X-ray dose.
Craniopharyngioma
Craniopharyngiomas are benign tumors that grow near the pituitary gland. Craniopharyngiomas can be solid tumors or cysts. About 10- 15% of pituitary tumors are craniopharyngiomas. They are most common in children, teens, and adults older than 50 years of age. Craniopharyngiomas often press on nerves, blood vessels or parts of the brain around the pituitary gland.21 20 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
It grows in the area of the pituitary gland and the nerves that relay vision from the eyes to the brain (optic nerves), and frequently grows up into the base of the brain. The tumors arise from cells that in the developing embryo had helped to form the normal pituitary gland. For reasons that are not understood, these cells begin to grow on their own, producing masses that often contain both solid tissue and fluid. In children, portions of the tumor frequently have calcium deposits. The fluid (cystic) portions of the tumors can reach very large size, and occasionally extend into both sides of the brain.22
Craniopharyngiomas often press on nerves, blood vessels or parts of the brain around the pituitary gland. These tumors cause symptoms in three major ways. Some children will initially have difficulties with hormonal functions since these tumors grow in or above the pituitary gland and because the pituitary gland regulates virtually all of the hormones in the body; including growth rate and the functions of the thyroid, adrenal, steroid, and sex glands. Perhaps the most common manifestation of this hormone effect is a fall-off in a normal growth rate due to a lack of growth hormone.23
A second form of clinical symptom is related to increased pressure within the brain. These tumors can grow up into the base of the brain, obstructing the chambers in the brain (ventricles) through which the fluid in the brain (CSF) circulates. This obstruction results in headache, nausea, and vomiting, which are all symptoms of the fluid and pressure building up inside the brain, and is known as a condition called hydrocephalus.24
A third, and unfortunately fairly common presentation of these tumors,
21 nursece4less.com nursece4less.com nursece4less.com nursece4less.com is a loss of vision. Because children rarely complain about slowly developing health problems, and because these tumors are located near the base of the brain where the optic nerves are located, it is possible for these tumors to put gradually increasing pressure on the optic nerves which leads to severe vision loss in one or both eyes in the peripheral or central part of the visual field. Many children will be initially diagnosed when they fail a vision test at school or when it is suddenly noted by their parents that their vision is dramatically reduced. Primary symptoms may include the following:21
• headaches
• mood swings or behavior changes
• confusion
• weight change
• drowsiness or fatigue
• nausea
• changes in vision
There continues to be a great deal of discussion among pediatric neurosurgeons throughout the world regarding the appropriate treatment for these tumors. The two most commonly used treatment options include complete excision of the tumor surgically or partial removal followed by radiation therapy.
There is no question that total removal of these tumors is perhaps the only way of guaranteeing their cure. Yet because of the nature of the tumors themselves, total removal may sometimes result in complications that affect the quality of life of the child following surgery. For example, the frequently intense scarring in the brain next to these tumors may make their removal difficult without injury to the
22 nursece4less.com nursece4less.com nursece4less.com nursece4less.com adjacent brain. Because the brain centers near the tumor typically control not only hormonal function but also the control of appetite and emotions, any of these functions could be adversely affected if these centers are injured during an attempt at total removal of the tumor. In addition, these tumors are often quite adherent to arteries that supply blood to vital areas of the brain. If these arteries are injured during the removal of the tumor, the patient could suffer a stroke resulting in permanent paralysis or other severe neurologic deficit. The bleeding that occurs when large arteries at the base of the brain are torn may be very difficult to stop.25,26
The surgeon must have as much information as is practicable to obtain regarding the tumor's boundaries and be able to use the utmost clinical judgment when removing the tumor in order to avoid or minimize these potential complications. Despite the most careful planning and intraoperative technical expertise, however, inadvertent injuries to these vital structures can still occur.27
Radiation therapy is an important part of treatment strategy in many institutions. Although these tumors are benign, their growth can be frequently slowed or stopped by radiation therapy. Various types of radiation therapy can be employed to treat craniopharyngioma including the delivery of external beam radiation to the tumor volume or the instillation of isotopes such as radioactive phosphorus (P32) or Yttrium directly within cystic portions of the tumor. When these treatments are employed, a skull opening must be made for either the placement of a semi-permanent tube into the cyst or to provide an avenue for the direct injection of the radioactive isotope into the cyst.28,29
23 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Dysembryoplastic Neuroepithelial Tumors
Dysembryoplastic neuroepithelial tumors (DNT) are rare, benign tumors that occur in the tissues covering the brain and spinal cord. These are benign (WHO Grade I) slow growing tumors arising from either cortical or deep grey matter. The vast majority, centered in cortical grey matter, arises from secondary germinal layers and is frequently associated with cortical dysplasia (up to 80% of cases). They characteristically cause intractable partial seizures. These tumors, found in children and teens, can cause seizures. A DNT usually begins in children and teenagers who are 20 years old or younger.30
While each child may experience symptoms differently, and symptoms may vary depending on the size and exact location of the tumor, the most common symptom for DNT is the presence of seizures that are difficult to control with anti-seizure medication. In fact, it’s not uncommon for children to have their first seizure before age 10 and then continue to experience them for several years before the diagnosis is made. Dysembryoplastic neuroepithelial tumors are found in the cerebrum, which is the part of the brain that controls thought, movement and sensation, so the child may experience other symptoms that relate to those functions.
The temporal lobe is the most common location of DNT, but all parts of the CNS containing grey matter are potential locations, which are shown below:
• temporal lobe: over 60% of cases • frontal lobe: 30% of cases • caudate nucleus 24 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
• cerebellum: presentation more commonly with ataxia rather than seizures • pons
These tumors consist of different types of abnormal cells including:31-33
• oligodendrocytes (cells that provide support and nourishment for cells that transmit nerve impulses)
• neurons
• astrocytes (connective tissue cells)
If the child’s clinician suspects that a dysembryoplastic neuroepithelial tumor is present, diagnostic tests may be performed that include:30,34
• Neurological exam – This exam tests a child’s reflexes, muscle strength, eye and mouth movement, coordination and alertness
• Computerized tomography scan (also called a CT or CAT scan) – This is a diagnostic procedure that uses x-rays and computer technology to produce cross-sectional images (often called slices), of the brain and spinal cord. A CT scan will identify low- density dysembryoplastic neuroepithelial tumors.
• Magnetic resonance imaging (MRI) – This is a procedure that uses large magnets, radiofrequencies and a computer to produce detailed images of a child’s organs and other bodily structures. It can help confirm the presence of a DNT and distinguish it from other types of tumors.
• Immunohistochemical and ultrastructural studies - Lab-based tests that can confirm the origin of DNT and help a child’s
25 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
clinician determine the best treatment include immunochemical and ultrastructural studies.
The most common treatment for DNT is to remove the tumor surgically. Because it is a benign tumor, and prognosis is good even if the entire tumor is not removed, radiation and chemotherapy are not used. The outlook for dysembryoplastic neuroepithelial tumors (DNT) is uniformly good regardless of how much of the tumor is removed.35
Ependymoma
Ependymomas are also glial cell tumors. They usually develop in the lining of the ventricles (passageways in the brain) or in the spinal cord. The most common place they are found in children is near the cerebellum. Ependymomas often block the flow of the CSF, bathing the brain and spinal cord, and causes increased intracranial pressure. Ependymomas can be slow growing, compared to other brain tumors, but may recur after treatment is completed.
