DOCSLIB.ORG

Pilocytic Astrocytoma

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Pilocytic Astrocytoma J.W. Van Goethem C. Venstermans S. Dekeyzer neuronal tumors including DNET, ganglioglioma, and S. Vanden Bossche neurocytoma S. Nicolay P. M . Pa r i ze l L. van den Hauwe B. Goraj Antwerp University Hospital - University of Antwerp, Antwerp,/BE K. Kamphuis-van Ulzen AZ KLINA, Brasschaat/BE (University Medical Center St Radboud, Nijmegen/NL) suggested reading • 14 entities • uncommon • 0.4%-2% of all CNS tumors Multinodular and vacuolating neuronal tumor: MVNT MVNT • 14 entities • multinodular and vacuolating neuronal tumor • uncommon • 1st described in 2013 • 0.4%-2% of all • considered malformative or dysplastic rather than a true neoplasm CNS tumors • seizures, headache, incidental finding • ĂĚƵůƚƐшϯϬLJĞĂƌƐ • parietal and temporal lobes • MRI: – juxtacortical clusters of tiny, well-defined, round or oval-shaped nodules Multinodular and vacuolating neuronal tumor: MVNT – hyperintense on T2 and FLAIR!!! (DD with PVS) – no mass effect, no edema Diffuse glioneuronal tumor with oligodendroglial features – no enhancement (3D TSE black-blood sequence) Myxoid glioneuronal tumor Polymorphous low-grade neuroepithelial tumor of the young: PLNTY • ‘leave me alone’ lesions MVNT 45-year-old woman, tinnitus, MR post fossa • multinodular and vacuolating neuronal tumor • 1st described in 2013 • considered malformative or dysplastic rather than a true neoplasm • seizures, headache, incidental finding • ĂĚƵůƚƐшϯϬLJĞĂƌƐ • parietal and temporal lobes • MRI: – juxtacortical clusters of tiny, well-defined, round or oval-shaped nodules – hyperintense on T2 and FLAIR!!! (DD with PVS) – no mass effect, no edema – no enhancement (3D TSE black-blood sequence) • ‘leave me alone’ lesions patientpa t ooff 882,2, dysphagiadysph overview • introduction • classification • dysembryoplastic neuroepithelial tumor (DNET) • ganglioglioma (GG) • central neurocytoma • potpourri… introduction classification neuronal & mixed neuronal-glial tumors • supratentorial parenchymal, incidentally infratentorial • aka glioneuronal tumors – dysembryoplastic neuroepithelial tumor (DNET) • varying morphologic patterns and biological behavior – ganglioglioma (GG), gangliocytoma • less common than pure glial neoplasms, accounting for 0.5%-2% of all – desmoplastic infantile ganglioglioma (DIG) primary brain tumors – papillary glioneural tumor • often seizures-associated • less biologically aggressive than most glial tumors • supratentorial intraventricular, incidentally extra-ventricular • have a more favorable prognosis – central neurocytoma & extraventricular neurocytoma remember: • WHO grade 1 or 2 -location -location -location Osborn AG. Osborn’s brain Goraj B. The Neuroradiology Journal 2008 classification • infratentorial intraventricular – rosette-forming glioneuronal tumor of the 4th ventricle dysembryoplastic neuroepithelial tumor • infratentorial parenchymal – dysplastic gangliocytoma of the cerebellum: WHO, grade 1 = Lhermitte – Duclos disease remember: – cerebellar liponeurocytoma -location -location Goraj B. The Neuroradiology Journal 2008 -location dysembryoplastic neuroepithelial tumor : DNET dysembryoplastic neuroepithelial tumor : DNET imaging • benign masses of the