The CNCF Handbook for Parents of Children with Neuroblastoma
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Ganglioneuroma of the Sacrum
https://doi.org/10.14245/kjs.2017.14.3.106 KJS Print ISSN 1738-2262 On-line ISSN 2093-6729 CASE REPORT Korean J Spine 14(3):106-108, 2017 www.e-kjs.org Ganglioneuroma of the Sacrum Donguk Lee1, Presacral ganglioneuromas are extremely rare benign tumors and fewer than 20 cases have been reported in the literature. Ganglioneuromas are difficult to be differentiated preoperatively Woo Jin Choe1, from tumors such as schwannomas, meningiomas, and neurofibromas with imaging modalities. 2 So Dug Lim The retroperitoneal approach for resection of presacral ganglioneuroma was performed for gross total resection of the tumor. Recurrence and malignant transformation of these tumors is rare. 1 Departments of Neurosurgery and Adjuvant chemotherapy or radiation therapy is not indicated because of their benign nature. 2Pathology, Konkuk University Medical Center, Konkuk University We report a case of a 47-year-old woman with a presacral ganglioneuroma. School of Medicine, Seoul, Korea Key Words: Ganglioneuroma, Presacral, Anterior retroperitoneal approach Corresponding Author: Woo Jin Choe Department of Neurosurgery, Konkuk University Medical Center, displacing the left sacral nerve roots, without 120-1 Neungdong-ro, Gwangjin-gu, INTRODUCTION Seoul 05030, Korea any evidence of bony invasion (Fig. 2). We performed surgery via anterior retrope- Tel: +82-2-2030-7625 Ganglioneuroma is an uncommon benign tu- ritoneal approach and meticulous adhesiolysis Fax: +82-2-2030-7359 mor of neural crest origin which is mainly loca- was necessary because of massive abdominal E-mail: [email protected] lized in the posterior mediastinum, retroperito- adhesion due to the previous gynecologic sur- 1,6) Received: August 16, 2017 neum, and adrenal gland . -
Downloaded 09/30/21 01:49 PM UTC NF2 Patients There Were Often Multiple Meningiomas
Neurosurg Focus 4 (3):Article 1, 1998 Neurofibromatosis type 2 and central neurofibromatosis Leonard I. Malis, M.D. The Mount Sinai School of Medicine, New York City, New York Neurofibromatosis type 2 (NF2) is a rare disease, affecting only approximately 1000 patients in the entire United States. The diagnosis requires the presence of bilateral acoustic neuromas, but many other tumors of the nervous system are also present. It is a very different disease from von Recklinghausen's neurofibromatosis, NF1. The remarkable genetic research in recent years has defined the origin of NF2 to be the lack of a specific suppressor protein, known as Merlin. While we await a method to replace this protein, the neurosurgical care of these patients is a formidable problem. The author reviews his personal series of 41 patients with NF2 treated during the past 30 years and presents 10 cases in detail to demonstrate their considerable range of differences and the treatment problems they have posed. Key Words * neurofibromatosis type 2 * bilateral acoustic neurofibromatosis * central neurofibromatosis * bilateral acoustic neuromas * bilateral vestibular schwannomas * Merlin * schwannomin This review is based on my personal series of 41 surgically treated patients with neurofibromatosis type 2 (NF2). The patients were cared for in the microsurgical era between 1970 and 1995 and received minimum follow-up care of 3 years (median 12 years). All of these people were referred because of bilateral acoustic neuromas. This was a quite young group, with the average age only 20 years, much younger than my patients with solitary acoustic neuromas. The youngest patient was 8 years old and the oldest 40 years old when first referred. -
Diagnostic Value of Preoperative Inflammatory Markers in Patients with Glioma: a Multicenter Cohort Study