Recurrence of ependymomas results in a more invasive tumor with more resistance to treatment. About 8-10% of tumors in children are ependymomas, which can also occur in the spine. They are most likely to occur about the age of five. Ependymomas range from low-grade to high-grade tumors.8
Ependymomas are relatively rare tumors, accounting for 2–3% of all primary brain tumors. However, they are the third most common brain tumor and spinal cord tumor in children. About 30% of pediatric ependymomas are diagnosed in children younger than three years of age. The location of ependymomas in adults tends to be different than 26 nursece4less.com nursece4less.com nursece4less.com nursece4less.com the location of ependymomas in children. In adults, 60% of these tumors are found in the spinal cord. In children, 90% of ependymomas are found in the brain, with the majority located in the posterior fossa.36
There are four major types of ependymomas: myxopapillary ependymomas, subependymomas, ependymomas and anaplastic ependymomas. Many pathologists also assign a grade (number) to ependymomas. The grade is based on how much the cells look like normal ependymal cells, although various grading systems exist. While the cells of a grade I tumor look somewhat unusual compared to normal ependymal cells, grade III tumor cells look definitely abnormal.
Most grade I tumors do not recur after complete surgical removal. Grades II and III tumors need additional treatment and have a higher likelihood of recurring. Myxopapillary ependymomas tend to occur in the lower part of the spinal column. Subependymomas usually occur near a ventricle. Both are slow growing, and are considered to be low- grade or grade I tumors. Ependymomas are the most common of the ependymal tumors, and are considered grade II tumors. These tumors are usually located along, within or adjacent to the ventricular system, often in the posterior fossa or in the spinal cord. Anaplastic ependymomas are high-grade tumors (grade III) and tend to be faster growing than low-grade tumors. They most commonly occur in the posterior fossa.8,36-38
Symptoms of an ependymoma are related to the location and size of the tumor. In neonates and infants, enlargement of the head may be one of the first symptoms. Irritability, sleepiness and vomiting may
27 nursece4less.com nursece4less.com nursece4less.com nursece4less.com develop as the tumor grows. In older children and adults, nausea, vomiting and headache are the most common symptoms. These are usually signs of increased pressure or hydrocephalus, which develops if the tumor blocks the drainage of cerebrospinal fluid. Fatigue is a common symptom people experience during the course of the illness. Headaches are a common symptom, and are generally worse in the morning. A tumor near the brainstem may cause one or both eyes to cross, balance problems or trouble walking.39
Neck pain may result from a tumor growing near the brainstem or the upper part of the spinal cord. Seizures, headaches and weakness of one side or one part of the body may occur with a tumor in the cerebral hemispheres. Spinal cord tumors often cause leg or back pain, which can be severe enough to awaken the person from sleep. Tingling sensations, numbness, or weakness in the arms or legs may also occur. Difficulty with bladder or bowel control may be caused by an ependymoma in the lower part of the spine.8
Juvenile Pilocytic Astrocytoma
Juvenile pilocytic astrocytoma (JPA) is a rare childhood brain tumor. In most cases, the tumor is a benign, slow growing tumor that usually does not spread to surrounding brain tissue. Symptoms of a JPA will vary depending upon the size and location of the tumor. Most symptoms result from increased pressure on the brain and include headaches, nausea, vomiting, balance problems and vision abnormalities.40
A JPA develops from certain star-shaped brain cells called astrocytes. Astrocytes and similar cells form tissue that surrounds and protects 28 nursece4less.com nursece4less.com nursece4less.com nursece4less.com other nerve cells found within the brain and spinal cord. Collectively, these cells are known as glial cells and the tissue they form is known as glial tissue. Tumors that arise from glial tissue, including astrocytomas, are collectively referred to as gliomas.41
Astrocytomas are classified according to a grading system developed by the World Health Organization (WHO). Astrocytomas come in four grades based upon how fast the cells are reproducing and the likelihood that they will spread or infiltrate nearby tissue. Grades I or II astrocytomas are nonmalignant and may be referred to as low- grade. JPA are Grade I tumors and, unlike the low-grade astrocytomas of adults, rarely upgrade and become malignant. Grades III and IV astrocytomas are malignant and may be referred to as high-grade astrocytomas. Anaplastic astrocytomas are grade III astrocytomas. Grade IV astrocytomas are known as glioblastoma multiforme.42,43
The exact cause of juvenile pilocytic astrocytomas is unknown. Researchers speculate that genetic and immunologic abnormalities, environmental factors (i.e., exposure to ultraviolet rays, certain chemicals, ionizing radiation), diet, stress, and/or other factors may play contributing roles in causing specific types of cancer. Investigators are conducting ongoing basic research to learn more about the many factors that may result in cancer. Astrocytomas occur with greater frequency with certain genetic disorders including neurofibromatosis type I, Li-Fraumeni syndrome, and tuberous sclerosis. The exact relationship between astrocytomas and these rare genetic disorders is unknown.44
29 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Medulloblastoma
Medulloblastoma is one of the most common brain tumors in children, and the most common malignant brain tumor. It accounts for about 15% of brain tumors in children. Medulloblastomas form in the cerebellum. They are named after the medulla, which connects the bottom of the brain to the top of the spinal cord. Medulloblastomas can spread to the spine or other parts of the body. Medulloblastomas occur primarily in children between the ages of 4 and 9, and affect boys more frequently than girls. Medulloblastomas can metastasize along the spinal cord.45
The exact cause(s) of medulloblastoma are not known. It does not appear to "run in families" or to be directly inherited from the parents. However, medulloblastoma is associated with certain chromosomal abnormalities that probably occur at some point during a child's development.
The most common symptom of this tumor is frequent, severe vomiting, that may especially occur in the morning. Less commonly seen are morning headaches, nausea, confusion, and visual changes, such as double vision. Typically, children with medulloblastoma come to the attention of parents and teachers because of unsteady walking and clumsiness with holding things. All of these symptoms are consistent with a problem in the cerebellar region of the brain. For example, constant vomiting results from excessive pressure in the brain due to tumor blockage of important pathways for cerebrospinal fluid flow.46-48 Please note that the symptoms mentioned here do not necessarily or automatically mean a child has a brain tumor, but
30 nursece4less.com nursece4less.com nursece4less.com nursece4less.com further medical evaluation is required to rule out the possibility of a cerebellar tumor, such as medulloblastoma.
In addition to "T" staging, medulloblastoma staging has been modified by including "M" staging, where the "M" stands for metastasis. These terms describe how far the tumor cells have spread from the original location, if at all. The M stage is determined not only by the surgeon's observations, but also in combination with MRI scans and lumbar cytology, and consists of 5 possible groups as shown below:46
• M0: No evidence of metastasis
• M1: Tumor cells found in cerebrospinal fluid (by lumbar puncture and cytology study)
• M2: Tumor beyond primary site but still in brain
• M3: Tumor deposits ("seeds") in spine area that are easily seen on MRI
• M4: Tumor spread to areas outside the CNS (outside both brain and spine)
Generally, treatment regimens are tailored to treat two groups; those with average risk and those with high-risk disease. Average risk patients are those that are older than 3 years, have no metastatic disease, and have less than 1.5 cc of residual tumor after surgery. If a child is under 3 years old, has metastatic disease, or has residual tumor left in the brain after surgery, he or she will be considered as having high-risk disease. Current trials are underway to further characterize medulloblastomas based on molecular aspects of tumor cells, but this approach remains investigational.49,50
31 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Surgery is typically the first component of therapy. It is very important to perform as complete a surgery as possible, with the goal being removal of all visible tumor, while sparing as much surrounding brain tissue as possible. This is then confirmed with a post-operative MRI scan of the brain to look for any leftover, or residual tumor. Based on what the MRI shows, the surgery is classified as one of the following:51
• Gross total resection - No evidence of any tumor left behind either at time of surgery or on post-surgery MRI
• Near-total resection - More than 90% of original tumor removed by surgery
• Subtotal resection - Anywhere from 51-90% of original tumor removed by surgery
• Partial resection - Anywhere from 10-50% of original tumor removed by surgery
• Any surgery that removed less than 10% of the original tumor is considered a biopsy, or sampling, of the tumor.