cerebral cortex or deep gray matter: temporal lobe (62%) and frontal lobe (31%) • wedge-shaped cortical mass, “points" toward ventricle • children and young adults: majority of patients < 20 y • multicystic/septated "bubbly" appearance • man > woman • hyperintense on T2-wi • patients present with partial complex seizures • rim of hyperintensity on FLAIR: “bright rim sign” – in 20% of cases of medically refractory epilepsy • no enhancement; 1/3 enhance – 2nd most common tumor-associated TLE • no surrounding vasogenic edema • grows slowly, surgery usually curative • remodelling the inner table of the skull • often associated with cortical dysplasia • a congenital malformation rather than a true neoplastic lesion? Koeller KK and Henry JM. RadioGraphics 2001 Koeller KK and Henry JM. RadioGraphics 2001 th Barkovich AJ and Raybaud C. Pediatric Neuroimaging, 5 ed. Osborn AG. Osborn’s brain T1-wi T2-wi FLAIR GRE T2* rCBV T1-wi + Gd a 22-year-old woman T1-wi T2-wi FLAIR seizures T1-wi + Gd DWI ADC FLAIR hyperintense rim sign T1-wi T2-wi FLAIR T1-wi + Gd T1-wi + Gd DWI ADC DD • other LGG: – diffuse astrocytoma – oligodendroglioma a 15-year-old girl – pleomorphic xanthoastrocytoma – ganglioglioma seizures T1-wi T2-wi FLAIR T1-wi + Gd T1-wi + Gd a 15-year-old girl a 41-year-old man ER: generalized seizures, loss of consciousness T2-wi T1-wi + Gd pathology: – histology: oligodendroglioma vs DNET – FISH 1p/19q: no codeletion DNET rCBV = 1.2 T1-wi T2-wi FLAIR T1-wi + Gd DWI ADC a 50-year-old man headache oligodendroglioma, WHO, grade 2 IDH status unknown, 1p/19q codeleted FLAIR T2-wi T1-wi + Gd T1-wi T2-wi ganglion cell tumors ganglioglioma - gangliocytoma WHO, grade 1 WHO, grade 2 ganglion cell tumors ganglioglioma • well-differentiated neoplasms • contain both ganglion cells and glial elements • contain mature but dysmorphic neurons • immunoreactivity for these 2 cellular populations • most ganglion cell tumors are histologically mixed tumors that – glial fibrillary acidic protein (GFAP) + contain both ganglion cell and glial elements: gangliogliomas – synaptophysin and neurofilament protein + • glial component: – the most common glioneuronal neoplasm in the CNS – astrocytes in varying states of differentiation – are the most common cause of tumor-related temporal lobe epilepsy, – affects the biologic behavior of the tumor accounting for 40% of all cases – usually has histologic features of pilocytic astrocytoma • tumors that demonstrate exclusive ganglion cell composition are – if less differentiated (as in HGG), more aggressive behavior very rare: gangliocytomas – malignant degeneration • glial component is absent in gangliocytoma Osborn AG. Osborn’s brain Koeller KK and Henry JM. RadioGraphics 2001 ganglioglioma, WHO grade 1 or 2 ganglioglioma: GG • 0.4%-0.9% of all intracranial tumors imaging: • young patients: 80% < 30 years; peak age 10-20 years • CT: – well-circumscribed mass of low density • predilection for cerebral hemispheres: – located in the cortical gray matter – temporal lobe 75% – little mass effect or edema – frontal lobe – solid portions: • superficial location • isodense, hypodense, mixed • expansive growth; well-defined margins • variable contrast enhancement • calcification: 35% of cases – peritumoral edema is rare – erosion of the