DOCSLIB.ORG

Neuroimaging Findings in Septal Dysembryoplastic

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read 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. It was Septal DNETs, like their cortical coun- described as a predominantly supratento- terparts, are World Health Organization rial, cortical-based tumor with multinodu- (WHO) grade I neoplasms. But, unlike lar architecture and cellular heterogeneity cortical DNETs, they mostly present with in 39 patients with medically intractable obstructive symptoms rather than sei- partial complex seizures.1 DNETs typically zures.4 On imaging, septal DNET can have mixed glial and neuronal elements on mimic more common neoplastic and non- histology.1 Typical imaging findings in- neoplastic entities that can arise from the clude a hypoattenuated lesion on CT that septum pellucidum or the foramen of shows T1-weighted hypointensity, T2- Monro. Neoplastic mimics include central weighted hyperintensity, and a characteris- neurocytomas, subependymomas, intra- tic FLAIR hyperintense peripheral rim.2 ventricular meningiomas, and low-grade Since the original case review in 1988, our gliomas, whereas nonneoplastic mimics in- understanding of DNET has evolved. It has clude parasitic cysts (eg, neurocysticerco- been documented that DNETs can occur sis) and colloid cysts. Herein, we describe elsewhere than the typical cortical loca- the clinical and imaging findings in 4 pa- tion.3-8 DNETs can be multifocal, can in- tients with septal DNET from our institu- crease in size during follow-up imaging, tion with histologic confirmation in 3. In with occasional recurrence after resec- addition, we present a systematic literature tion, and, also, very rarely, can undergo review of septal DNET and describe imag- malignant transformation after radiation ing findings of more common mimics of therapy.9-11 The less common supraten- septal DNET. 212 ͉ Neurographics 2019 May/June;9(3):212–219; www.neurographics.org Fig 1. Surgically proved septal DNET. A 6-year-old girl presented with obstructive hydrocephalus. A, Axial CT shows a nearly imperceptible left lateral ventricle hypoattenuated septal DNET (arrow) close to the foramen of Monro, causing left lateral ventricle outflow obstruction. B, Axial T2WI shows the lesion to be homogeneously hyperintense (arrow), blending with the CSF background. C, Axial contrast-enhanced T1WI shows the absence of enhancement in the septal DNET (arrow). D, Axial ADC demonstrates facilitated diffusion in the lesion (arrow). E, Axial FLAIR better delineates the septal lesion (arrow) compared with other MR images and CT. Fig 2. Septal DNET recurrence 1 year after gross total resection. A, A high-resolution axial T2 sequence better delineates the walls of the hyperintense intraventricular mucoid recurrent septal tumor (arrow). B, Axial FLAIR sequence, showing a hyperintense rim around the recurrent tumor (arrow), a finding also previously reported with cortical DNET. C, Coronal contrast-enhanced T1WI, revealing the relation of the recurrent tumor to the foramen of Monro (arrow). A small, postsurgical, interhemispheric subdural collection is also present (white star). PATIENT 1 A 6-year-old girl presented with headaches. An initial CT was suspicious for a septal mass that was causing obstruc- tive hydrocephalus. On MR imaging, a cystic lesion was evident at the foramen of Monro, initially reported as a possible atypical colloid cyst (in view of the absence of typical CT hyperattenuation) or a parasitic or glioependy- mal cyst (Fig 1). The patient underwent gross total resection with histopathology that showed a low-grade glioma. Fol- Fig 3. Surgically proved septal DNET. A 22-year-old woman with ob- structive hydrocephalus. A, Axial T2WI, showing an elliptical, hyperin- low-up imaging after a year demonstrated recurrence (Fig tense septal DNET located between the leaves of the anterior inferior 2). Histopathologic examination of the repeated resection septum (arrow). B, Axial FLAIR sequence reveals a hyperintense rim was characteristic for a septal DNET-like lesion. The pa- around the centrally hypointense septal DNET (arrow). A correct preop- tient’s postoperative course was complicated by pituitary erative diagnosis was made in this case. insufficiency and persistent hydrocephalus that required a ventriculoperitoneal shunt. There was no recurrence in the lucidum. There was no evidence of progression in the 6-year 9-year follow-up after the second resection. follow-up. PATIENT 2 PATIENT 3 A 22-year-old woman presented with acute-onset severe A 14-year-old girl presented with acute-onset headaches, headaches, nausea, and emesis. MR imaging revealed a sep- nausea, and vomiting. MR imaging revealed an intraven- tal mass that caused obstructive hydrocephalus (Fig 3), tricular mass in the left lateral ventricle along the septum and a preoperative diagnosis of septal DNET was made. pellucidum, which caused obstructive hydrocephalus and The patient underwent subtotal resection with histology was reported as a colloid cyst (Fig 4). The patient under- consistent with a DNET-like neoplasm of the septum pel- went gross total resection with histopathology initially re- Neurographics 2019 May/June;9(3):212–219; www.neurographics.org ͉ 213 Fig 4. Surgically proved septal DNET. A 14-year-old girl presented with Fig 6. Septal DNET. A, Low-magnification (40x) and (B) high-magnifica- obstructive hydrocephalus. A, Axial T2WI, showing a homogeneously tion (100x) images, showing oligodendrocyte like cells (large arrows) hyperintense, rounded septal DNET in the left lateral ventricle attached aligned in columns in a background of mucoid matrix with floating neu- to the anterior-inferior aspect of the septum pellucidum (arrow). B, Axial rons (small arrow) (H&E). Cortical DNET shows similar findings. FLAIR sequence reveals a homogeneously hypointense tumor (arrow). There is no hyperintense FLAIR rim in this patient. The initial histopatho- logic diagnosis was low-grade glioma in this patient, and a local recur- septum pellucidum is a relatively preferred site. The other rence (not shown) after 2 years was treated by gamma knife. A retro- reported sites are the basal ganglia, third ventricle, brain spective review of the histopathology was consistent with a DNET-like 5,7,8 neoplasm of the septum pellucidum. stem, and cerebellum. A systematic review of the Eng- lish literature conducted using PubMed and internet search identified 11 peer-reviewed publications that detailed 30 patients with DNET that involved the septum pellucidum near the foramen of Monro, which, in conjunction with our 4 reported patients, yielded a total of 34 patients (Table 1).3-5,9,12-19 In a large series of 51 DNETs, only 2 patients had lesions that occurred within the ventricles.9 Baisden et al4 reported the single largest series of septal DNET in the English literature to date, with 10 patients. In contradistinction to cortical DNETs, patients with Fig 5. MRI, suggestive of a septal DNET. A 62-year-old woman with head- septal lesions overwhelmingly presented with headache or ache. A, Axial T2WI, showing a homogeneous T2-hyperintense tumor splaying the septal leaves (arrow), an appearance similar to the patient other obstructive symptoms, with only 3 patients who pre- illustrated in Fig 3. B, A hyperintense rim is present around the tumor in sented with seizure and 3 tumors identified incidentally. this axial FLAIR image (arrow). The tumor remained stable for 6 months, The mean age of diagnosis was 22.6 years (range, 3–62 and the patient remains on a serial follow-up protocol. years) and the male-to-female ratio was 10 to 23. The mean imaging follow-up among the 17 patients for whom fol- ported as a low-grade glioma (oligodendroglioma versus low-up was reported was 4.1 years, during which time 3 oligoastrocytoma).
Load more

