DOCSLIB.ORG

Olfactory Neuroblastoma: Everything Radiologists Should Know

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Olfactory Neuroblastoma: Everything Radiologists Should Know Review article Olfactory Neuroblastoma: Everything Radiologists Should Know Raquel Navas-Campo1 Leticia Moreno Caballero1 Ana Gasos Lafuente1 Pilar Tobajas Morlana1 Eduardo Séez Valero1 María José Gimeno Peribáñez1 1 Hospital Clínico Universitario Lozano Blesa, Zaragoza, Spain Abstract Olfactory neuroblastoma (ONB) is a rare malignant tumor that originates from olfactory neuroepithelial cells. Its early diagnosis is difficult due to the low specificity of symptoms. Imaging tests play an important role in the diagnosis and surgical planning of ONB; therefore, it is important that radiologists know the characteristic findings and the different classifications that will help to choose the most appropriate treatment for each tumor. Keywords Olfactory neuroblastoma, Esthesioneuroblastoma, Cancer of the Head and Neck, Computed Tomography, Magnetic Resonance Imaging Introduction of life and the second peak in the sixth decade of life, some recent studies support a uniform distribution across all ages, Olfactory neuroblastoma (ONB) is a rare malignancy of the with a peak in the fifth and sixth decades of life.3,4,6.12,13 upper nasal cavity, also known as esthesioneuroblastoma, Nonspecificity of symptoms and local aggressiveness lead to esthesioneuroepithelioma, esthesioneurocytoma or olfactory the development of locally advanced disease with submuco- placode.1 It was first described by Berger et al.2 in 1924, and sal spread to the paranasal sinuses and the anterior cranial the most widely accepted term at this time is “olfactory neu- fossa through the cribriform plate in a large number of pa- roblastoma”.3,4 tients.14-16 Metastatic disease affects between 17% and 48% The exact origin of this tumor, as well as the location and of patients.17 Cervical lymph nodes are the most common cell type are under debate.5 Proposed anatomic sites of ori- sites of metastasis, with an incidence ranging between 20% gin include Jacobson organ (vomeronasal organ), the sphe- and 30%, followed by the lung, bone and, rarely, the liver, nopalatine ganglion, the ectodermal olfactory placode, Loci’s mediastinum, adrenal gland, ovary, spleen or parotid gland or ganglion, sympathetic ganglia of the nasal mucosa, and the even the breast.12,18-24 nasal mucosa itself. However, the most likely site of origin of Imaging plays an important role in the diagnosis and surgical ONB is the basal neural cells of the olfactory mucosa.5-7 This planning of ONB. Therefore, it is essential that radiologists is supported by the fact that neural filaments are present in know the characteristic findings of ONB on the various imag- tumor cells, as demonstrated by Trojanowaski et al.8 in 1982, ing modalities and the classifications that will help to choose and by molecular analysis, as demonstrated by Carney et al.9 the most appropriate treatment for each tumor. in 1995, with the expression of the Drosophila achaete-scute gene (hASH1), involved in immature olfactory neuronal de- velopment.5,6,10 Histology and immunohistochemistry This tumor accounts for approximately 2% to 3% of all intra- nasal malignancies.11 It affects both genders equally. It may Histologically, ONB is composed of small round blue cells occur at all ages and, although it was thought to have a bi- slightly larger than mature lymphocytes. These cells pos- modal age distribution with one peak in the second decade sess round-to-oval-shaped nuclei, with scanty cytoplasm and Rev. Argent. Radiol. 