inner table of the calvarium Barkovich AJ and Raybaud C. Pediatric Neuroimaging, 5th ed. Koeller KK and Henry JM. RadioGraphics 2001 NCCT ganglioglioma: GG ganglioglioma: GG imaging: • MRI: variable appearance – peripheral location within the cerebral hemispheres – sharply or poorly defined margins – solid, cystic, “cyst with a mural nodule”, cluster of small cysts – variable SI on T1-wi (mixed), hyperintense ĨŽĐƵƐьĐĂůĐŝĨŝĐĂƚŝŽŶ – hyperintense on T2-wi – variable enhancement of solid portions Barkovich AJ and Raybaud C. Pediatric Neuroimaging, 5th ed. From: Koeller KK and Henry JM. RadioGraphics 2001 a 9-year-old boy T1-wi T2-wi FLAIR seizures T1-wi + Gd DWI ADC a 14-year-old boy • November 2019: – ambulance: seizures • Prev: deja-vues Alice in Wonderland Syndrome T2-wi T1-wi + Gd • difficulties in finding his words • August 2020 T1-wi + Gd fMRI-motor a 14-year-old boy a 14-year-oldy boyy NCCT 1 NCCT 1 November 2019 November 2019 2019 a 14-year-old boy a 14-year-old boy NCCT 2 August 2020 2020 a 14-year-old boy a 14-year-old boy: seizures a 11-year-old girl T1-wi T2-wi FLAIR complex partial seizures T1-wi + Gd DWI ADC a 16-year-old boy • previous medical history: CO intoxication • complex partial seizures for 3 months • clinical neurological examination: normal • EEG: normal • R/depakine 500-1000mg/day • MRI pathology: ganglioglioma vs pilocytic astrocytoma T2-wi T1-wi + Gd T2-wi FLAIR T1-wi + Gd T1-wi T1-wi + Gd GRE T2* neurosurgery • fMRI: Broca: OK • partial resection – ingrowth insula and lentiform nucleus T2-wi FLAIR T1-wi + Gd • DD: – ganglioglioma – pleomorphic xanthoastrocytoma (PXA) – (pilocytic astrocytoma) neuropathology differential diagnostic problem: • histological features of both – pleomorphic xanthoastrocytoma (PXA) – ganglioglioma • presence of ganglion cells • strong GFAP immunoreactivity: +++ • absence of reticuline network • increased proliferative activity: grade II • final diagnosis: ganglioglioma, grade II causes of temporal lobe epilepsy • most common = mesial temporal sclerosis • tumor-associated temporal lobe epilepsy – ganglioglioma (40%) central neurocytoma – DNET (20%) – diffuse low-grade astrocytoma (20%) – other (20%) WHO, grade 2 • pilocytic astrocytoma • pleomorphic xanthoastrocytoma • oligodendroglioma Osborn AG. Osborn’s brain central neurocytoma, WHO grade 2 central neurocytoma NECT NECT • young adults • WHO, grade 2 lesions • body of lateral ventricles rd • 3 and lateral ventricles • lesion attached to septum pellucidum, extending through foramen of Monro in contralateral – may arise extraventricular ventricle • young and middle-aged adults (< 40 years of age) • attached to the septum pellucidum FLAIR T2 • calcification is common • CT: - mixed density, heterogeneous cystic appearance • previously called (can be confused with) intraventricular - hydrocephalus common oligodendroglioma - calcification in 50-70%, hemorrhage rare T1 + gd T1 + gd - moderate heterogeneous enhancement • MRI: - heterogeneous mass, isointense on T1-wi - bubbly appearance on T2-wi - heterogenously hyperintense on FLAIR FLAIR T2-wi T1-wi + Gd subependymoma
Load more

Recommended publications
  • 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,
    [Show full text]
  • 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.