CLINICAL ARTICLE J Neurosurg 129:583–592, 2018 Diagnostic value of preoperative inflammatory markers in patients with glioma: a multicenter cohort study *Shi-hao Zheng, MD,1 Jin-lan Huang, PhD,2,4 Ming Chen, MD,3 Bing-long Wang, BS,2 Qi-shui Ou, PhD,2 and Sheng-yue Huang, BS1 1Department of Neurosurgery, Fujian Provincial Hospital; 2Department of Clinical Laboratory, First Affiliated Hospital of Fujian Medical University, Fuzhou, Fujian; 3Department of Neurosurgery, Xin Hua Hospital, Affiliated with Shanghai Jiao Tong University School of Medicine, Shanghai; and 4Laboratory Medicine Center, Nanfang Hospital, Southern Medical University, Guangzhou, Guangdong, People’s Republic of China OBJECTIVE Glioma is the most common form of brain tumor and has high lethality. The authors of this study aimed to elucidate the efficiency of preoperative inflammatory markers, including neutrophil/lymphocyte ratio (NLR), derived NLR (dNLR), platelet/lymphocyte ratio (PLR), lymphocyte/monocyte ratio (LMR), and prognostic nutritional index (PNI), and their paired combinations as tools for the preoperative diagnosis of glioma, with particular interest in its most aggressive form, glioblastoma (GBM). METHODS The medical records of patients newly diagnosed with glioma, acoustic neuroma, meningioma, or nonle- sional epilepsy at 3 hospitals between January 2011 and February 2016 were collected and retrospectively analyzed. The values of NLR, dNLR, PLR, LMR, and PNI were compared among patients suffering from glioma, acoustic neuroma, meningioma, and nonlesional epilepsy and healthy controls by using nonparametric tests. Correlations between NLR, dNLR, PLR, LMR, PNI, and tumor grade were analyzed. Receiver operating characteristic (ROC) curve analysis was performed to evaluate the diagnostic significance of NLR, dNLR, PLR, LMR, PNI, and their paired combinations for glioma, particularly GBM. -
A MODEL PATIENT JEROME GROOPMAN Four Students in Their
A MODEL PATIENT JEROME GROOPMAN Four students in their third year at Harvard Medical School recently met a patient named Mr. Martin. The students’ mentors, two physicians, told them that Martin had come to the emergency room complaining of abdominal pain that had grown steadily worse over several days. Martin was lying on a stretcher, moaning. A monitor next to the stretcher indicated that his blood pressure was dangerously low—eighty over fifty-four—and his heart was racing at a hundred and eighteen beats per minute. An X-ray mounted on a light box on the wall showed loops of distended bowel, called an ileus. The intestine can swell like this when it is obstructed or inflamed. “It hurts!” Martin cried as the students reviewed his chart. “They told me you’d give me something for the pain.” “Should we give him something?” one student asked. “I guess so,” another replied. The first student emptied a syringe of morphine into an intravenous line attached to Martin’s arm. Within a few seconds, Martin stopped moaning. Then the monitor started to beep rapidly. Martin had stopped breathing. The syringe had contained twenty milligrams of morphine, a potentially lethal dose for someone in his condition. The students began to perform CPR. One passed an endotracheal tube through Martin’s vocal cords into his airway. Another began to pump oxygen into the tube. Dr. Nancy Oriol, the dean of students at Harvard Medical School, showed me a videotape of the students’ encounter with Martin. “The students didn’t figure it out,” she said. -
Malignant CNS Solid Tumor Rules
Malignant CNS and Peripheral Nerves Equivalent Terms and Definitions C470-C479, C700, C701, C709, C710-C719, C720-C725, C728, C729, C751-C753 (Excludes lymphoma and leukemia M9590 – M9992 and Kaposi sarcoma M9140) Introduction Note 1: This section includes the following primary sites: Peripheral nerves C470-C479; cerebral meninges C700; spinal meninges C701; meninges NOS C709; brain C710-C719; spinal cord C720; cauda equina C721; olfactory nerve C722; optic nerve C723; acoustic nerve C724; cranial nerve NOS C725; overlapping lesion of brain and central nervous system C728; nervous system NOS C729; pituitary gland C751; craniopharyngeal duct C752; pineal gland C753. Note 2: Non-malignant intracranial and CNS tumors have a separate set of rules. Note 3: 2007 MPH Rules and 2018 Solid Tumor Rules are used based on date of diagnosis. • Tumors diagnosed 01/01/2007 through 12/31/2017: Use 2007 MPH Rules • Tumors diagnosed 01/01/2018 and later: Use 2018 Solid Tumor Rules • The original tumor diagnosed before 1/1/2018 and a subsequent tumor diagnosed 1/1/2018 or later in the same primary site: Use the 2018 Solid Tumor Rules. Note 4: There must be a histologic, cytologic, radiographic, or clinical diagnosis of a malignant neoplasm /3. Note 5: Tumors from a number of primary sites metastasize to the brain. Do not use these rules for tumors described as metastases; report metastatic tumors using the rules for that primary site. Note 6: Pilocytic astrocytoma/juvenile pilocytic astrocytoma is reportable in North America as a malignant neoplasm 9421/3. • See the Non-malignant CNS Rules when the primary site is optic nerve and the diagnosis is either optic glioma or pilocytic astrocytoma. -