2020;84(1): 17-31 17 Olfactory Neuroblastoma: Everything Radiologists Should Know sharply defined chromatin. They also have layers of malignant There is growing evidence in literature that this classification cells organized into lobules through slender vascular fibrous may be used as an indicator for prognosis. High-grade tu- septa, true neural rosettes (Flexner– Wintersteiner type) and mors in Hyams classification have been found to be associ- pseudorosettes (Homer Wright type).23 ated with more aggressive locoregional disease and a worst The differential diagnosis of ONB includes all small round survival outcome than low-grade tumors.4,34,35 Furthermore, blue cell tumors occurring in the sinonasal tract. These in- Hyams classification may add value to guide the selection of clude epithelial neoplasms such as sinonasal undifferentiated adjuvant therapy.34 carcinoma, sinonasal neuroendocrine carcinoma, squamous carcinoma and melanoma; non-epithelial neoplasms such as rhabdomyosarcoma, lymphoproliferative tumors and benign Clinical manifestations lesions such as inverted papilloma and schwannoma.25 Differential diagnosis is possible, especially in cases of high- The most common presenting symptoms are nasal obstruc- grade lesions, only by immunohistochemistry.26 The tumor tion followed by recurrent epistaxis. Other symptoms include cells of ONB express neuronal markers such as synaptophy- headache, facial pain, hyposmia and rhinorrhea.36.45 Occa- sin, chromogranin or neuron-specific enolase, as well as cal- sionally, it may occur as an asymptomatic nasal mass that retinin, which differentiate them from other types of small is found incidentally.45 Advanced stage disease manifests round blue cell tumors.27,28 with symptoms related to local extension beyond the nose The cells in the periphery of the lobules are highly reactive and paranasal sinuses, such as epiphora, diplopy, proptosis for protein S100. Cytokeratin is usually negative, although in and loss of visual acuity.36,43-45 Rarely, patients may present some isolated cases it may exhibit an irregular expression.29 with symptoms of intracranial involvement, such as frontal lobe symptoms and seizures.38,45 On rare occasions, paraneo- plastic symptoms have been reported resulting from ectopic Histological Classification hormonal secretion, including Cushing syndrome and hypo- natremia secondary to adrenocorticotropic hormone (ACTH) The histological grading system proposed by Hyams et al.30 and antidiuretic hormone (ADH) secretion, respectively.45-48 (Table 1) was developed in 1988 as an important tool for Locally, advanced disease may present signs of invasion and prognosis, both of recurrence and survival..30,31 The criteria destruction of surrounding structures, such as the orbit, underlying this classification are the mitotic activity, native brain, facial soft tissues and skin.3,6,38 architecture conservation, matrix features, nuclear poly- Because of the nonspecificity of the early symptoms of ONB, morphism, the existence of rosettes and tumor necrosis.32 there is generally a delay in diagnosis, with an average time This grading system stratifies tumors into four groups rang- of 6 to 12 months between the onset of symptoms and di- ing from well differentiated (grade I) to least differentiated agnosis.3,6,19,38,49 (grade IV). This is a subjective complex system and sometimes definitive separation into grades becomes difficult. For this reason, there has been a tendency to group these categories Imaging Diagnosis into low-grade, comprising grades I and II, and high-grade, comprising grades III and IV, in order to provide and easier Imaging is very important in the diagnosis and staging of description of these tumors in correlation with prognosis.33 ONB. Generally, a combination of computed tomography Table 1: The Hyams histological grading system. * HW rosettes: true rosettes (Flexner– Wintersteiner type). * FW Pseudoro- settes (Homer Wright type). GRADE Preservation of Mitotic Nuclear Fibrillary Rosettes Necrosis lobular architecture index polymorphism matrix I + None None Prominent HW rosettes None II + Low Moderate Present HW rosettes None III + / - Moderate Prominent Present FW pseudorosettes Rare IV + / - High Marked Absent None Frequent 18 Rev. Argent. Radiol. 