Dysembryoplastic Neuroepithelial Tumours Compared with Other
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Charts Chart 1: Benign and Borderline Intracranial and CNS Tumors Chart
Charts Chart 1: Benign and Borderline Intracranial and CNS Tumors Chart Glial Tumor Neuronal and Neuronal‐ Ependymomas glial Neoplasms Subependymoma Subependymal Giant (9383/1) Cell Astrocytoma(9384/1) Myyppxopapillar y Desmoplastic Infantile Ependymoma Astrocytoma (9412/1) (9394/1) Chart 1: Benign and Borderline Intracranial and CNS Tumors Chart Glial Tumor Neuronal and Neuronal‐ Ependymomas glial Neoplasms Subependymoma Subependymal Giant (9383/1) Cell Astrocytoma(9384/1) Myyppxopapillar y Desmoplastic Infantile Ependymoma Astrocytoma (9412/1) (9394/1) Use this chart to code histology. The tree is arranged Chart Instructions: Neuroepithelial in descending order. Each branch is a histology group, starting at the top (9503) with the least specific terms and descending into more specific terms. Ependymal Embryonal Pineal Choro id plexus Neuronal and mixed Neuroblastic Glial Oligodendroglial tumors tumors tumors tumors neuronal-glial tumors tumors tumors tumors Pineoblastoma Ependymoma, Choroid plexus Olfactory neuroblastoma Oligodendroglioma NOS (9391) (9362) carcinoma Ganglioglioma, anaplastic (9522) NOS (9450) Oligodendroglioma (9390) (9505 Olfactory neurocytoma Ganglioglioma, malignant (()9521) anaplastic (()9451) Anasplastic ependymoma (9505) Olfactory neuroepithlioma Oligodendroblastoma (9392) (9523) (9460) Papillary ependymoma (9393) Glioma, NOS (9380) Supratentorial primitive Atypical EdEpendymo bltblastoma MdllMedulloep ithliithelioma Medulloblastoma neuroectodermal tumor tetratoid/rhabdoid (9392) (9501) (9470) (PNET) (9473) tumor -
Central Nervous System Tumors General ~1% of Tumors in Adults, but ~25% of Malignancies in Children (Only 2Nd to Leukemia)
Last updated: 3/4/2021 Prepared by Kurt Schaberg Central Nervous System Tumors General ~1% of tumors in adults, but ~25% of malignancies in children (only 2nd to leukemia). Significant increase in incidence in primary brain tumors in elderly. Metastases to the brain far outnumber primary CNS tumors→ multiple cerebral tumors. One can develop a very good DDX by just location, age, and imaging. Differential Diagnosis by clinical information: Location Pediatric/Young Adult Older Adult Cerebral/ Ganglioglioma, DNET, PXA, Glioblastoma Multiforme (GBM) Supratentorial Ependymoma, AT/RT Infiltrating Astrocytoma (grades II-III), CNS Embryonal Neoplasms Oligodendroglioma, Metastases, Lymphoma, Infection Cerebellar/ PA, Medulloblastoma, Ependymoma, Metastases, Hemangioblastoma, Infratentorial/ Choroid plexus papilloma, AT/RT Choroid plexus papilloma, Subependymoma Fourth ventricle Brainstem PA, DMG Astrocytoma, Glioblastoma, DMG, Metastases Spinal cord Ependymoma, PA, DMG, MPE, Drop Ependymoma, Astrocytoma, DMG, MPE (filum), (intramedullary) metastases Paraganglioma (filum), Spinal cord Meningioma, Schwannoma, Schwannoma, Meningioma, (extramedullary) Metastases, Melanocytoma/melanoma Melanocytoma/melanoma, MPNST Spinal cord Bone tumor, Meningioma, Abscess, Herniated disk, Lymphoma, Abscess, (extradural) Vascular malformation, Metastases, Extra-axial/Dural/ Leukemia/lymphoma, Ewing Sarcoma, Meningioma, SFT, Metastases, Lymphoma, Leptomeningeal Rhabdomyosarcoma, Disseminated medulloblastoma, DLGNT, Sellar/infundibular Pituitary adenoma, Pituitary adenoma, -
Desmoplastic Infantile Ganglioglioma/Astrocytoma (DIG/DIA) Are Distinct Entities with Frequent BRAFV600 Mutations