Kathy Giusti and the Multiple Myeloma Research Foundation
9-814-026 JUNE 25, 2014 RICHARD G. HAMERMESH JOSHUA D. MARGOLIS MATTHEW G. PREBLE Kathy Giusti and the Multiple Myeloma Research Foundation This is the model for cancer. It is the model for medicine; of what we have to do.1 — Eric Lander, founding director of the Broad Institute Kathy Giusti (HBS ’85) was driving home one evening in January 1996 when she got the call from her doctor.2 He asked if Giusti and her husband Paul (HBS ’85) could come to his office.3 Sensing bad news, Giusti wanted to know what was wrong.4 Her doctor relented and told Giusti that she had multiple myeloma (MM), a rare and fatal cancer.5 Early the next morning, Giusti went to a bookstore near her Chicago, Illinois home and headed for the medical section.6 “We were going through these books, and I started thinking ‘Oh my God, what just happened to my life,’” she recalled. After her appointment, Giusti’s worst fears were confirmed. She could expect to live for just a few more years—three or four at the most. Up to that moment everything seemed to be going as planned.7 Since graduating from Harvard Business School (HBS) just over a decade earlier, Giusti had had one career success after another. At the time of her diagnosis, she was the executive in charge of global operations for G.D. Searle & Co.’s (Searle) arthritis drugs division.8 The Giustis had recently had their first child and just started trying to have a second.9 Now, all was in flux. -
In Their Own Words
DEPARTMENT OF MEDICINE ANNUAL REPORT 2017 Department of Medicine Beth Israel Deaconess Medical Center 330 Brookline Avenue, Boston, MA 02215 617-667-7000 bidmc.org/medicine IN THEIR OWN WORDS Beth Israel Deaconess Medical Center is a patient care, teaching and research affiliate of Harvard Medical School and consistently ranks as a national leader among independent hospitals in National Institutes of Health funding. BIDMC is in the community with Beth Israel Deaconess Hospital-Milton, Beth Israel Deaconess Hospital-Needham, Beth Israel Deaconess Hospital-Plymouth, Anna Jaques Hospital, Cambridge Health Alliance, Lawrence General Hospital, Signature Healthcare, Beth Israel Deaconess HealthCare, Community Care Alliance and Atrius Health. BIDMC is also clinically affiliated with the Joslin Diabetes Center and Hebrew SeniorLife and is a research partner of Dana-Farber/Harvard Cancer Center and The Jackson Laboratory. BIDMC is the official hospital of the Boston Red Sox. For more information, visit www.bidmc.org. The Department of Medicine wishes to thank the many individuals who contributed to this report, including department and division leaders. We also thank Gigi Korzenowski and Jerry Clark of Korzenowski Design, and Jennie Greene, Meera Kanabar, and Jacqueline St. Onge of the Department of Medicine. The photography in this report was done by BIDMC’s Jim Dwyer and Danielle Duffey, who also helped with photo research. Jane Hayward, of BIDMC Media Services, provided expert copy editing and design consultation. We also thank several members of the Development and Communications Departments for their input. Last but not least, we wish to thank all of the individuals featured in these pages for contributing their time and perspectives to this year’s annual report. -
Acoustic Neuroma: a Basic Overview