2020;84(1): 17-31 R. Navas-Campo et al. (CT) and magnetic resonance imaging (MRI) is required for 12).55 On MRI, ONB appears hypointense to gray matter on an optimal evaluation of the extent of the tumor.38,49 Both T1-weighted images and iso- to hyperintense to gray matter tests allow diagnosis, definition of the extent and stage of on T2-weighted images (Figs. 10-15).56 The tumor demon- the tumor and of the surgical approach, and monitoring and strates homogeneous enhancement except for areas of ne- evaluation of the treatment response. crosis or hemorrhage. In addition, MRI can differentiate the Thin-slice CT scan (1-mm slice thickness) with reconstruction tumor from entrapped secretions, which are hyperintense on in coronal and sagittal planes is the best initial imaging test T2-weighted images.53,55 The classic imaging findings include for assessing ONB. On CT, ONB appears as a homogeneous a “dumbbell-shaped” mass extending across the cribriform soft-tissue mass in the nasal vault with uniform and moderate plate (Fig. 12), with the narrow portion at the cribriform contrast enhancement (Figs. 1 and 2). Scattered speckled cal- plate. Peripheral tumor cysts (Figs. 5b, 12, 14, 16) and speck- cifications may be present (Figs. 2-4, 5a, 6).50 The main value led calcifications are quite characteristic of ONB.1 of CT is that it provides better definition of bone involvement MRI fat-saturated sequences help distinguish tumor from or- than MRI. CT helps to evaluate bone erosion of the cribriform bital fat and muscle. A smooth margin of the tumor-fat inter- plate, fovea ethmoidalis and lamina papyracea (Figs. 1-4, 5a, face suggests that the lesion is contained by periorbital fascia 8). However, bone remodeling without erosion may occur in (Fig. 15), while an irregular margin favors frank invasion of some cases due to the indolent growth pattern.3,35,49 CT scans the orbit.35,49,57 However, the definitive diagnosis of invasion generally show a lytic pattern and very rarely dominant hy- of dura and periorbital tissues is possible only at surgery.49 perostosis mimicking fibrous dysplasia.51 CT is also useful to Imaging of the neck in patients with ONB is crucial because assess regional neck and distant metastasis.52,53 neck lymph nodes metastases are found at presentation in MRI is the imaging modality of choice for a detailed evalua- 5% of patients.33 Furthermore, it has been estimated that tion of the locoregional extent and staging of the tumor.
Load more

Recommended publications
  • Marginal Tumor Cysts As a Diagnostic MR Finding
    Sinonasal Esthesioneuroblastoma with Intracranial Extension: Marginal Tumor Cysts as a Diagnostic MR Finding Peter M. Som, Mika Lidov, Margaret Brandwein, Peter Catalano, and Hugh F. Biller PURPOSE: To determine whether the MR finding of cysts along the intracranial margin of sinonasal esthesioneuroblastomas can be considered to suggest this tumor. METHODS: MR scans of 54 patients who had sinonasal lesions with intracranial extension were examined specifically for cysts along the intracranial margins of the lesions. RESULTS: Only 3 of the 54 patients had these cysts, and all 3 of these patients had esthesioneuroblastoma. Surgical pathologic findings of one speci­ men showed the cyst to be marginally located within the tumor. CONCLUSION: If cysts are seen on MR along the intracranial margin of a sinonasal mass, this finding highly suggests esthesia­ neuroblastoma. Index terms: Esthesioneuroblastoma; Nose, magnetic resonance AJNR Am J Neuroradio/15:1259-1262, Aug 1994 Radiologists often seem on a constant quest amelanotic melanomas, and embryonal rhab­ to give histologic diagnosis for disease seen on domyosarcomas ( 2). All of these neoplasms are sectional imaging studies. Rarely can this be undifferentiated, small-cell tumors, and like es­ accomplished. However, sometimes the pres­ thesioneuroblastoma can occur in the nasal ence of one or more imaging findings can allow fossa, with spread to the paranasal sinuses and the radiologist to offer a histologic diagnosis that anterior cranial fossa. Electron microscopy and has a very high degree of reliability. With this histochemical testing are required to establish a aim in mind, we examined the imaging studies definitive diagnosis (3). of 54 patients who had sinonasal masses with Initially, there was hope that radiologists extension into the anterior cranial fossa.