Published OnlineFirst July 13, 2018; DOI: 10.1158/1541-7786.MCR-17-0507 Genomics Molecular Cancer Research Desmoplastic Infantile Ganglioglioma/ Astrocytoma (DIG/DIA) Are Distinct Entities with Frequent BRAFV600 Mutations Anthony C. Wang1, David T.W. Jones2, Isaac Joshua Abecassis3, Bonnie L. Cole4, Sarah E.S. Leary5, Christina M. Lockwood6, Lukas Chavez2, David Capper7, Andrey Korshunov7, Aria Fallah1, Shelly Wang8, Chibawanye Ene3, James M. Olson5, J. Russell Geyer5, Eric C. Holland3, Amy Lee3, Richard G. Ellenbogen3, and Jeffrey G. Ojemann3 Abstract Desmoplastic infantile ganglioglioma (DIG) and desmo- transformation were found, and sequencing of the recurrence plastic infantile astrocytoma (DIA) are extremely rare tumors demonstrated a new TP53 mutation in one case, new ATRX that typically arise in infancy; however, these entities have not deletion in one case, and in the third case, the original tumor been well characterized in terms of genetic alterations or harbored an EML4–ALK fusion, also present at recurrence. clinical outcomes. Here, through a multi-institutional collab- DIG/DIA are distinct pathologic entities that frequently harbor V600 oration, the largest cohort of DIG/DIA to date is examined BRAF mutations. Complete surgical resection is the ideal using advanced laboratory and data processing techniques. treatment, and overall prognosis is excellent. While, the small Targeted DNA exome sequencing and DNA methylation sample size and incomplete surgical records limit a definitive profiling were performed on tumor specimens obtained from conclusion about the risk of tumor recurrence, the risk appears different patients (n ¼ 8) diagnosed histologically as DIG/ quite low. In rare cases with wild-type BRAF, malignant DIGA. Two of these cases clustered with other tumor entities, progression can be observed, frequently with the acquisition and were excluded from analysis. -
Gliomatosis Cerebri with Neurofibromatosis: an Autopsy-Proven Case
Child's Nerv Syst (1999) 15:219-221 © Springer-Verlag 1999 BRILF Önal Gliomatosis cerebri with neurofibromatosis: Çiçek an autopsy-proven case Gliomatosis cerebri Received: 15 July 1998 Abstract is a stereotactic ante-mortem diagnosis glial neoplastic process that is dif- and the other after autopsy. in this fusely distributed through neural paper. the autopsy-proven case Ç. Önal (BI) 1 • Ç. structures, whose anatomical config- of this rare disease with coexistent Department of Neurosurgery, uration remains Among the neurofibromatosis - the sixth lstanbul School of Medicine, more than 19,000 cases hospitalized case reported in the literature - Universitv of Istanbul, Çapa. Turkey in Istanbul University Istanbul is presented. School of Medicine Department of address: 1 Cad. Özenç Sok .. 4/9. Neurosurgery throughout the past Key words Gliomatosis cerebri · TR-81080 Erenköy-lstanbul. Turkey 45 years, only 2 cases were diag- Neurofibromatosis · Autopsy · Fax: +90-422-34 !O 726 nosed as gliomatosis cerebri. 1 by GFAP tened gyri showing diffuse oedema and no enhancement lntroduction (Fig. 1 Antibiotic and therapy was begun when the initial diagnosis of meningitis was made. No change was noted on Gliomatosis cerebri is a rare tumour of neuroepithelial or- follow-up CT performed 5 days later. MRI studie~ showed diffuse igin with vague presentation [l. 3. 5-8). Even hypo-isointense lesions in T1 and extensive lesions in with the aid T2-weighted and proton density tPDJ images (Figs. 2. 3). The me- of the most sophisticated techniques, ante-mortem diagno- dulla spinalis was in spite of an therapy. her condi- sis is difficult [2, 8). -
The Genetic Landscape of Ganglioglioma Melike Pekmezci1, Javier E
Pekmezci et al. Acta Neuropathologica Communications (2018) 6:47 https://doi.org/10.1186/s40478-018-0551-z RESEARCH Open Access The genetic landscape of ganglioglioma Melike Pekmezci1, Javier E. Villanueva-Meyer2, Benjamin Goode1, Jessica Van Ziffle1,3, Courtney Onodera1,3, James P. Grenert1,3, Boris C. Bastian1,3, Gabriel Chamyan4, Ossama M. Maher5, Ziad Khatib5, Bette K. Kleinschmidt-DeMasters6, David Samuel7, Sabine Mueller8,9,10, Anuradha Banerjee8,9, Jennifer L. Clarke10,11, Tabitha Cooney12, Joseph Torkildson12, Nalin Gupta8,9, Philip Theodosopoulos9, Edward F. Chang9, Mitchel Berger9, Andrew W. Bollen1, Arie Perry1,9, Tarik Tihan1 and David A. Solomon1,3* Abstract Ganglioglioma is the most common epilepsy-associated neoplasm that accounts for approximately 2% of all primary