After surgery, external radiation to the entire CNS (craniospinal irradiation, or CSI) is recommended to prevent the tumor from recurring in this area, leading to relapse. Even if complete surgery is performed, low-dose radiation to the brain and spine is very important for local control (within the CNS). This is the region that is most at-risk for the tumor returning. At the present time, the role of chemotherapy in medulloblastoma is the standard of care as part of tri-modality therapy, along with surgery and radiation, to help increase long-term disease-free survival. Chemotherapy is standard adjuvant (after surgery) therapy for all patients, regardless of their risk stratification.52,53
32 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Optic Nerve Glioma
An optic nerve glioma (also called an optic pathway glioma) is a slow- growing brain tumor that arises in or around the optic nerve, which connects the eye to the brain. As the tumor progresses, it presses on the optic nerve, causing a child’s vision to worsen. Blindness can occur, but only in about 5 percent of cases. While these are serious tumors, they have a high cure rate.54
Nearly 75 percent of optic nerve gliomas, which may affect one or both eyes, occur in children younger than 10, with most younger than 5 years of age at the time of diagnosis. Optic nerve gliomas account for 5 percent of all childhood brain tumors. Because the optic system is located near the hormone center of the brain, these tumors can affect the body's endocrine functions, such as hormone production, salt and water balance, appetite and sleep.
Optic pathway gliomas (OPGs) are an important subtype of pediatric gliomas. Gliomas are the most common type of primary central nervous system tumor in children, making up approximately 50% of all pediatric brain and CNS tumors with an overall population incidence rate of 3-4 per 100,000. Gliomas that specifically arise from the optic pathway represent approximately 2%-5% of intracranial tumors in children. These tumors principally occur in the first decade of life and the incidence decreases with increasing age. Optic nerve gliomas are found in or around the nerves that send messages from the eyes to the brain. They are frequently found in persons who have neurofibromatosis, a condition a child is born with that makes him/her more likely to develop tumors in the brain.
33 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Persons usually experience loss of vision, as well as hormone problems, since these tumors are usually located at the base of the brain where hormonal control is located and arise from the optic nerve. These are typically difficult to treat due to the surrounding sensitive brain structures.
In patients with NF1, the diagnosis of an OPG is often made on routine screening eye examinations. When OPGs present clinically, the list of potential signs and symptoms is long and variable. Broadly speaking, the list of presenting features can be divided into the four categories: ocular, neurological, endocrine, or other.55-58
Decreased visual acuity Optic atrophy Ocular Papilledema Nystagmus Proptosis
Headache Nausea/vomiting Neurological Seizures Cranial nerve palsy Developmental regression
Precocious puberty Endocrine Short stature Diabetes insipidus
Diencephalic syndrome Other Increasing head circumference
Although there are a number of symptoms associated with this type of tumor, the most common symptoms of brain tumors in the optic nerve may include:
• headaches 34 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
• visual changes or double vision
• clumsy, uncoordinated walk
Most often, optic pathway glioma is treated with chemotherapy – a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce – to shrink the tumor and stabilize or improve vision. If the tumor has affected the child's endocrine system (a network of gland controlling hormones), he or she may also need hormone replacement therapy possibly lifelong.
While surgery is not commonly performed for this type of brain tumor, it can sometimes relieve symptoms and/or improve vision. Expert pediatric neural surgeons have a wealth of experience in determining whether surgery is the right option. Most of the time, chemotherapy can stop the progression of optic pathway gliomas. But if the child's tumor is resistant to chemotherapy, radiation may also be an option.42,59,60
The survival rate for optic pathway gliomas is near 90 percent. Older children and those with neurofibromatosis 1 have better outcomes. In fact, two-thirds of children with NF1 experience spontaneous remission of their optic pathway gliomas. If death does occur, it’s not from the tumor itself, but from the damage on the hypothalamus from the pressure applied by the tumor. A damaged hypothalamus could result in a loss of control of salt levels. High salt levels can cause death. The odds of complete blindness from these tumors are less than 5 percent, but children may experience a smaller field of vision or loss of peripheral vision as a result of the tumor.
35 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
The best predictor of how much vision your child will have is how good his or her vision was when diagnosed. Generally, better original vision correlates with better vision following treatment. Additionally, children who are relatively young at diagnosis tend to preserve their vision better than older kids. In many cases, any damage to the child's vision may be reversed. If the nerve is squeezed and the squeezing stops, it will regain its former shape and start to work again, like a sponge. But if the squeezing goes on for longer than around six months, there’s less of a chance that the nerve will recover and there is a high risk for blindness.61-63
Optic nerve glioma recurrence may take place many years after initial treatment. A glioma usually recurs in the same place as the original tumor, but can also occur in other parts of the brain or spinal cord. Local radiation therapy is the usual treatment. Chemotherapy and radiation therapy are options for patients who have only been treated surgically.
Pineal Tumor
These tumors originate from normal cells in the pineal gland. The pineal gland is located in the center of the brain and is involved in the secretion of specific hormones. Tumor types occurring in the pineal region may or may not involve the pineal gland. True pineal cell tumors, pineocytoma and pineoblastoma, and mixed pineal tumors, are covered in this section.
The pineal gland is located at the rear of the third ventricle, which is one of the fluid-filled cavities of the brain. Pineal tumors come from
36 nursece4less.com nursece4less.com nursece4less.com nursece4less.com the normal cells of the pineal gland. There are three types of pineal tumors:64
• Pineocytoma: Slow-growing, grade II tumor.
• Pineoblastoma: More aggressive, grade IV, malignant tumor. A grade III intermediate form has also been described.
• Mixed Pineal Tumor: Contains a combination of cell types.
Symptoms are most often caused by blockage of the cerebrospinal fluid flow and problems with the eye movement pathways. Headache, nausea and vomiting, and double vision are common.
Pineal region tumors represent less than 1% of all primary brain tumors; however, 3% to 8% of childhood brain tumors occur in this area. These tumors tend to occur in young adults between 20 and 40 years old. About 10%-20% of the tumors, particularly pineoblastoma, have the potential to spread through the cerebrospinal fluid. This usually occurs late in the disease. The tumors, however, rarely spread elsewhere in the body.
Like many tumor types, the exact cause of pineal cell tumors is not known. However, scientists have identified chromosomal abnormalities, which may play a role in the development of these tumors.
Standard treatment for these kinds of tumors is radiation therapy. Radiation of the entire brain and spinal cord is recommended in patients with pineoblastoma. Chemotherapy may also be considered, particularly if the tumor has spread or if it regrows.
37 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Surgery may be possible in some individuals to determine the tumor type and remove part of the tumor. In some cases, placement of a shunt (similar to a drain) is used to relieve pressure caused by buildup of cerebrospinal fluid.64-66
Primitive Neuroectodermal Tumor
Primitive neuroectodermal tumor (PNET) is a name used for tumors, which appear identical under the microscope to medulloblastoma, but occur primarily in the cerebrum. PNET is used by some to refer to tumors such as the pineoblastoma, polar spongioblastoma, medulloblastoma, and medulloepithelioma. Except for medulloblastoma, these are all very rare tumors.
A PNET occurs primarily in the cerebrum, but can spread to other parts of the brain and spine.
A PNET contains underdeveloped brain cells, is highly malignant, and tends to spread throughout the central nervous system. These tumors often contain areas of dead tumor cells (necrosis) and cysts. Fluid surrounding the tumor is not uncommon.
Because they tend to be large tumors, symptoms of increased pressure in the brain and mass effect are often present. Seizures are common.