    [Show full text]
  • Headmirror's ENT in a Nutshell Esthesioneuroblastoma Experts: Garret Choby, M.D. and Jamie Van Gompel, M.D. Presentation (0:28
    Headmirror’s ENT in a Nutshell Esthesioneuroblastoma Experts: Garret Choby, M.D. and Jamie Van Gompel, M.D. Presentation (0:28) - Symptomatology o Nasal obstruction o Hyposmia or anosmia o Epistaxis o Headache, vision changes o Rare presentation cervical metastasis (5% patients) - Physical Examination o High nasal vault tumor (olfactory cleft) o Fleshy mass, moderately vascular o Thorough cranial nerve and neck examination - Epidemiology o Bimodal age distribution o SEER database middle to late age (40-60s) - Differential Diagnosis o Squamous cell carcinoma o Sinonasal undifferentiated carcinoma o Sinonasal neuroendocrine carcinoma o Lymphoma (imaging can look very similar) o Rhabdomyosarcoma o Ewing Sarcoma o Sinonasal mucosal melanoma Pathophysiology (2:20) - Theorized to arise from bipolar cell from nasal epithelium (upper 1.5 cm of the nasal vault) - Small blue round cell tumor or neuroendocrine tumor - Homer-Wright rosettes (1/3 of patients) - Flexner wintersteiner rosettes Workup (6:00) - Imaging o CT scan . Boney erosion at the skull base o MRI . Uniformly enhancing mass in the upper nasal vault with possible extension into the paranasal sinuses and intracranially . Dumbbell appearance is not reliable sign clincally o PET-CT scan . Especially for Kadish C-D lesions . Metastatic work up usually not performed for small lesions or Hyams grade 1-2 - Biopsy o Most important next step after imaging, especially in clinic - Grading System o Kadish Staging System . A: confined into the nasal cavity . B: extension into the paranasal sinuses . C: tumor beyond nasal cavity and paranasal sinus which can include cribiform plate, erosion of skull base, intracranial cavity or orbital invasion . D: cervical nodes or distant metastasis o Hyams Grading System .
    [Show full text]
  • Endonasal Endoscopic Approach for Skull Base Neurofibroma: Is It Viable? 1Ashok Gupta, 2MK Tiwari, 3Neha Chauhan, 4Navjot Kaur, 5Vaiphei Kim
    AIJCR Ashok Gupta et al 10.5005/jp-journals-10013-1237 CASE REPORT Endonasal Endoscopic Approach for Skull Base Neurofibroma: Is It Viable? 1Ashok Gupta, 2MK Tiwari, 3Neha Chauhan, 4Navjot Kaur, 5Vaiphei Kim ABSTRACT CASE REPORT Anterior skull base and sinonasal schwannomas are rare A 25 years female presented to outpatient services of entities. Earlier, safest way to remove these tumors, with best Department of Otorhinolaryngology—Head and Neck success rates, was open craniofacial surgery but recently with Surgery, Postgraduate Institute of Medical Education introduction of endoscopic endonasal approach; complete and Research, Chandigarh with complaints of left sided resection of these rare entities is possible. We present one facial pain since 20 years. The pain was intermittent and such case of skull base neurofibroma which was resected in entirety via a purely endoscopic endonasal approach. episodic. The episode lasted for 15 to 20 minutes and was mild to moderate in intensity. It was more on the frontal Keywords: Endonasal, Endoscopic, Schwannoma, Skull base. region on left side and extended to the left eye and face. It How to cite this article: Gupta A, Tiwari MK, Chauhan N, was relieved with medication in the form of pain killers. Kaur N, Kim V. Endonasal Endoscopic Approach for Skull Base There was no history of nasal discharge, altered smell, Neurofibroma: Is It Viable?. Clin Rhinol An Int J 2015;8(2):72-75. decreased or double vision, seizures, fever, neck rigidity Source of support: Nil of any focal neurological deficit. There was no significant past medical or surgical, family or personal history. Conflict of interest: None On examination, her ENT examination was grossly normal.