Acoustic Neuroma: A Basic Overview IMPORTANT POINTS TO KNOW ABOUT AN ACOUSTIC NEUROMA: • An acoustic neuroma is a benign tumor. • It is usually slow growing and expands at its site of origin. • The most common first symptom is hearing loss in the tumor ear. • The cause is unknown. • A large tumor pushes on the surface of the brain but does not grow into the brain tissue. • Continued tumor growth can be life threatening. • The treatment options are observation, surgical removal or radiation. WHAT IS AN ACOUSTIC NEUROMA? An acoustic neuroma (sometimes termed a vestibular schwannoma or neurolemmoma) is a benign (non- cancerous) tissue growth that arises on the eighth cranial nerve leading from the brain to the inner ear. This nerve has two distinct parts, one part associated with transmitting sound and the other with sending balance information to the brain from the inner ear. These pathways, along with the facial nerve, lie adjacent to each other as they pass through a bony canal called the internal auditory canal. This canal is approximately 2 cm (0.8 inches) long and it is here that acoustic neuromas originate from the sheath surrounding the eighth nerve. The facial nerve provides motion of the muscles of facial expression. Acoustic neuromas usually grow slowly over a period of years. They expand in size at their site of origin and when large can displace normal brain tissue. The brain is not invaded by the tumor, but the tumor pushes the brain as it enlarges. The slowly enlarging tumor protrudes from the internal auditory canal into an area behind the temporal bone called the cerebellopontine angle. -
Inspirations from the Field the Proverbial
The Newsletter of The Arnold P. Gold Foundation A Public Foundation Dedicated to Fostering Humanism in Medicine Thanksgiving 1999 The Proverbial Inspirations from the field Last Straw! It was a proverbial “last straw” that brought us to Dr. Arnold Gold’s office, “TheWhite Coat–Just in 1995. A few days before our son an item of clothing!! Anthony was to become a first grader, he rushed towards me and the force of Yet we endow it with great his impact knocked me against a wall in symbolism and power. our house,driving my hand into the skin of my face,narrowly missing my eye. When I told my mother about this speech, she told me a story that she had This was only the latest in a number of never shared with me before. My physical “accidents” in which he hurt me, father was a patient in the hospital and for which he would later apologize. where I was a resident. She remembers Anthony had been discharged from two seeing me walking down the hall Members of the Mercer University School of Medicine, child-care centers by age four, not for towards my father’s room. I was dressed Class of 2003, recite the Oath of Geneva during the School’s aggression but for not listening to the August 8, 1999 White Coat Ceremony in Macon, Georgia. in my white coat with a group of distin- teacher and/or being uncooperative. His guished white-coat-clad attending qualities I look for in my physicians are behavior at home was even worse and physicians. -
Acoustic Neuroma
Acoustic Neuroma DISORDERS This document is adapted from materials available from the National Institutes of Health WHAT IS AN ACOUSTIC NEUROMA? VESTIBULAR An acoustic neuroma (also known as vestibular schwannoma or acoustic SCHWANNOMA neurinoma) is a benign (nonmalignant), usually slow-growing tumor that develops from the balance and hearing nerves supplying the inner ear. A benign slow-growing The tumor comes from an overproduction of Schwann cells—the cells that tumor on the normally wrap around nerve fibers to help support and insulate nerves. balance/hearing nerve. HOW DOES IT DEVELOP? As the acoustic neuroma grows, it compresses the hearing and balance nerves, usually causing unilateral (one-sided) hearing loss, tinnitus ARTICLE (ringing in the ear), and dizziness or loss of balance. As it grows, it can also interfere with the facial sensation nerve (the trigeminal nerve), causing facial numbness. It can also exert pressure on nerves controlling the muscles of the face, causing facial weakness or paralysis on the side of the tumor. Vital life-sustaining functions can be threatened when large 03 tumors cause severe pressure on the brainstem and cerebellum. Unilateral acoustic neuromas account for approximately eight percent of all tumors inside the skull; one out of every 100,000 individuals per year develops an acoustic neuroma. Symptoms may develop in individuals DID THIS ARTICLE at any age, but usually occur between the ages of 30 and 60 years. HELP YOU? Unilateral acoustic neuromas are not hereditary. SUPPORT VEDA @ HOW IS IT DIAGNOSED? VESTIBULAR.ORG Early detection of an acoustic neuroma is sometimes difficult because the symptoms related to its early stages may be subtle, if present at all. -