PNETs most often occur in very young children. Like many tumor types, the exact cause of PNET is not known.
38 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Surgery is the standard initial treatment for these tumors. Because of their large size, tendency to spread, and extensive blood supply, total removal is rarely possible. In children three years and older and in young adults, radiation therapy to the entire brain and spine usually follows surgery. Very young children are usually not treated with chemotherapy until they are older.67-69
Rhabdoid Tumor
Malignant rhabdoid tumor is a rare childhood tumor that commonly starts in the kidneys but also can occur in other soft tissues or in the brain, where it is referred to as atypical teratoid/rhabdoid tumor. Malignant rhabdoid tumor occurs most commonly in infants and toddlers; the average age of diagnosis is 15 months old. There are about 20 to 25 new cases of malignant rhabdoid tumor diagnosed each year in the United States. Cells from a malignant rhabdoid tumor can metastasize to other areas of the body.
There is no clear cause of malignant rhabdoid tumor. Researchers have discovered that a gene called SMARCB1 (this gene also goes by the names INI1, SNF5 and BAF47) is mutated in nearly all rhabdoid tumors, including malignant rhabdoid tumor and atypical teratoid/rhabdoid tumor. Mutations in this gene can also sometimes occur in a patient’s normal, non-tumor cells, increasing their risk of developing additional rhabdoid tumors.
The treatment of malignant rhabdoid tumor involves a combination of therapies including surgery, radiation and chemotherapy. However, because this tumor is rare and aggressive, there is no defined standard of care, and treatment options may be tailored to the child's 39 nursece4less.com nursece4less.com nursece4less.com nursece4less.com situation. The child's doctor and other members of the care team will discuss the options with the patient and his or her family in depth. Prompt medical attention and aggressive therapy are important for the best prognosis. The child’s prognosis and treatment options depend on a number of different factors:
• the extent of the disease
• the size and location of the tumor
• the tumor's characteristics when examined under a microscope
• the presence or absence of metastasis
• the tumor's response to therapy
• child age and overall health
• child's tolerance of specific medications, procedures or therapies
• new developments in treatment
In general, malignant rhabdoid tumors are very aggressive and difficult to treat. Patients often initially respond well to treatment, but relapse either during or shortly after treatment remains a risk. Children with germline mutations to SMARCB1 also have a high risk of developing second tumors. Prompt medical attention and appropriate therapy are important for the best prognosis.37,67,70,71
Spinal Cord Tumors
Spinal cord tumors are benign or malignant growths in or near the spinal cord. They are less common in children than brain tumors and occur primarily in children 10 to 16 years old. Spinal cord tumors may arise from the spinal cord region (primary tumor) or spread to the cord from other organs (metastatic tumor). Metastatic spinal cord tumors,
40 nursece4less.com nursece4less.com nursece4less.com nursece4less.com which are cancerous, are more common in children than primary spinal cord tumors.72
About 90% of primary spinal cord tumors begin in cells next to the spinal cord. These include:
• Meningioma - is a usually benign, slow growing tumor that starts in the meninges — the outer three layers of tissue between the skull and the brain that cover and protect the brain just under the skull.
• Acoustic neuroma (vestibular schwannoma) - is a benign, slow growing tumor of the nerve that connects the ear to the brain (the hearing nerve).
• Neurofibroma - is a benign tumor that arises from nerves, usually those close to the surface of the body such as nerves of the skin or tissue just beneath the skin.
• Ependymoma - is the most common primary intramedullary (within the spinal cord) tumor.
About 10% of primary spinal cord tumors begin in the cells within the spinal cord.4
Summary
Brain and spinal cord tumors have been discussed as the most frequent type of solid tumor in children under 15 years of age, representing approximately 20 per cent of all childhood cancers. These tumors vary and therefore cannot be assumed to have a common etiology. The International Classification of Childhood Cancer (ICCC) groups childhood tumors mainly on the basis of morphology (shape and structure of the cancer cells) and diagnostic groups. There is 41 nursece4less.com nursece4less.com nursece4less.com nursece4less.com expanding research and debate among pediatric neurologists and surgeons worldwide regarding the appropriate treatment for childhood brain tumors. The common treatment options include surgical excision of the tumor, chemotherapy and radiation therapy.
Brain tumors in children can occur in any part of the brain, leading to varied symptoms based on the type of tumor, location and growth. Many factors influence how a child will experience and report symptoms associated with a brain tumor, such as the age and ability of the child to communicate symptoms. Clinicians may have to depend on the observation of parents and teachers to determine how the child diagnosed with a brain tumor may be affected.
Please take time to help NurseCe4Less.com course planners evaluate the nursing knowledge needs met by completing the self-assessment of Knowledge Questions after reading the article, and providing feedback in the online course evaluation.
Completing the study questions is optional and is NOT a course requirement.
42 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
1. ______are the most frequent type of solid tumor in children under 15 years of age.
a. Leukemias b. Brain and spinal cord tumors c. Non-malignant peripheral nerve tumors d. Meningioma
2. Brain and spinal cord tumors
a. are a single, type cancer and are histologically similar. b. have similar patterns of incidence. c. are of several, distinct histological types. d. are related because they have a common etiology.
3. True or False: The meninges are three membranes that completely cover the brain and spinal cord.
a. True b. False
4. ______are the core components of the brain, spinal cord, and peripheral nerves, and the human brain has approximately 100 billion of them.
a. Neurons b. Cranial nerves c. Synapses d. Glial cells
5. The International Classification of Childhood Cancer (ICCC) groups childhood tumors mainly on the basis of
a. age of onset. b. malignant and non-malignant types. c. secondary versus primary cancers. d. morphology (shape and structure of the cancer cells).
43 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
6. The ______is made of nerve fibers, deep in the brain, that connect the two halves of the cerebral hemispheres.
a. medulla oblongata b. corpus callosum c. pons d. tentorium
7. A tumor called ______can originate from the meninges.
a. craniopharyngioma b. medulloblastoma c. ependymoma d. meningioma
8. True or False: The International Classification of Childhood Cancer (ICCC), which groups childhood tumors, includes non-malignant tumors of blood vessels and peripheral nerves because they are so integrated with the brain.
a. True b. False
9. Which of the following statements is/are true about astrocytoma tumors?
a. About half of childhood brain tumors are astrocytomas. b. They are most common in children between the ages of 1 and 4. c. They develop more often in the cerebellum than in the cerebrum. d. All of the above
10. Surgery is the most common treatment for children with
a. choroid plexus tumors (CPTs). b. brain stem gliomas. c. optic nerve glioma. d. All of the above
44 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
11. The largest area of the brain is the ______, which consists of the left and right cerebral hemispheres.
a. medulla oblongata b. cerebellum c. midbrain d. cerebrum
12. ______occur almost exclusively in children, usually of school-age, and the child may experience problems with double vision, movement of the face or one side of the body, or difficulty with walking and coordination.
a. Primitive neuroectodermal tumors (PNETs) b. Juvenile pilocytic astrocytomas c. Brain stem gliomas d. Pineoblastoma tumors
13. Which of the following tumors can cause hydrocephalus, commonly known as “water on the brain,” as they grow?
a. Anaplastic astrocytoma tumors b. Choroid plexus tumors c. Craniopharyngiomas d. Rhabdoid tumors
14. ______tumors are malignant and can cause weakness, unsteady walking and a loss of sensation.
a. Anaplastic astrocytoma b. Dysembryoplastic neuroepithelial c. Craniopharyngiomas d. Choroid plexus tumors
15. Symptoms occurring in a child who has a choroid plexus tumor (CPT) may include which of the following?
a. Headaches Nausea and vomiting b. Nausea and vomiting that are worse in the morning c. Feeling irritable and decreased energy d. All of the above 45 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
16. True or False: In developing countries, brain and spinal tumors are usually outnumbered not only by leukemias but also by lymphomas.
a. True b. False
17. Craniopharyngiomas are benign tumors that grow near the
a. frontal lobe. b. pineal gland. c. pituitary gland. d. optic chiasm.