    [Show full text]
  • Esthesioneuroblastoma, Neuroendocrine Carcinoma, and Sinonasal Undifferentiated Carcinoma: Differentiation in Diagnosis and Treatment
    THIEME Review Article S149 Esthesioneuroblastoma, Neuroendocrine Carcinoma, and Sinonasal Undifferentiated Carcinoma: Differentiation in Diagnosis and Treatment Shirley Y. Su1 Diana Bell2 Ehab Y. Hanna1 1 Department of Head and Neck Surgery, University of Texas MD Address for correspondence Ehab Y. Hanna, MD, Department of Head Anderson Cancer Center, Houston, Texas, United States and Neck Surgery, University of Texas MD Anderson Cancer Center, 2 Department of Pathology, University of Texas MD Anderson Cancer 1515 Holcombe Blvd., Suite 1445, Houston, TX 77030-4009, Center, Houston, Texas, United States United States (e-mail: [email protected]). Int Arch Otorhinolaryngol 2014;18:S149–S156. Abstract Introduction Malignant sinonasal tumors comprise less than 1% of all neoplasms. A wide variety of tumors occurring primarily in this site can present with an undifferenti- ated or poorly differentiated morphology. Among them are esthesioneuroblastomas, sinonasal undifferentiated carcinomas, and neuroendocrine carcinomas. Objectives We will discuss diagnostic strategies, recent advances in immunohis- tochemistry and molecular diagnosis, and treatment strategies. Data Synthesis These lesions are diagnostically challenging, and up to 30% of sinonasal malignancies referred to the University of Texas MD Anderson Cancer Center Keywords are given a different diagnosis on review of pathology. Correct classification is vital, as ► sinonasal malignancy these tumors are significantly different in biological behavior and response to treat- ► esthesioneuroblas- ment. The past decade has witnessed advances in diagnosis and therapeutic modalities toma leading to improvements in survival. However, the optimal treatment for esthesioneur- ► sinonasal oblastoma, sinonasal undifferentiated carcinoma, and neuroendocrine carcinoma undifferentiated remain debated. We discuss advances in immunohistochemistry and molecular diag- carcinoma nosis, diagnostic strategies, and treatment selection.
    [Show full text]
  • Second Revised Proposed Regulation of the State
    SECOND REVISED PROPOSED REGULATION OF THE STATE BOARD OF HEALTH LCB File No. R057-16 February 5, 2018 EXPLANATION – Matter in italics is new; matter in brackets [omitted material] is material to be omitted. AUTHORITY: §§1, 2, 4-9 and 11-15, NRS 457.065 and 457.240; §3, NRS 457.065 and 457.250; §10, NRS 457.065; §16, NRS 439.150, 457.065, 457.250 and 457.260. A REGULATION relating to cancer; revising provisions relating to certain publications adopted by reference by the State Board of Health; revising provisions governing the system for reporting information on cancer and other neoplasms established and maintained by the Chief Medical Officer; establishing the amount and the procedure for the imposition of certain administrative penalties by the Division of Public and Behavioral Health of the Department of Health and Human Services; and providing other matters properly relating thereto. Legislative Counsel’s Digest: Existing law defines the term “cancer” to mean “all malignant neoplasms, regardless of the tissue of origin, including malignant lymphoma and leukemia” and, before the 78th Legislative Session, required the reporting of incidences of cancer. (NRS 457.020, 457.230) Pursuant to Assembly Bill No. 42 of the 78th Legislative Session, the State Board of Health is: (1) authorized to require the reporting of incidences of neoplasms other than cancer, in addition to incidences of cancer, to the system for reporting such information established and maintained by the Chief Medical Officer; and (2) required to establish an administrative penalty to impose against any person who violates certain provisions which govern the abstracting of records of a health care facility relating to the neoplasms the Board requires to be reported.
    [Show full text]
  • Involvement of the Olfactory Apparatus by Gliomas
    CLINICAL REPORT HEAD & NECK Involvement of the Olfactory Apparatus by Gliomas X. Wu, Y. Li, C.M. Glastonbury, and S. Cha ABSTRACT SUMMARY: The olfactory bulbs and tracts are central nervous system white matter tracts maintained by central neuroglia. Although rare, gliomas can originate from and progress to involve the olfactory apparatus. Through a Health Insurance Portability and Accountability Act–compliant retrospective review of the institutional teaching files and brain MR imaging reports spanning 10 years, we identified 12 cases of gliomas involving the olfactory bulbs and tracts, including 6 cases of glioblastoma, 2 cases of ana- plastic oligodendroglioma, and 1 case each of pilocytic astrocytoma, diffuse (grade II) astrocytoma, anaplastic astrocytoma (grade III), and diffuse midline glioma. All except the pilocytic astrocytoma occurred in patients with known primary glial tumors else- where. Imaging findings of olfactory tumor involvement ranged from well-demarcated enhancing masses to ill-defined enhancing infiltrative lesions to nonenhancing masslike FLAIR signal abnormality within the olfactory tracts. Familiarity with the imaging find- ings of glioma involvement of the olfactory nerves is important for timely diagnosis and treatment of recurrent gliomas and to dis- tinguish them from other disease processes. ABBREVIATIONS: GBM ¼ glioblastoma multiforme; TMZ ¼ temozolomide; EGFR ¼ epidermal growth factor receptor; IDH1 ¼ Isocitrate dehydrogenase 1; MGMT ¼ O6-methylguanine methyltransferase he olfactory bulbs and tracts are central nervous system white bulbs and tracts and to differentiate them from other masses of Tmatter tracts extending directly to the cerebrum, maintained the anterior cranial fossa. by a combination of specialized olfactory ensheathing cells and central neuroglia, including astrocytes and oligodendrocytes.1 As Case Series a result, gliomas can rarely originate from and progress to involve – the olfactory apparatus.