Hemorrhagic Vestibular Schwannoma: an Unusual Clinical Entity Case Report
Neurosurg Focus 5 (3):Article 9, 1998 Hemorrhagic vestibular schwannoma: an unusual clinical entity Case report Dean Chou, M.D., Prakash Sampath, M.D., and Henry Brem, M.D. Departments of Neurological Surgery and Neuro-Oncology, The Johns Hopkins Hospital, Baltimore, Maryland Hemorrhagic vestibular schwannomas are rare entities, with only a few case reports in the literature during the last 25 years. The authors review the literature on vestibular schwannoma hemorrhage and the presenting symptoms of this entity, which include headache, nausea, vomiting, sudden cranial nerve dysfunction, and ataxia. A very unusual case is presented of a 36-year-old man, who unlike most of the patients reported in the literature, had clinically silent vestibular schwannoma hemorrhage. The authors also discuss the management issues involved in more than 1000 vestibular schwannomas treated at their institution during a 25-year period. Key Words * acoustic tumor * schwannoma * neuroma * hemorrhage * hemorrhagic vestibular tumor Vestibular schwannomas are the most common tumors found in the cerebellopontine angle (CPA), comprising approximately 80% of all tumors arising in this region. They are usually slow-growing, benign tumors that manifest clinically by directly compressing the neural elements traversing the CPA, including the pons, cerebellum, and the lower cranial nerves. Hemorrhage into a vestibular schwannoma is rare and has been shown to present with acute neurological changes and deterioration. We review the literature on the presenting symptoms of hemorrhagic vestibular schwannomas and describe the case of a man with a large hemorrhagic vestibular schwannoma who presented with signs and symptoms of a slow-growing, insidious compressive lesion. In doing so, we hope to shed light on this unusual clinical entity and discuss management issues. -
Coexisting MS and Lhermitte-Duclos Disease Casperson Et Al
Neuroradiology : Coexisting MS and Lhermitte-Duclos Disease Casperson et al. Coexisting MS and Lhermitte -Duclos Disease Bria K. Casperson1, Victor Anaya-Baez1, Stephen S. Kirzinger2, Ronald Sattenberg1, Jens O. Heidenreich1* 1. Department of Radiology, School of Medicine, University of Louisville, Louisville, KY, USA 2. Department of Adult Neurology, School of Medicine, University of Louisville, University Neurologists, PSC, Louisville, KY, USA * Correspondence: Jens O. Heidenreich, M.D., Assistant Professor of Radiology [email protected], University of Louisville Hospital, 530 S. Jackson Street, Louisville, Kentucky, 40202, USA ( [email protected]) Radiology Case. 2010 Aug; 4(8):1-6 :: DOI: 10.3941/jrcr.v4i8.476 ABSTRACT We report the case of a patient with pre-existing multiple sclerosis, who presented with horizontal diplopia, and a prior episode of progressive ataxia and dizziness lasting one week. While initially attributed to multiple www.RadiologyCases.com sclerosis, subsequent imaging demonstrated a concurrent left cerebellar gangliocytoma, also known as Lhermitte-Duclos disease. CASE REPORT CASE REPORT On recent examination, in 2010, our patient demonstrated visual acuity on the left of 20/50 and on the right of 20/30. A 41 year old female with known multiple sclerosis (MS) She had slowed cognitive processing speed and word finding presented to the Emergency Department with horizontal problems, as well as decreased verbal fluency. Symptoms of JournalRadiology of Case Reports diplopia after one previous episode of progressive ataxia and impulsivity and depression were apparent. She demonstrated dizziness, which was one week in duration. On physical exam, no cerebellar abnormalities at this time. All other areas of the visual acuity was decreased to 20/30 on the left eye.