    [Show full text]
  • Impact of Adjuvant Radiotherapy in Patients with Central Neurocytoma: a Multicentric International Analysis
    cancers Article Impact of Adjuvant Radiotherapy in Patients with Central Neurocytoma: A Multicentric International Analysis Laith Samhouri 1,†, Mohamed A. M. Meheissen 2,3,† , Ahmad K. H. Ibrahimi 4, Abdelatif Al-Mousa 4, Momen Zeineddin 5, Yasser Elkerm 3,6, Zeyad M. A. Hassanein 2,3 , Abdelsalam Attia Ismail 2,3, Hazem Elmansy 3,6, Motasem M. Al-Hanaqta 7 , Omar A. AL-Azzam 8, Amr Abdelaziz Elsaid 2,3 , Christopher Kittel 1, Oliver Micke 9, Walter Stummer 10, Khaled Elsayad 1,*,‡ and Hans Theodor Eich 1,‡ 1 Department of Radiation Oncology, University Hospital Münster, Münster 48149, Germany; [email protected] (L.S.); [email protected] (C.K.); [email protected] (H.T.E.) 2 Alexandria Clinical Oncology Department, Alexandria University, Alexandria 21500, Egypt; [email protected] (M.A.M.M.); [email protected] (Z.M.A.H.); [email protected] (A.A.I.); [email protected] (A.A.E.) 3 Specialized Universal Network of Oncology (SUN), Alexandria 21500, Egypt; [email protected] (Y.E.); [email protected] (H.E.) 4 Department of Radiotherapy and Radiation Oncology, King Hussein Cancer Center, Amman 11942, Jordan; [email protected] (A.K.H.I.); [email protected] (A.A.-M.) 5 Department of Pediatrics, King Hussein Cancer Center, Amman 11942, Jordan; [email protected] 6 Cancer Management and Research Department, Medical Research Institute, Alexandria University, Alexandria 21500, Egypt 7 Military Oncology Center, Royal Medical Services, Amman 11942, Jordan; [email protected] 8 Princess Iman Research Center, King Hussein Medical Center, Royal Medical Services, Amman 11942, Jordan; Citation: Samhouri, L.; Meheissen, [email protected] 9 M.A.M.; Ibrahimi, A.K.H.; Al-Mousa, Department of Radiotherapy and Radiation Oncology, Franziskus Hospital Bielefeld, A.; Zeineddin, M.; Elkerm, Y.; 33699 Bielefeld, Germany; [email protected] 10 Department of Neurosurgery, University Hospital Münster, 48149 Münster, Germany; Hassanein, Z.M.A.; Ismail, A.A.; [email protected] Elmansy, H.; Al-Hanaqta, M.M.; et al.
    [Show full text]
  • Sudden Unexpected Death from Extraventricular Neurocytoma. a Case Report and Review of the Literature
    Case Report J Forensic Sci & Criminal Inves Volume-3 Issue -1 April 2017 Copyright © All rights are reserved by Panagiotis Mylonakis DOI: 10.19080/JFSCI.2017.03.555603 Sudden Unexpected Death from Extraventricular Neurocytoma. A Case Report and Review of the Literature Panagiotis Mylonakis1, Stefanos Milias2, Dimitrios Pappas3 and Antigony Mitselou4* 1Medical Examiner’s Office of Thessaloniki, Greece 2Department of Pathology, 424 Military Hospital of Thessaloniki, Greece 3Department of Pathology, 401 Military Hospital of Athens, Greece 4Department of Forensic Pathology and Toxicology, University of Ioannina, Greece Submission: April 06, 2017; Published: April 19, 2017 *Corresponding author: Panagiotis Mylonakis, MD, Medical Examiner’s Office of Thessaloniki, Thessaloniki 54012, P.O.BOX: 19757, Greece, Tel: Email: Abstract Fatal brain tumors are often diagnosed well before death. Rarely, they are associated to sudden and unexpected death and encountered in medico legal autopsy practice. Neurocytomas are unusual neuronal tumors especially affecting young people and commonly arise in the ventricles with a benign outcome. Currently, these tumors have been well recognized outside the limits of the cerebral ventricules and in these unexpectedly due to a previously undiagnosed extra ventricular neurocytoma. instances, have been called “exta ventricular neurocytomas” (EVNs). The authors present the case of a 35 year-old male who died suddenly and Keywords: Brain tumors; Neurocytoma; Extra ventricular neurocytoma; Sudden death; Autopsy Introduction due to a clinically undiagnosed extra ventricular neurocytoma Central neurocytomas (CNs) are benign tumors which located on the midbrain. usually arise from the lateral ventricles [1,2]. Extra ventricular neurocytomas (EVNs) refer to tumors with similar or identical Case Report biological and histopathological characteristics to CNs, but History which arise from extra ventricular parenchymal tissue [2].