    [Show full text]
  • An Unusual ENT Presentation of Retinoblastoma:10.5005/Jp-Journals-10013-1291 a Diagnostic Dilemma Coase Rep Rt
    AIJCR An Unusual ENT Presentation of Retinoblastoma:10.5005/jp-journals-10013-1291 A Diagnostic Dilemma COASE REP RT An Unusual ENT Presentation of Retinoblastoma: A Diagnostic Dilemma 1Tamoghna Jana, 2Moushumi Sengupta, 3Saumik Das, 4Asok K Saha, 5Subhasis Saha ABSTRACT histopathological slides and immunohistochemical study Retinoblastoma is the most common intraocular tumor of are therefore an essential tool to rule out any diagnostic childhood. These tumors, though they respond to treatment, dilemma. are prone to develop secondary malignancy, recurrence, and metastasis, which may present as sinonasal mass. We are CASE REPORT presenting a rare case of metastatic retinoblastoma of sinonasal region in a 3-year-old male child. The mode of presentation A 3-year-old male child presented to the ear, nose, and and management of the case is presented along with a review throat outpatient department of the Medical College and of the literature. Hospital in April 2013 with a painful, tender swelling of Keywords: Metastatic, Retinoblastoma, Sinonasal. the right side of face, which developed 3 weeks earlier and How to cite this article: Jana T, Sengupta M, Das S, Saha was rapidly increasing in size. The patient had a history AK, Saha S. An Unusual ENT Presentation of Retinoblastoma: of enucleation of the left eye in June 2012. Histopathologi- A Diagnostic Dilemma. Clin Rhinol An Int J 2016;9(3):149-152. cal examination confirmed it as primary retinoblastoma Source of support: Nil with involvement of the choroid and sclera for which the patient received postoperative radiotherapy by 21 Conflict of interest: None fraction, which was completed in September 2012.
    [Show full text]
  • 2018 Solid Tumor Rules Lois Dickie, CTR, Carol Johnson, BS, CTR (Retired), Suzanne Adams, BS, CTR, Serban Negoita, MD, Phd
    Solid Tumor Rules Effective with Cases Diagnosed 1/1/2018 and Forward Updated November 2020 Editors: Lois Dickie, CTR, NCI SEER Carol Hahn Johnson, BS, CTR (Retired), Consultant Suzanne Adams, BS, CTR (IMS, Inc.) Serban Negoita, MD, PhD, CTR, NCI SEER Suggested citation: Dickie, L., Johnson, CH., Adams, S., Negoita, S. (November 2020). Solid Tumor Rules. National Cancer Institute, Rockville, MD 20850. Solid Tumor Rules 2018 Preface (Excludes lymphoma and leukemia M9590 – M9992) In Appreciation NCI SEER gratefully acknowledges the dedicated work of Dr. Charles Platz who has been with the project since the inception of the 2007 Multiple Primary and Histology Coding Rules. We appreciate the support he continues to provide for the Solid Tumor Rules. The quality of the Solid Tumor Rules directly relates to his commitment. NCI SEER would also like to acknowledge the Solid Tumor Work Group who provided input on the manual. Their contributions are greatly appreciated. Peggy Adamo, NCI SEER Elizabeth Ramirez, New Mexico/SEER Theresa Anderson, Canada Monika Rivera, New York Mari Carlos, USC/SEER Jennifer Ruhl, NCI SEER Louanne Currence, Missouri Nancy Santos, Connecticut/SEER Frances Ross, Kentucky/SEER Kacey Wigren, Utah/SEER Raymundo Elido, Hawaii/SEER Carolyn Callaghan, Seattle/SEER Jim Hofferkamp, NAACCR Shawky Matta, California/SEER Meichin Hsieh, Louisiana/SEER Mignon Dryden, California/SEER Carol Kruchko, CBTRUS Linda O’Brien, Alaska/SEER Bobbi Matt, Iowa/SEER Mary Brandt, California/SEER Pamela Moats, West Virginia Sarah Manson, CDC Patrick Nicolin, Detroit/SEER Lynda Douglas, CDC Cathy Phillips, Connecticut/SEER Angela Martin, NAACCR Solid Tumor Rules 2 Updated November 2020 Solid Tumor Rules 2018 Preface (Excludes lymphoma and leukemia M9590 – M9992) The 2018 Solid Tumor Rules Lois Dickie, CTR, Carol Johnson, BS, CTR (Retired), Suzanne Adams, BS, CTR, Serban Negoita, MD, PhD Preface The 2007 Multiple Primary and Histology (MPH) Coding Rules have been revised and are now referred to as 2018 Solid Tumor Rules.