18. Craniopharyngioma tumors arise from cells in ______that helped to form the normal pituitary gland.
a. the cerebrospinal fluid b. pons c. the developing embryo d. the brain stem
19. The most common symptom for dysembryoplastic neuroepithelial tumors (DNTs) is
a. the presence of seizures. b. unsteady walking. c. blindness. d. loss of nerve sensations.
20. Some children will initially have difficulties with hormonal functions since ______tumors grow in or above the pituitary gland.
a. ependymomas b. craniopharyngioma c. juvenile pilocytic astrocytoma d. anaplastic astrocytoma
46 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
21. Which of the following statements are true about ependymoma tumors in children?
a. They are commonly found near the cerebellum. b. They usually develop in the pineal gland. c. They are neuronal cell tumors. d. All of the above
22. In children, 90% of ependymomas are found in the brain, with the majority located in the
a. corpus callosum. b. posterior fossa. c. reticular formation. d. spinal cord.
23. True or False: A fairly common presentation of craniopharyngioma tumors, is a loss of vision.
a. True b. False
24. Which of the following is NOT one of the four major types of ependymomas?
a. Neuroepithelial b. Myxopapillary c. Subependymomas d. Anaplastic
25. ______ependymomas are high-grade tumors (grade III) and tend to be faster growing than low-grade tumors.
a. Subependymomas b. Myxopapillary c. Anaplastic d. None of the above
47 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
26. The most common treatment for dysembryoplastic neuroepithelial tumors (DNTs) is
a. chemotherapy. b. surgery. c. anticonvulsant medication. d. radiation therapy.
27. Juvenile pilocytic astrocytoma (JPA) is a rare childhood brain tumor that, in most cases,
a. is a benign tumor. b. is a fast-growing tumor. c. spreads to surrounding brain tissue. d. upgrades and becomes malignant.
28. True or False: The most common symptom for dysembryoplastic neuroepithelial tumors (DNTs) is the presence of seizures but fortunately they are easily controlled with anti-seizure medication.
a. True b. False
29. Medulloblastoma is one of the most common brain tumors in children, and it
a. is usually benign. b. affects girls more frequently than boys. c. is directly inherited from a parent or parents. d. is the most common malignant brain tumor.
30. The most common symptom of medulloblastoma is frequent, severe
a. vomiting. b. headaches. c. confusion. d. double vision.
48 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
31. Optic nerve glioma (also called an optic pathway glioma)
a. is a slow-growing brain tumor. b. may cause blindness. c. has a high cure rate. d. All of the above
32. Most often, optic pathway glioma is treated with
a. chemotherapy. b. laser surgery. c. radiation therapy. d. medication.
33. True or False: With dysembryoplastic neuroepithelial tumors (DNTs), if the entire tumor is not removed during surgery, radiation and chemotherapy are used as follow-up treatments.
a. True b. False
34. Primitive neuroectodermal tumor (PNET) is a name used for tumors that appear identical under the microscope to ______, but occur primarily in the cerebrum.
a. anaplastic astrocytoma b. craniopharyngioma c. choroid plexus cells d. medulloblastoma
35. Malignant rhabdoid tumor is a rare childhood tumor that commonly starts in
a. the cerebellum. b. the brain stem. c. the kidneys. d. the hypothalamus.
49 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
36. Symptoms for pineal tumors are most often caused by
a. blockage of the cerebrospinal fluid flow. b. problems with the eye movement pathways. c. Answers a., and b., above d. seizures.
37. True or False: Because malignant rhabdoid tumors are rare and aggressive, there is no defined standard of care, and treatment options may be tailored to the child's situation.
a. True b. False
38. Standard treatment for pineal tumors is
a. chemotherapy. b. placement of a shunt to drain amniotic fluid. c. radiation therapy. d. surgery.
39. The average age of diagnosis for the malignant rhabdoid tumor is
a. 10 to 16 years old. b. 15 months old. c. 8 years old. d. 2 to 4 years old.
40. Spinal cord tumors in children are growths in or near the spinal cord that
a. occur primarily in children 10 to 16 years old. b. are more commonly benign, primary tumors. c. occur primarily in infants. d. are always malignant.
50 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
CORRECT ANSWERS:
1. ______are the most frequent type of solid tumor in children under 15 years of age.
b. Brain and spinal cord tumors
p. 5: “Brain and spinal cord tumors are the most frequent type of solid tumor in children under 15 years of age and second only to leukemias among all malignancies.”
2. Brain and spinal cord tumors
c. are of several, distinct histological types.
p. 5: “Brain and spinal cord tumors … cannot be considered together as a single entity as they are of several distinct histological types, with their own patterns of incidence, and cannot be assumed to have a common etiology.”
3. True or False: The meninges are three membranes that completely cover the brain and spinal cord.
a. True
p. 9: “The meninges are three membranes that completely cover the brain and spinal cord.”
4. ______are the core components of the brain, spinal cord, and peripheral nerves, and the human brain has approximately 100 billion of them.
a. Neurons
p. 10: “Neurons are the core components of the brain, spinal cord, and peripheral nerves, and the human brain has approximately 100 billion of them.”
51 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
5. The International Classification of Childhood Cancer (ICCC) groups childhood tumors mainly on the basis of
d. morphology (shape and structure of the cancer cells).
p. 42: “The International Classification of Childhood Cancer (ICCC) groups childhood tumors mainly on the basis of morphology (shape and structure of the cancer cells) and diagnostic groups.”
6. The ______is made of nerve fibers, deep in the brain, that connect the two halves of the cerebral hemispheres.
b. corpus callosum
p. 7: “The corpus callosum is made of nerve fibers, deep in the brain, that connect the two halves of the cerebral hemispheres.”
7. A tumor called ______can originate from the meninges.
d. meningioma
p. 9: “A tumor called meningioma can originate from the meninges.”
8. True or False: The International Classification of Childhood Cancer (ICCC), which groups childhood tumors, includes non-malignant tumors of blood vessels and peripheral nerves because they are so integrated with the brain.
b. False
p. 11: “Non-malignant tumors of blood vessels and peripheral nerves, including hemangiomas, hemangioblastomas, and schwannomas, are not included in the ICCC.”
52 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
9. Which of the following statements is/are true about astrocytoma tumors?
a. About half of childhood brain tumors are astrocytomas.
pp. 15-16: “About half of childhood brain tumors are astrocytomas.”
10. Surgery is the most common treatment for children with
a. choroid plexus tumors (CPTs).
p. 19: “Surgery is the most common form of CPT treatment except in patients who should not have surgery because of their age or health or because of the tumor’s location.”
11. The largest area of the brain is the ______, which consists of the left and right cerebral hemispheres.
d. cerebrum
p. 6: “The largest area of the brain is the cerebrum, which consists of the left and right cerebral hemispheres.”
12. ______occur almost exclusively in children, usually of school-age, and the child may experience problems with double vision, movement of the face or one side of the body, or difficulty with walking and coordination.
c. Brain stem gliomas
p. 17: “Brain stem gliomas occur almost exclusively in children; the group most often affected is the school-age child.”
53 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
13. Which of the following tumors can cause hydrocephalus, commonly known as “water on the brain,” as they grow?
b. Choroid plexus tumors
p. 18: “As they grow, choroid plexus tumors can cause hydrocephalus, a build-up of cerebrospinal fluid, commonly known as ‘water on the brain.’”