    [Show full text]
  • Low-Grade Central Nervous System Tumors
    Neurosurg Focus 12 (2):Article 1, 2002, Click here to return to Table of Contents Low-grade central nervous system tumors M. BEATRIZ S. LOPES, M.D., AND EDWARD R. LAWS, JR., M.D. Departments of Pathology (Neuropathology) and Neurological Surgery, University of Virginia Health Sciences Center, Charlottesville, Virginia Low-grade tumors of the central nervous system constitute 15 to 35% of primary brain tumors. Although this cate- gory of tumors encompasses a number of different well-characterized entities, low-grade tumors constitute every tumor not obviously malignant at initial diagnosis. In this brief review, the authors discuss the pathological classification, diagnostic procedures, treatment, and possible pathogenic mechanisms of these tumors. Emphasis is given in the neu- roradiological and pathological features of the several entities. KEY WORDS • glioma • astrocytoma • treatment outcome Low-grade gliomas of the brain represent a large pro- toses. The pilocytic (juvenile) astrocytoma is a character- portion of primary brain tumors, ranging from 15 to 35% istic, more circumscribed lesion occurring primarily in in most reported series.1–5 They include a remarkable di- childhood and with a predilection for being located in the versity of lesions, all of which have been lumped together cerebellum. It usually appears as a cystic tumor with a under the heading of "low-grade glioma." This category mural nodule. The tumor tissue itself may have features of includes virtually every tumor of glial origin that is not microcystic degeneration and Rosenthal fibers which are overtly malignant at the time of initial diagnosis. degenerative structures in the astrocytic processes. Other reasonably common types of low-grade gliomas include CLASSIFICATION OF GLIOMAS the low-grade oligodendroglioma and the low-grade ependymoma, which is usually anatomically related to the Table 1 provides a classification of low-grade tumors of ventricular ependymal lining.
    [Show full text]
  • Gamma Knife Radiosurgery As a Primary Treatment for Central Neurocytoma
    CLINICAL ARTICLE J Neurosurg 134:1459–1465, 2021 Gamma Knife radiosurgery as a primary treatment for central neurocytoma Chiman Jeon, MD, Kyung Rae Cho, MD, Jung Won Choi, MD, PhD, Doo-Sik Kong, MD, PhD, Ho Jun Seol, MD, PhD, Do-Hyun Nam, MD, PhD, and Jung-Il Lee, MD, PhD Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea OBJECTIVE This study was performed to evaluate the role of Gamma Knife radiosurgery (GKRS) as a primary treat- ment for central neurocytomas (CNs). METHODS The authors retrospectively assessed the treatment outcomes of patients who had undergone primary treat- ment with GKRS for CNs in the period between December 2001 and December 2018. The diagnosis of CN was based on findings on neuroimaging studies. The electronic medical records were retrospectively reviewed for additional rel- evant preoperative data, and clinical follow-up data had been obtained during office evaluations of the treated patients. All radiographic data were reviewed by a dedicated neuroradiologist. RESULTS Fourteen patients were treated with GKRS as a primary treatment for CNs in the study period. Seven pa- tients (50.0%) were asymptomatic at initial presentation, and 7 (50.0%) presented with headache. Ten patients (71.4%) were treated with GKRS after the diagnosis of CN based on characteristic MRI findings. Four patients (28.6%) initially underwent either stereotactic or endoscopic biopsy before GKRS. The median tumor volume was 3.9 cm3 (range 0.46–18.1 cm3). The median prescription dose delivered to the tumor margin was 15 Gy (range 5.5–18 Gy).