    [Show full text]
  • Molecular Cytogenetic Analysis in the Study of Brain Tumors: Findings and Applications
    Neurosurg Focus 19 (5):E1, 2005 Molecular cytogenetic analysis in the study of brain tumors: findings and applications JANE BAYANI, M.H.SC., AJAY PANDITA, D.V.M., PH.D., AND JEREMY A. SQUIRE, PH.D. Department of Applied Molecular Oncology, Ontario Cancer Institute, Princess Margaret Hospital, University Health Network; Arthur and Sonia Labatt Brain Tumor Research Centre, Hospital for Sick Children; and Departments of Laboratory Medicine and Pathobiology and Medical Biophysics, University of Toronto, Ontario, Canada Classic cytogenetics has evolved from black and white to technicolor images of chromosomes as a result of advances in fluorescence in situ hybridization (FISH) techniques, and is now called molecular cytogenetics. Improvements in the quality and diversity of probes suitable for FISH, coupled with advances in computerized image analysis, now permit the genome or tissue of interest to be analyzed in detail on a glass slide. It is evident that the growing list of options for cytogenetic analysis has improved the understanding of chromosomal changes in disease initiation, progression, and response to treatment. The contributions of classic and molecular cytogenetics to the study of brain tumors have pro- vided scientists and clinicians alike with new avenues for investigation. In this review the authors summarize the con- tributions of molecular cytogenetics to the study of brain tumors, encompassing the findings of classic cytogenetics, interphase- and metaphase-based FISH studies, spectral karyotyping, and metaphase- and array-based comparative genomic hybridization. In addition, this review also details the role of molecular cytogenetic techniques in other aspects of understanding the pathogenesis of brain tumors, including xenograft, cancer stem cell, and telomere length studies.