14. ______tumors are malignant and can cause weakness, unsteady walking and a loss of sensation.
a. Anaplastic astrocytoma
p. 16: “This brain tumor is malignant. An anaplastic astrocytoma can produce symptoms such as weakness, unsteady walking and a loss of sensation.”
15. Symptoms occurring in a child who has a choroid plexus tumor (CPT) may include which of the following?
a. Headaches Nausea and vomiting b. Nausea and vomiting that are worse in the morning c. Feeling irritable and decreased energy d. All of the above [correct answer]
p. 19: “CPT symptoms vary depending on the tumor’s location and size and the patient’s age. If a child has a CPT, symptoms may include the following: Headaches; Nausea and vomiting (that are worse in the morning and improve as the day goes on); Feeling irritable and decreased energy”
16. True or False: In developing countries, brain and spinal tumors are usually outnumbered not only by leukemias but also by lymphomas.
a. True
p. 12: “In developing countries, brain and spinal tumors are usually outnumbered not only by leukemias but also by lymphomas, and the recorded incidence of brain and spinal tumors is often no more than 15 per million.”
54 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
17. Craniopharyngiomas are benign tumors that grow near the
c. pituitary gland.
p. 20: “Craniopharyngiomas are benign tumors that grow near the pituitary gland.”
18. Craniopharyngioma tumors arise from cells in ______that helped to form the normal pituitary gland.
c. the developing embryo
p. 20:” Craniopharyngiomas … arise from cells that in the developing embryo had helped to form the normal pituitary gland.”
19. The most common symptom for dysembryoplastic neuroepithelial tumors (DNTs) is
a. the presence of seizures.
p. 24: “While each child may experience symptoms differently, and symptoms may vary depending on the size and exact location of the tumor, the most common symptom for DNT is the presence of seizures that are difficult to control with anti-seizure medication.”
20. Some children will initially have difficulties with hormonal functions since ______tumors grow in or above the pituitary gland.
b. craniopharyngioma
p. 21: “Some children will initially have difficulties with hormonal functions since these tumors grow in or above the pituitary gland….”
55 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
21. Which of the following statements are true about ependymoma tumors in children?
a. They are commonly found near the cerebellum.
p. 26: “Ependymomas are also glial cell tumors. They usually develop in the lining of the ventricles (passageways in the brain) or in the spinal cord. The most common place they are found in children is near the cerebellum.”
22. In children, 90% of ependymomas are found in the brain, with the majority located in the
b. posterior fossa.
p. 26: “In children, 90% of ependymomas are found in the brain, with the majority located in the posterior fossa.”
23. True or False: A fairly common presentation of craniopharyngioma tumors, is a loss of vision.
a. True
p. 21: “Craniopharyngiomas often press on nerves, blood vessels or parts of the brain around the pituitary gland…. A third, and unfortunately fairly common presentation of these tumors, is a loss of vision.”
24. Which of the following is NOT one of the four major types of ependymomas?
a. Neuroepithelial
p. 26: “There are four major types of ependymomas: myxopapillary ependymomas, subependymomas, ependymomas and anaplastic ependymomas.”
56 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
25. ______ependymomas are high-grade tumors (grade III) and tend to be faster growing than low-grade tumors.
c. Anaplastic
p. 27: “Anaplastic ependymomas are high-grade tumors (grade III) and tend to be faster growing than low-grade tumors.”
26. The most common treatment for dysembryoplastic neuroepithelial tumors (DNTs) is
b. surgery.
p. 25: “The most common treatment for DNT is to remove the tumor surgically.”
27. Juvenile pilocytic astrocytoma (JPA) is a rare childhood brain tumor that, in most cases,
a. is a benign tumor.
p. 28: ”Juvenile pilocytic astrocytoma (JPA) is a rare childhood brain tumor. In most cases, the tumor is a benign, slow growing tumor that usually does not spread to surrounding brain tissue.”
28. True or False: The most common symptom for dysembryoplastic neuroepithelial tumors (DNTs) is the presence of seizures but fortunately they are easily controlled with anti-seizure medication.
b. False
p. 24: “While each child may experience symptoms differently, and symptoms may vary depending on the size and exact location of the tumor, the most common symptom for DNT is the presence of seizures that are difficult to control with anti-seizure medication.”
57 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
29. Medulloblastoma is one of the most common brain tumors in children, and it
d. is the most common malignant brain tumor.
p. 29: “Medulloblastoma is one of the most common brain tumors in children, and the most common malignant brain tumor.”
30. The most common symptom of medulloblastoma is frequent, severe
a. vomiting.
p. 30: “The most common symptom of this tumor is frequent, severe vomiting, that may especially occur in the morning.”
31. Optic nerve glioma (also called an optic pathway glioma)
a. is a slow-growing brain tumor. b. may cause blindness. c. has a high cure rate. d. All of the above [correct answer]
p. 32: “An optic nerve glioma (also called an optic pathway glioma) is a slow-growing brain tumor that arises in or around the optic nerve, which connects the eye to the brain. As the tumor progresses, it presses on the optic nerve, causing a child’s vision to worsen. Blindness can occur, but only in about 5 percent of cases. While these are serious tumors, they have a high cure rate.”
32. Most often, optic pathway glioma is treated with
a. chemotherapy.
p. 34: “Most often, optic pathway glioma is treated with chemotherapy – a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce – to shrink the tumor and stabilize or improve vision.”
58 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
33. True or False: With dysembryoplastic neuroepithelial tumors (DNTs), if the entire tumor is not removed during surgery, radiation and chemotherapy are used as follow-up treatments.
b. False
p. 25: “The most common treatment for DNT is to remove the tumor surgically. Because it is a benign tumor, and prognosis is good even if the entire tumor is not removed, radiation and chemotherapy are not used. The outlook for dysembryoplastic neuroepithelial tumors (DNT) is uniformly good, regardless of how much of the tumor is removed.”
34. Primitive neuroectodermal tumor (PNET) is a name used for tumors that appear identical under the microscope to ______, but occur primarily in the cerebrum.
d. medulloblastoma
p. 38: “Primitive neuroectodermal tumor (PNET) is a name used for tumors, which appear identical under the microscope to medulloblastoma, but occur primarily in the cerebrum.”
35. Malignant rhabdoid tumor is a rare childhood tumor that commonly starts in
c. the kidneys.
p. 39: “Malignant rhabdoid tumor is a rare childhood tumor that commonly starts in the kidneys but also can occur in other soft tissues or in the brain, where it is referred to as atypical teratoid/rhabdoid tumor.”
36. Symptoms for pineal tumors are most often caused by
a. blockage of the cerebrospinal fluid flow. b. problems with the eye movement pathways. c. Answers a., and b., above [correct answer] d. seizures.
p. 37: “Symptoms are most often caused by blockage of the cerebrospinal fluid flow and problems with the eye movement 59 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
pathways. Headache, nausea and vomiting, and double vision are common.”
37. True or False: Because malignant rhabdoid tumors are rare and aggressive, there is no defined standard of care, and treatment options may be tailored to the child's situation.
a. True
p. 40: “The treatment of malignant rhabdoid tumor involves a combination of therapies including surgery, radiation and chemotherapy. However, because this tumor is rare and aggressive, there is no defined standard of care, and treatment options may be tailored to the child's situation. The child's doctor and other members of the care team will discuss the options with the patient and his or her family in depth. Prompt medical attention and aggressive therapy are important for the best prognosis.”
38. Standard treatment for pineal tumors is
c. radiation therapy.
p. 37: “Standard treatment for these kinds of tumors [pineal tumors] is radiation therapy.”
39. The average age of diagnosis for the malignant rhabdoid tumor is
b. 15 months old.
p. 39: “Malignant rhabdoid tumor occurs most commonly in infants and toddlers; the average age of diagnosis is 15 months old.”