    [Show full text]
  • Central Neurocytoma: Case Report and Review of Literature
    Curr Neurobiol 2017; 8 (1): 10-14 ISSN 0975-9042 Central Neurocytoma: Case Report and Review of Literature Babak Abdolkarimi1*, Soheila Zareifar2, Fazl Saleh3, Mansureh Shokripoor4 1Pediatric Hematology/Oncology assistant professor, Lorestan University of Medical Sciences. khoramabad, Iran. 2Hematology Research Center, Pediatric Hematology/Oncology Department, Amir oncology Hospital, Shiraz University of Medical Sciences. Shiraz, Iran. 3Pediatric Hematology/Oncology assistant professor, Hormozgan University of Medical Sciences. Bandarabbas, Iran. 4Pathology department, Shiraz University of Medical Sciences. Shiraz, Iran. Abstract Central neurocytoma is a rare intra-ventricular brain tumor that affects young adults and presents with increased intracranial pressure secondary to obstructive hydrocephalus. Usually, it has a good prognosis after sufficient surgical intervention, but in some patients the clinical course is more invasive. In this report, we report a case of childhood central neurocytoma with focusing on incidence and chemotherapy treatment at our oncology center. Keywords: Central neurocytoma, Pediatric, Brain tumor. Accepted January 30, 2017 Introduction Case Presentation Central neurocytoma is an extremely rare benign tumor The patient was a 3.5-year-old Afganian boy resident in that arise most of the times in the lateral ventricles near the Fars province in Iran was admitted to Namazi hospital in Monro foramina ( 0.1 - 0.5% of all primary brain tumors). Shiraz with headache, nausea and vomiting that had lasted Also this entity is rarer in pediatrics compared with adult. for 21 days before admission (Figure 1). It was first explained in 1982 by Hassoun and ws arranged In physical examination, bilateral papilledema was as WHO grade II tumors [1,2,3]. noted, without any neurological deficits.
    [Show full text]
  • Central Neurocytoma: a Review of Clinical Management and Histopathologic Features
    Brain Tumor Res Treat 2016;4(2):49-57 / pISSN 2288-2405 / eISSN 2288-2413 REVIEW ARTICLE http://dx.doi.org/10.14791/btrt.2016.4.2.49 Central Neurocytoma: A Review of Clinical Management and Histopathologic Features Seung J. Lee1, Timothy T. Bui1, Cheng Hao Jacky Chen1, Carlito Lagman1, Lawrance K. Chung1, Sabrin Sidhu1, David J. Seo1, William H. Yong3, Todd L. Siegal4, Minsu Kim5, Isaac Yang1,2 1Department of Neurosurgery, University of California, Los Angeles, Los Angeles, CA, USA 2Jonsson Comprehensive Cancer Center, University of California, Los Angeles, Los Angeles, CA, USA 3Department of Pathology & Laboratory Medicine, University of California, Los Angeles, Los Angeles, CA, USA 4Department of Radiology, Division of Neuroradiology, Cooper University Hospital, Camden, NJ, USA 5Department of Neurosurgery, Yeungnam University College of Medicine, Daegu, Korea Central neurocytoma (CN) is a rare, benign brain tumor often located in the lateral ventricles. CN may Received September 1, 2016 cause obstructive hydrocephalus and manifest as signs of increased intracranial pressure. The goal of Revised September 21, 2016 treatment for CN is a gross total resection (GTR), which often yields excellent prognosis with a very high Accepted September 21, 2016 rate of tumor control and survival. Adjuvant radiosurgery and radiotherapy may be considered to im- Correspondence prove tumor control when GTR cannot be achieved. Chemotherapy is also not considered a primary Isaac Yang treatment, but has been used as a salvage therapy. The radiological features of CN are indistinguishable Department of Neurosurgery, from those of other brain tumors; therefore, many histological markers, such as synaptophysin, can be University of California, Los Angeles, very useful for diagnosing CNs.