brain tumors. While a subset of gangliogliomas are known to harbor the activating p.V600E mutation in the BRAF oncogene, the genetic alterations responsible for the remainder are largely unknown, as is the spectrum of any additional cooperating gene mutations or copy number alterations. We performed targeted next-generation sequencing that provides comprehensive assessment of mutations, gene fusions, and copy number alterations on a cohort of 40 gangliogliomas. Thirty-six harbored mutations predicted to activate the MAP kinase signaling pathway, including 18 with BRAF p.V600E mutation, 5 with variant BRAF mutation (including 4 cases with novel in-frame insertions at p.R506 in the β3-αC loop of the kinase domain), 4 with BRAF fusion, 2 with KRAS mutation, 1 with RAF1 fusion, 1 with biallelic NF1 mutation, and 5 with FGFR1/2 alterations. Three gangliogliomas with BRAF p.V600E mutation had concurrent CDKN2A homozygous deletion and one additionally harbored a subclonal mutation in PTEN. -
Pediatric and Adolescent Oligodendrogliomas
Pediatric and Adolescent Oligodendrogliomas Harold Tice, 1 Patrick D. Barnes, 1 Liliana Goumnerova,2 R. Michael Scott,2 and Nancy J . Tarbell3 PURPOSE: To review the clinical and imaging findings in pediatric and adolescent intracranial pure oligodendrogliomas. METHODS: The clinical, CT, and MR data in 39 surgically proved pure oligodendrogliomas were retrospectively reviewed. RESULTS: The frontal or temporal lobes were involved in 32 (82%) cases. Seventy percent of the tumors were hypodense on CT, three-fourths were hypointense on T1-weighted images, and all were hyperintense on spin-density and T2- weighted images. Fewer than 40% of the lesions demonstrated calcification, and nearly 60% had well-defined margins. Mass effect was seen in fewer than half of the cases, and edema could be separately identified in only one case. Tumor enhancement was seen in fewer than 25%. In 39 cases after partial (3), subtotal (16), or total (20) resection, follow-up studies demonstrated stability over a mean period of 5 years. CONCLUSION: The findings in this pediatric series of pure oligodendrogliomas (without mixed cell elements) differ from previous adult series in that ca lcifi cation, contrast enhancement, and edema are seen less frequently. In addition, very slow or no growth is often characteristic, and these patients have an excellent prognosis with su rgical resection. Index terms: Oligodendroglioma; Brain, occipital lobe; Brain neoplasms, computed tomography; Brain neoplasms, magnetic resonance; Brain neoplasms, in infants and children; Pediatric neuro radiology AJNR 14:1293-1300, Nov /Dec 1993 Oligodendrogliomas comprise 4% to 7% of all eluded presentation, course, and the length of time from primary intracranial gliomas (1). -
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. -
An Unusual Brain Tumour of the Neuron Series
J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.45.2.139 on 1 February 1982. Downloaded from Journal of Neurology, Neurosiurgery, and Psychiatry 1982;45:139-142 Cerebral ganglioglio-neuroblastoma: an unusual brain tumour of the neuron series DARAB K DASTUR From the Neuropathology Unit, Post-Graduate Research Laboratories, Grant Medical College and JJ Group of Hospitals, Bombay, India SUMMARY The pathology of an unusual intracranial neuroectodermal tumour of the neuron series is described and its possible histogenesis discussed. The tumour, in a child aged 5 years with an enlarged head since infancy, presented as a large solid intra-cerebral mass. Histological examination showed four types of cells; (i) the stroma, forming the bulk of the tumour, was astrocytomatous; (ii) lobules of ill defined cells bearing small circular nuclei, representing immature neuroblasts; (iii) the same or other lobules containing neurons in various stages of development; and (iv) dense clusters of cells with hyperchromatic nuclei attempting rosettes, representing an overtly malignant neuro- blastoma. This tumour was designated "ganglioglio-neuroblastoma" and probably originated from a Protected by copyright. slow growing ganglioglioma. A brief account of cerebral neuroblastoma was given types.3 6 The subject has been very adequately 20 years ago by Russell and Rubinstein1 in the first summarised recently by Russell and Rubinstein7 and edition of their book. However, Horten and Rubinstein and Herman.8 In a chapter devoted to Rubinstein2 through their detailed pathological neuroblastoma and ganglioneuroma, Willis9 does report of 35 cases may be said to have established the not report any neuroblastoma of the central nervous cerebral neuroblastoma as a nosological entity with a system. -
COMMON BRAIN TUMORS Yarema Bezchlibnyk MD, Phd Assistant Professor of Neurosurgery Morsani School of Medicine, University of South Florida 2
1 COMMON BRAIN TUMORS Yarema Bezchlibnyk MD, PhD Assistant Professor of Neurosurgery Morsani School of Medicine, University of South Florida 2 OVERVIEW • To review the epidemiology, diagnosis, management, and prognosis of common brain tumors, including: • Metastatic tumors • Meningiomas • Glial tumors • High-grade astrocytomas/GBM • Anaplastic gliomas (AA, AO, AOA) • Low-grade gliomas (LGG) 3 EPIDEMIOLOGY OF BRAIN TUMORS • Overall incidence of brain tumors • ~21/100 000 • Metastases are the most common brain tumor seen clinically in adults • ~50% of brain tumors • Meningiomas = ~18% • Gliomas = ~12% • GBM = ~7% • Anaplastic and low-grade gliomas = ~5% • Pituitary adenomas = ~7% • Schwanommas = ~5% • Other = ~8% METASTATIC BRAIN TUMORS EPIDEMIOLOGY OF METASTATIC BRAIN TUMORS • 98,000-170,000 new cases diagnosed each year in U.S. • Peak age 50-70 • 25% of patients with systemic cancer have CNS metastasis on autopsy • 50-80% are multiple TUMOR TYPE AND INCIDENCE/RISK OF BRAIN METS LOCATION OF BRAIN METASTASES Most commonly found at the grey-white matter junction and superficial distal arterial fields The distribution reflects the blood flow to different regions 80% in cerebrum 15% cerebellum 5% brainstem Brem: Neurosurgery, Volume 57(5) Supplement.November 2005.S4-5-S4-9 CLINICAL PRESENTATION • 80% = Metachronous (>2 month after diagnosis of cancer) • 20% = Precocious (1st sign of cancer) or Synchronous (identified at or around the time of the cancer diagnosis) • Neurological symptoms may develop gradually or acutely • Headache, alteration in cognitive function • Other symptoms depending on location: • Focal weakness, ataxia, gait disturbance, cranial nerve findings, hydrocephalus, headache • 20% develop seizures • 15% present with brain bleed IMAGING 10 MANAGEMENT: OVERVIEW • Look for primary cancer • Stage the disease • Precocious • Biopsy or resection for tissue diagnosis • Synchronous or metachronous • Resection vs. -
Dysembryoplastic Neuroepithelial Tumor Originally Diagnosed As
ARTICLE Dysembryoplastic neuroepithelial tumor originally diagnosed as astrocytoma and oligodendroglioma Tumor neuroepitelial disembrioplásico diagnosticado originalmente como astrocitoma ou oligodendroglioma Diego Cassol Dozza1, Flávio Freinkel Rodrigues2, Leila Chimelli3 ABSTRACT Dysembryoplastic neuroepithelial tumor (DNT), described in 1988 and introduced in the WHO classification in 1993, affects predominantly children or young adults causing intractable complex partial seizures. Since it is benign and treated with surgical resection, its recognition is important. It has similarities with low-grade gliomas and gangliogliomas, which may recur and become malignant. Objectives: To investigate whether DNT was previously diagnosed as astrocytoma, oligodendroglioma, or ganglioglioma and to determine its frequency in a series of low-grade glial/glio-neuronal tumors. Methods: Clinical, radiological, and histological aspects of 58 tumors operated from 1978 to 2008, classified as astrocytomas (32, including 8 pilocytic), oligodendrogliomas (12), gangliogliomas (7), and DNT (7), were reviewed.Results: Four new DNT, one operated before 1993, previously classified as astrocytoma (3) and oligodendroglioma (1), were identified. One DNT diagnosed in 2002 was classified once more as angiocentric glioma. Therefore, 10 DNT (17.2%) were identified. Conclusions: Clinical-radiological and histopathological correlations have contributed to diagnose the DNT. Key words: dysembryoplastic neuroepithelial tumor, low-grade gliomas, epilepsy. RESUMO O tumor neuroepitelial -