60 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
40. Spinal cord tumors in children are growths in or near the spinal cord that
a. occur primarily in children 10 to 16 years old.
p. 41: “Spinal cord tumors are benign or malignant growths in or near the spinal cord. They are less common in children than brain tumors and occur primarily in children 10 to 16 years old.”
References Section
The References below include published works and in-text citations of published works that are intended as helpful material for your further reading.
1. Brain Anatomy | Mayfield Clinic [Internet]. [cited 2013 Sep 30]. Available from: http://www.mayfieldclinic.com/PE AnatBrain.htm#.Ukn-c2ATHZw 2. American Association of Neurological Surgeons - Anatomy of the Brain [Internet]. [cited 2013 Sep 30]. Available from: http://www.aans.org/Patient Information/Conditions and Treatments/Anatomy of the Brain.aspx 3. Anatomy of the Brain: Stroke Center at University Hospital, Newark, New Jersey [Internet]. [cited 2013 Sep 30]. Available from: http://www.uhnj.org/stroke/anatomy.htm 4. Brandão LA, Poussaint TY. Pediatric brain tumors. Neuroimaging Clin N Am. 2013;23(3):499–525. 5. Xu J, Margol a, Asgharzadeh S, Erdreich-Epstein a. Pediatric brain tumor cell lines. J Cell Biochem. 2015;116(September 2014):218–24. 6. A. P, M. C, A. M. “Tumor-like” lesions of the pediatric brain. Vol. 1, Journal of Pediatric Neuroradiology. 2012. p. 261–7. 7. Cowherd SM. Tumor staging and grading: a primer. Methods Mol Biol. 2012;823:1–18. 8. Vitanza NA, Partap S. Pediatric Ependymoma. J Child Neurol. 2015; 9. Danielsson A, Nemes S, Tisell M, Lannering B, Nordborg C, Sabel M, et al. MethPed: a DNA methylation classifier tool for the identification of pediatric brain tumor subtypes. Clin Epigenetics. 2015;7(1):62. 10. Shah SS, Dellarole A, Peterson EC, Bregy A, Komotar R, Harvey PD, et al. Long-term psychiatric outcomes in pediatric brain tumor survivors. Vol. 31, Child’s Nervous System. 2015. p. 653–63. 11. Perry A, Wesseling P. Histologic classification of gliomas. In: Handbook of Clinical Neurology. 2016. p. 71–95. 61 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
12. Bonfield CM, Steinbok P. Pediatric cerebellar astrocytoma: a review. Vol. 31, Child’s Nervous System. 2015. p. 1677–85. 13. Minturn JE, Fisher MJ. Gliomas in children. Curr Treat Options Neurol. 2013;15(3):316–27. 14. Charles NA, Holland EC, Gilbertson R, Glass R, Kettenmann H. The brain tumor microenvironment. Glia. 2012;60(3):502–14. 15. Schonberg DL, Lubelski D, Miller TE, Rich JN. Brain tumor stem cells: Molecular characteristics and their impact on therapy. Vol. 39, Molecular Aspects of Medicine. 2013. p. 82–101. 16. Ogiwara H, Dipatri AJ, Alden TD, Bowman RM, Tomita T. Choroid plexus tumors in pediatric patients. Br J Neurosurg. 2012;26(1):32–7. 17. Koh EJ, Wang K-C, Phi JH, Lee JY, Choi JW, Park S-H, et al. Clinical outcome of pediatric choroid plexus tumors: retrospective analysis from a single institute. Childs Nerv Syst. 2014;30(2):217–25. 18. Grimm SA, Chamberlain MC. Choroid Plexus Tumors. In: The Choroid Plexus and Cerebrospinal Fluid: Emerging Roles in CNS Development, Maintenance, and Disease Progression. 2015. p. 65–76. 19. Gozali AE, Britt B, Shane L, Gonzalez I, Gilles F, Mccomb JG, et al. Choroid plexus tumors; management, outcome, and association with the Li-Fraumeni syndrome: The Children’s Hospital Los Angeles (CHLA) experience, 1991-2010. Pediatr Blood Cancer. 2012;58(6):905–9. 20. Jaiswal S, Vij M, Mehrotra A, Kumar B, Nair A, Jaiswal AK, et al. Choroid plexus tumors: A clinico-pathological and neuro-radiological study of 23 cases. Asian J Neurosurg. 2013;8(1):29–35. 21. H. L. M. Craniopharyngioma. Endocr Rev. 2014;35(3):513–43. 22. Rath SR, Lee S, Kotecha RS, Taylor M, Junckerstorff RC, Choong CSY. Childhood craniopharyngioma: 20-year institutional experience in Western Australia. J Paediatr Child Health. 2013;49(5):403–8. 23. Clark AJ, Cage T a., Aranda D, Parsa AT, Auguste KI, Gupta N. Treatment-related morbidity and the management of pediatric craniopharyngioma. J Neurosurg Pediatr. 2012;10(4):293–301. 24. Müller HL. Childhood craniopharyngioma. Pituitary. 2013;16(1):56–67. 25. Trippel M, Nikkhah G. Stereotactic neurosurgical treatment options for craniopharyngioma. Vol. 3, Frontiers in Endocrinology. 2012. 26. Cohen M, Bartels U, Branson H, Kulkarni A V, Hamilton J. Trends in treatment and outcomes of pediatric craniopharyngioma, 1975-2011. Neuro Oncol. 2013;15(6):767–74. 27. Mallucci C, Pizer B, Blair J, Didi M, Doss A, Upadrasta S, et al. 28. Management of craniopharyngioma: the liverpool experience following the introduction of the CCLG guidelines. introducing a new risk assessment grading system. Child’s Nerv Syst. 2012;28(8):1181–92. 29. M.F. I, F. O, S. B, C.F. D. A rare presentation of craniopharyngioma: Delayed puberty. BMJ Case Rep. 2012. 30. Aggarwal A, Fersht N, Brada M. Radiotherapy for craniopharyngioma. Vol. 16, Pituitary. 2013. p. 26–33. 31. Chassoux F, Daumas-Duport C. Dysembryoplastic neuroepithelial 32. tumors: Where are we now’. Epilepsia. 2013;54(SUPPL. 9):129–34. 62 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
33. Daghistani R, Miller E, Kulkarni A V., Widjaja E. Atypical characteristics and behavior of dysembryoplastic neuroepithelial tumors. Neuroradiology. 2013;55(2):217–24. 34. Chassoux F, Rodrigo S, Mellerio C, Landré E, Miquel C, Turak B, et al. Dysembryoplastic neuroepithelial tumors: An MRI-based scheme for epilepsy surgery. Neurology. 2012;79(16):1699–707. 35. Zhang J-G, Hu W-Z, Zhao R-J, Kong L-F. Dysembryoplastic 36. neuroepithelial tumor: a clinical, neuroradiological, and pathological study of 15 cases. J Child Neurol. 2014;29(11):1441–7. 37. Consales A, Striano P, Nozza P, Morana G, Ravegnani M, Piatelli G, et al. Glioneuronal tumors and epilepsy in children: Seizure outcome related to lesionectomy. Minerva Pediatr. 2013;65:609–16. 38. Chassoux F, Landré E, Mellerio C, Laschet J, Devaux B, Daumas- 39. Duport C. Dysembryoplastic neuroepithelial tumors: Epileptogenicity related to histologic subtypes. Clin Neurophysiol. 2013;124(6):1068– 78. 40. Kim JH, Huang Y, Griffin AS, Rajappa P, Greenfield JP. Ependymoma in children: Molecular considerations and therapeutic insights. Clin Transl Oncol. 2013;15(10):759–65. 