    [Show full text]
  • Pediatric Supratentorial Intraventricular Tumors
    Neurosurg Focus 10 (6):Article 4, 2001, Click here to return to Table of Contents Pediatric supratentorial intraventricular tumors DANIEL Y. SUH, M.D., PH.D., AND TIMOTHY MAPSTONE, M.D. Department of Neurosurgery, Emory University School of Medicine, and the Children’s Healthcare of Atlanta-Egleston, Atlanta, Georgia A variety of mass lesions can arise within or in proximity to the ventricular system in children. These lesions are relatively uncommon, and they present a unique diagnostic and surgical challenge. The differential diagnosis is deter- mined by tumor location in the ventricular system, clinical presentation, age of the patient, and the imaging character- istics of the lesion. In this report the authors provide an introduction to and an overview of the most common pediatric supratentorial intraventricular tumors. The typical radiographic features of each tumor and location preference within the ventricular system are reviewed. Management and treatment considerations are discussed. Examination of tissue samples to obtain diagnosis is usually required for accurate treatment planning, and resection without adjuvant thera- pies is often curative. The critical management decision frequently involves determining which lesions are appropri- ate for surgical therapy. Care ful preoperative neuroimaging is extremely useful in planning surgery. Knowledge of the typical imaging characteristics of these tumors can help to determine the diagnosis with relative certainty when a tis- sue sample has not been obtained, because a small subset of these lesions can be managed expectantly. KEY WORDS • intraventricular tumor • pediatric tumor • brain lesion • hydrocephalus One tenth of all CNS neoplasms present within or in certain anatomical locations and in certain age groups.61,75, proximity to the ventricular system.85 These neoplasms 80,85,92,124,133 Table 1 provides a list of common pediatric comprise a heterogeneous group with regard to tumor type intraventricular tumors by ventricular location.
    [Show full text]
  • January 2020
    Fred Hutch Cancer Surveillance System January, 2020 REGISTRAR PIP Visit SEER*Educate: A comprehensive training platform for registry professionals Clinical Grade for CNS Primaries Using the WHO Grading System for Selected Tumors of the CNS Mary Trimble, CTR If we have a diagnostic biopsy of a tumor, we can code the clinical grade regardless of the primary site. What is odd about Central Nervous System (CNS) tumors (Figures 1 and 2) is that we can code a clinical grade in the absence of a diagnostic biopsy or histologic confirmation for these primaries. For any other primary, we must have histologic or cytologic confirmation in order to code grade at all. So, what makes the Central Nervous System different? How is it we are able to code clinical grade without any histologic confirmation of the tumor? The World Health Organization (WHO) Classification of Tumors of the Central Nervous System, 4th Edition, released in 2016, provides the WHO grading system of CNS tumors as a uniform way to categorize CNS tumors. Figure 1 Figure 2 Lobes of the Cerebellum Pituitary and Pineal Glands American Joint CommiAee on Cancer (AJCC) Manual, 8th EdiFon - Table 72.2 This four-tiered grade scale helps to classify select CNS histologies based on proliferative potential and the risk of spread or recurrence. In other words, the WHO grade is a malignancy scale where the higher the WHO grade, the more aggressive the clinical course expected for the tumor.” This WHO classification can be seen as part of a larger clinical definition for select histologies and can therefore be used to assign grade without histologic confirmation.