2004 Implementation Guidelines: Collaborative Staging and Benign/Borderline Intracranial and CNS Tumors
NAACCR 2004 Implementation Guidelines: Collaborative Staging and Benign/Borderline Intracranial and CNS Tumors November 6, 2003 Revised November 14, 2003 NORTH AMERICAN ASSOCIATION OF CENTRAL CANCER REGISTRIES 2004 Implementation Guidelines Table of Contents NAACCR Collaborative Staging Implementation Work Group ............................................5 Benign Brain Tumor Implementation Work Group................................................................6 Acronyms and Abbreviations Used............................................................................................8 1 Introduction..........................................................................................................................9 1.1 Collaborative Staging.......................................................................................9 1.2 Benign and Borderline Intracranial and Central Nervous System Tumors ...10 2 Collaborative Staging System...........................................................................................11 2.1 Standard Setting Organization Collaborative Staging Reporting Requirements 11 2.1.1 CoC Reporting Requirements.........................................................................11 2.1.2 NPCR Reporting Requirements......................................................................12 2.1.3 SEER Reporting Requirements ......................................................................12 2.1.4 Canadian Council of Cancer Registries..........................................................13 2.2 -
New Jersey State Cancer Registry List of Reportable Diseases and Conditions Effective Date March 10, 2011; Revised March 2019
New Jersey State Cancer Registry List of reportable diseases and conditions Effective date March 10, 2011; Revised March 2019 General Rules for Reportability (a) If a diagnosis includes any of the following words, every New Jersey health care facility, physician, dentist, other health care provider or independent clinical laboratory shall report the case to the Department in accordance with the provisions of N.J.A.C. 8:57A. Cancer; Carcinoma; Adenocarcinoma; Carcinoid tumor; Leukemia; Lymphoma; Malignant; and/or Sarcoma (b) Every New Jersey health care facility, physician, dentist, other health care provider or independent clinical laboratory shall report any case having a diagnosis listed at (g) below and which contains any of the following terms in the final diagnosis to the Department in accordance with the provisions of N.J.A.C. 8:57A. Apparent(ly); Appears; Compatible/Compatible with; Consistent with; Favors; Malignant appearing; Most likely; Presumed; Probable; Suspect(ed); Suspicious (for); and/or Typical (of) (c) Basal cell carcinomas and squamous cell carcinomas of the skin are NOT reportable, except when they are diagnosed in the labia, clitoris, vulva, prepuce, penis or scrotum. (d) Carcinoma in situ of the cervix and/or cervical squamous intraepithelial neoplasia III (CIN III) are NOT reportable. (e) Insofar as soft tissue tumors can arise in almost any body site, the primary site of the soft tissue tumor shall also be examined for any questionable neoplasm. NJSCR REPORTABILITY LIST – 2019 1 (f) If any uncertainty regarding the reporting of a particular case exists, the health care facility, physician, dentist, other health care provider or independent clinical laboratory shall contact the Department for guidance at (609) 633‐0500 or view information on the following website http://www.nj.gov/health/ces/njscr.shtml.