41. Xu J, Margol A, Asgharzadeh S, Erdreich-Epstein A. Pediatric Brain Tumor Cell Lines. J Cell Biochem. 2014;(September). 42. Mohankumar KM, Currle DS, White E, Boulos N, Dapper J, Eden C, et al. An in vivo screen identifies ependymoma oncogenes and tumor- suppressor genes. Nat Genet. 2015;47(8):878–87. 43. Witt H, Korshunov A, Pfister SM, Milde T. Molecular approaches to 44. ependymoma: the next step(s). Curr Opin Neurol. 2012;25(6):745–50. 45. Hong CS, Lehman NL, Sauvageau E. A pilocytic astrocytoma mimicking a clinoidal meningioma. Case Rep Radiol. 2014;2014:524574. 46. Pereira FO, Lombardi IA, Mello AY, Romero FR, Ducati LG, Gabarra RC, et al. Pilomyxoid astrocytoma of the brainstem. Rare Tumors. 2013;5(2):65–7. 47. Yong EXZ, McKelvie P, Murphy M, Wang YY. Anaplastic pilocytic 48. astrocytoma. J Clin Neurosci. 2014;21(11):1993–6. 49. Chourmouzi D, Papadopoulou E, Konstantinidis M, Syrris V, 50. Kouskouras K, Haritanti A, et al. Manifestations of pilocytic 51. astrocytoma: A pictorial review. Vol. 5, Insights into Imaging. 2014. p. 387–402. 52. Cong YW, Jiang J, Wang XF, Dong XH, Tong ZG, Qi JP. Pilocytic astrocytoma. Chinese J Contemp Neurol Neurosurg. 2015;15(2):148– 52. 53. Millard NE, De Braganca KC. Medulloblastoma. J Child Neurol. 2015;0883073815600866-. 54. Gerber NU, Mynarek M, von Hoff K, Friedrich C, Resch A, Rutkowski S. Recent developments and current concepts in Medulloblastoma. Vol. 40, Cancer Treatment Reviews. 2014. p. 356–65. 55. K. VH, S. R. Medulloblastoma. Curr Treat Options Neurol. 2012;14(4):416–26. 63 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
56. Remke M, Ramaswamy V, Taylor MD. Medulloblastoma molecular dissection: the way toward targeted therapy. Curr Opin Oncol. 2013;25(6):674–81. 57. Northcott PA, Korshunov A, Pfister SM, Taylor MD. The clinical implications of medulloblastoma subgroups. Nat Rev Neurol. 2012;8(6):340–51. 58. Eberhart CG. Three Down and One To Go: Modeling Medulloblastoma Subgroups. Vol. 21, Cancer Cell. 2012. p. 137–8. 59. Dubuc AM, Morrissy AS, Kloosterhof NK, Northcott PA, Yu EPY, Shih D, et al. Subgroup-specific alternative splicing in medulloblastoma. Acta Neuropathol. 2012;123(4):485–99. 60. Gopalakrishnan V, Tao R-H, Dobson T, Brugmann W, Khatua S. Medulloblastoma development: tumor biology informs treatment decisions. CNS Oncol. 2015;4(2):79–89. 61. DeSouza R-M, Jones BRT, Lowis SP, Kurian KM. Pediatric 62. medulloblastoma - update on molecular classification driving targeted therapies. Front Oncol. 2014;4(July):176. 63. Nair AG, Pathak RS, Iyer VR, Gandhi RA. Optic nerve glioma: An 64. update. Vol. 34, International Ophthalmology. 2014. p. 999–1005. 65. Low A. Treatment for glioma ( astrocytoma ). Cancer Res. 2012;1–5. 66. Omuro A. Glioblastoma and Other Malignant Gliomas. Jama. 2013;310(17):1842. 67. Ramos A, Hilario A, Lagares A, Salvador E, Perez-Nuñez A, Sepulveda J. Brainstem Gliomas. Semin Ultrasound, CT MRI. 2013;34(2):104–12. 68. Westphal M, Lamszus K. Circulating biomarkers for gliomas. Nat Rev Neurol. 2015;11(10):556–66. 69. Ahluwalia MS, Chang SM. Medical therapy of gliomas. Vol. 119, Journal of Neuro-Oncology. 2014. p. 503–12. 70. 60. Kao HW, Chiang SW, Chung HW, Tsai FY, Chen CY. Advanced MR imaging of gliomas: An update. Vol. 2013, BioMed Research 71. International. 2013. 72. Marko NF, Weil RJ. The molecular biology of WHO grade II gliomas. Neurosurg Focus. 2013;34(2):E1. Cage TA, Mueller S, Haas-Kogan D, Gupta N. High-Grade Gliomas in Children. Vol. 23, Neurosurgery Clinics of North America. 2012. p. 515– 23. 73. Liu GT, Katowitz J a, Rorke-Adams LB, Fisher MJ. Optic pathway gliomas: neoplasms, not hamartomas. JAMA Ophthalmol. 2013;131(5):646–50. 74. Yang E, Chi SN, Silvera VM. Pineal Region Tumors. In: Handbook of Neuro-Oncology Neuroimaging: Second Edition. 2016. p. 585–602. 75. Morgenstern PF, Souweidane MM. Pineal region tumors: Simultaneous endoscopic third ventriculostomy and tumor biopsy. Vol. 79, World Neurosurgery. 2013. 76. Mattogno PP, Frassanito P, Massimi L, Tamburrini G, Novello M, 77. Lauriola L, et al. Spontaneous Regression of Pineal Lesions: Ghost 64 nursece4less.com nursece4less.com nursece4less.com nursece4less.com
Tumor or Pineal Apoplexy? World Neurosurg. 2016;88:64–9. 78. Gajjar A, Bowers DC, Karajannis MA, Leary S, Witt H, Gottardo NG. Pediatric brain tumors: Innovative genomic information is transforming the diagnostic and clinical landscape. Vol. 33, Journal of Clinical Oncology. 2015. p. 2986–98. 79. Ali S, Joseph NM, Perry A, Barajas RF, Cha S. Apparent diffusion 80. coefficient in glioblastoma with PNET-like components, a GBM variant. J Neurooncol. 2014;119(2):353–60. 81. De Braganca KC, Packer RJ. Treatment options for medulloblastoma and CNS primitive neuroectodermal tumor (PNET). Curr Treat Options Neurol. 2013;15(5):593–606. 82. Reddy CK, Divakar Rao A, Ballal CK, Chakraborti S. Rhabdoid meningioma: Report of two cases. J Clin Diagnostic Res. 2015;9(2):PD05-PD06. 83. Levy JMM, Thorburn A. Modulation of pediatric brain tumor autophagy and chemosensitivity. J Neurooncol. 2012;106(2):281–90. 84. Garber ST, Bollo RJ, Riva-Cambrin JK. Pediatric spinal pilomyxoid astrocytoma. J Neurosurg Pediatr. 2013;12(5):511–6.
The information presented in this course is intended solely for the use of healthcare professionals taking this course, for credit, from NurseCe4Less.com. The information is designed to assist healthcare professionals, including nurses, in addressing issues associated with healthcare.
The information provided in this course is general in nature, and is not designed to address any specific situation. This publication in no way absolves facilities of their responsibility for the appropriate orientation of healthcare professionals. Hospitals or other organizations using this publication as a part of their own orientation processes should review the contents of this publication to ensure accuracy and compliance before using this publication.
Hospitals and facilities that use this publication agree to defend and indemnify, and shall hold NurseCe4Less.com, including its parent(s), subsidiaries, affiliates, officers/directors, and employees from liability resulting from the use of this publication.
The contents of this publication may not be reproduced without written permission from NurseCe4Less.com.
65 nursece4less.com nursece4less.com nursece4less.com nursece4less.com