    [Show full text]
  • Neuroimaging Findings in Septal Dysembryoplastic
    BRAIN ABBREVIATION KEY DNET ϭ dysembryoplastic neuroepithelial tumor WHO ϭ World Health Organization Received August 30, 2018; accepted November 19, 2018. Neuroimaging Findings in Septal From the Departments of Neuroradiology (P.P.B., P.D., T.J.E.M., S.H.P., J.H.D.) and Dysembryoplastic Neuroepithelial Pathology (Neuropathology) (M.B.L.), University of Virginia Health System, Charlottesville, Tumor: Why Is It Important to Virginia. Previously presented as electronic Recognize This Entity? educational exhibit at the 56th Annual Meeting of the American Society of Neuroradiology, June P.P. Batchala, P. Darvishi, T.J. Eluvathingal Muttikkal, S.H. Patel, M.B. Lopes, and 2–7, 2018, Vancouver, British J.H. Donahue Columbia, Canada. Disclosures S. Patel—UNRELATED: Grants/grants pending: RSNA, Comments: RSNA scholar grant for ABSTRACT research unrelated to the current A dysembryoplastic neuroepithelial tumor like neoplasm of the septum pellucidum is a rare manuscript. entity that can be easily mistaken for more common pathologies, such as a colloid cyst, Please address correspondence to Prem P. Batchala, MD, Department central neurocytoma, or glioma involving the septum pellucidum. On histopathologic ex- of Neuroradiology, University of amination, these tumors can mimic central neurocytomas, subependymomas, or oligoden- Virginia Medical Center, 1215 Lee Street, Charlottesville, VA 22908; drogliomas. Here, we describe clinical and imaging findings in 4 patients with dysembryo- e-mail: [email protected]. plastic neuroepithelial tumor like neoplasms arising from the septum pellucidum. A http://dx.doi.org/10.3174/ng.1800044 systematic review of the literature follows. INTRODUCTION torial extracortical locations of DNET The term “dysembryoplastic neuroepithe- include the third ventricle, basal ganglia, lial tumor (DNET)” was originally coined and septum pellucidum.4,5,7 by Daumas-Duport et al1 in 1988.
    [Show full text]
  • Central Neurocytoma – Case Report
    SCIENTIA PLENA VOL. 9, NUM. 2 2013 www.scientiaplena.org.br Central neurocytoma – case report Neurocitoma central – relato de caso I. C. R. Martins 1; A. P. Percicote 2; M. A. D. Pianovski 1; M. V. R. Araki 3 1Department of Pediatric Oncology, Hospital Erastus Gaertner, 81520-060, Curitiba-PR, Brazil 2Department of pathology, Hospital Erastus Gaertner, 81520-060, Curitiba-PR, Brazil 3Federal University of Sergipe, 49100-000, São Cristóvão-SE, Brazil [email protected] (Received on June 26, 2012, accepted on February 5, 2013) Central neurocytoma presents as a tumor located in the third ventricle, destroying the anterior part of the fornix, the septum pelucidum, invading lateral ventricle. The patients may have symptoms of amnesic and behavior disturbances; headache and clinical evidence of raised intracranial pressure. Differential diagnosis must be established with ependimomas, astrocytomas, intraventricular oligodendromas or primary cerebral neuroblastomas – diagnosis is made by CT or MRI, electron microscopy and immunohistochemical methods. This case report describes a central neurocytoma in a 16 year old patient, the signs and symptoms, imaging (Computed Tomography and Magnetic Nuclear Resonance), histopathological and immunohistochemistry analysis and the treatment applied. Keywords: Central neurocytoma; CNS tumors; pediatrics O neurocitoma central se apresenta como um tumor localizado no terceiro ventrículo, destruindo a porção anterior do fórnix, o septo pelúcido, invadindo o ventrículo lateral. Os pacientes podem apresentar sintomas como distúrbios amnésicos e de comportamento, dor de cabeça e evidência clinica de pressão intracraniana elevada. O diagnóstico diferencial deve ser estabelecido com ependimomas, astrocitomas, oligodendromas intraventriculares ou neuroblastomas cerebrais primários – o diagnóstico é estabelecido por tomografia computadorizada ou ressonância nuclear magnética, microscopia eletrônica e métodos de imuno-histoquimica.
    [Show full text]