    [Show full text]
  • Cranial and Spinal Leptomeningeal Dissemination In
    Surgical Neurology International OPEN ACCESS Editor: S. A. Enam, MD SNI: Unique Case Observations, a supplement to Surgical Neurology International For entire Editorial Board visit : Aga Kahn University; Karachi, http://www.surgicalneurologyint.com Sindh, Pakistan Cranial and spinal leptomeningeal dissemination in esthesioneuroblastoma: Two reports of distant central nervous system metastasis and rationale for treatment Walavan Sivakumar, Nathan Oh1, Aaron Cutler, Howard Colman, William T. Couldwell Department of Neurosurgery, Clinical Neurosciences Center, University of Utah, Salt Lake City, Utah 84132, 1Department of Neurosurgery, Loma Linda University, Loma Linda, California 92354, USA E‑mail: Walavan Sivakumar ‑ [email protected]; Nathan Oh ‑ [email protected]; Aaron Cutler ‑ [email protected]; Howard Colman ‑ [email protected]; *William T. Couldwell ‑ [email protected] *Corresponding author Received: 18 June 15 Accepted: 08 October 15 Published: 25 November 15 Abstract Background: Esthesioneuroblastoma is a locally aggressive cancer of the nasal cavity. While systemic metastasis can occur in 10-30% of patients, there are only six reported cases of distal metastasis from leptomeningeal dissemination. Access this article online Case Description: The authors report two cases of esthesioneuroblastoma treated Website: previously with multimodal therapy in which distal metastatic recurrence was found www.surgicalneurologyint.com and describe their treatment protocol, which has resulted in long-term success. DOI:
    [Show full text]
  • Olfactory Neuroblastoma
    Head and Neck Pathol (2009) 3:252–259 DOI 10.1007/s12105-009-0125-2 HEAD AND NECK PRE-MEETING OF THE 22ND EUROPEAN CONGRESS OF PATHOLOGY Olfactory Neuroblastoma Lester D. R. Thompson Received: 15 June 2009 / Accepted: 22 June 2009 / Published online: 16 July 2009 Ó Humana 2009 Abstract Few neoplasms are unique to the sinonasal Introduction tract, but sinonasal undifferentiated carcinoma and olfac- tory neuroblastoma are malignant tumors which require Few neoplasms are unique to the sinonasal tract, but sin- unique management. Due to the rarity of these tumors, onasal undifferentiated carcinoma and olfactory neuro- practicing pathologists are not always aware of their dis- blastoma are malignant tumors which require unique tinctive clinical, radiographic, histologic, immunohisto- management. Due to the rarity of these tumors, practicing chemical, and molecular features. These cases are pathologists are not always aware of their distinctive frequently submitted for consultation, further suggesting clinical, radiographic, histologic, immunohistochemical, the diagnostic difficulties inherent to these tumors. Spe- and molecular features. These cases are frequently sub- cifically, olfactory neuroblastoma is a neoplasm that can mitted for consultation, further suggesting the diagnostic histologically mimic many tumors within the sinonasal difficulties inherent to these tumors. Specifically, olfactory tract, making recognition of this tumor important, as the neuroblastoma is a neoplasm that can histologically mimic management frequently requires a bicranial-facial surgical many tumors within the sinonasal tract, making recognition approach, a trephination procedure which can be quite of this tumor important as the management requires a bi- technically difficult and challenging to achieve a good cranial-facial surgical approach, a trephination procedure result.
    [Show full text]
  • Pediatric Brain Tumors Pre, Intra & Post Op Evaluation and Management
    Pediatric Brain Tumors Pre, Intra & Post Op Evaluation and Management Timothy M. George, MD, FACS, FAAP PEDIATRIC BRAIN TUMORS BACKGROUND: • Incidence: Third most common pediatric tumor type – (leukemia, neuroblastoma, brain cancers, lymphomas, Wilms, germ cell tumors, retinoblastoma, other types) • Midline location (third or fourth ventricle) • Typical presenting S&S: headache, seizures, neurological deficits, endocrinopathy PEDIATRIC BRAIN TUMORS • Most frequently, they come from "young" cells. ("immature" or "primitive" cells) that have not reached full maturity. • They are developing at the same time as the child is developing. • Brain tumor types mirror the way a normal cell matures from its very beginning as a "primitive" brain cell (a precursor) towards becoming an adult cell type. • “Cancer Stem Cell” PEDIATRIC BRAIN TUMORS Brain tumors behave differently than other tumors in the body Types: Intraparenchymal (benign or malignant) Extra-axial (generally benign) Brain Tumor Types • Astrocytoma / Glioma Astrocytomas are tumors that arise from brain cells called astrocytes. Gliomas originate from glial cells, most often astrocytes. • Atypical Teratoid / Rhabdoid Tumor (ATRT) This rare, high-grade tumor occurs most commonly in children younger than 2... • Brain Stem Glioma The brain stem consists of the midbrain, pons and medulla located deep in the posterior part of the brain. Usually malignant, sometimes benign. • Choroid Plexus Tumor The choroid plexus papilloma is a rare, benign tumor most common in children under the age of 2. • Craniopharyngioma Craniopharyngiomas result from the growth of cells that have failed to migrate to their usual area just below the back of the skull early in fetal development. • Ependymoma Ependymomas arise from cells lining the passageways in the brain that produce and store the cerebrospinal fluid or CSF.
    [Show full text]