Recommended publications
  • Intraventricular Neuroepithelial Tumors: Surgical Outcome, Technical Considerations and Review of Literature A
    Aftahy et al. BMC Cancer (2020) 20:1060 https://doi.org/10.1186/s12885-020-07570-1 RESEARCH ARTICLE Open Access Intraventricular neuroepithelial tumors: surgical outcome, technical considerations and review of literature A. Kaywan Aftahy1* , Melanie Barz1, Philipp Krauss1, Friederike Liesche2, Benedikt Wiestler3, Stephanie E. Combs4,5,6, Christoph Straube4, Bernhard Meyer1 and Jens Gempt1 Abstract Background: Intraventricular neuroepithelial tumors (IVT) are rare lesions and comprise different pathological entities such as ependymomas, subependymomas and central neurocytomas. The treatment of choice is neurosurgical resection, which can be challenging due to their intraventricular location. Different surgical approaches to the ventricles are described. Here we report a large series of IVTs, its postoperative outcome at a single tertiary center and discuss suitable surgical approaches. Methods: We performed a retrospective chart review at a single tertiary neurosurgical center between 03/2009–05/ 2019. We included patients that underwent resection of an IVT emphasizing on surgical approach, extent of resection, clinical outcome and postoperative complications. Results: Forty five IVTs were resected from 03/2009 to 05/2019, 13 ependymomas, 21 subependymomas, 10 central neurocytomas and one glioependymal cyst. Median age was 52,5 years with 55.6% (25) male and 44.4% (20) female patients. Gross total resection was achieved in 93.3% (42/45). 84.6% (11/13) of ependymomas, 100% (12/21) of subependymomas, 90% (9/10) of central neurocytomas and one glioependymal cyst were completely removed. Postoperative rate of new neurological deficits was 26.6% (12/45). Postoperative new permanent cranial nerve deficits occurred in one case with 4th ventricle subependymoma and one in 4th ventricle ependymoma.
    [Show full text]
  • 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
    [Show full text]
  • 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]
  • Seizure Prognosis of Patients with Low-Grade Tumors
    View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector Seizure 21 (2012) 540–545 Contents lists available at SciVerse ScienceDirect Seizure jou rnal homepage: www.elsevier.com/locate/yseiz Seizure prognosis of patients with low-grade tumors a b,c c,d c,e Cynthia A. Kahlenberg , Camilo E. Fadul , David W. Roberts , Vijay M. Thadani , c,e a,f c,e, Krzysztof A. Bujarski , Rod C. Scott , Barbara C. Jobst * a Dartmouth College, Hanover, NH, United States b Sections of Hematology, Oncology and Neurology, Dartmouth Hitchcock Medical Center, Lebanon, NH, United States c Dartmouth Medical School, Hanover, NH, United States d Section of Neurosurgery, Department of Surgery, Dartmouth-Hitchcock Medical Center, Lebanon, NH, United States e Department of Neurology, Dartmouth-Hitchcock Medical Center, Lebanon, NH, United States f Neurosciences Unit, Institute of Child Health, University College London, Great Ormond Street Hospital for Children NHS Trust, London, UK A R T I C L E I N F O A B S T R A C T Article history: Purpose: Seizures frequently impact the quality of life of patients with low grade tumors. Management is Received 12 January 2012 often based on best clinical judgment. We examined factors that correlate with seizure outcome to Received in revised form 24 May 2012 optimize seizure management. Accepted 24 May 2012 Methods: Patients with supratentorial low-grade tumors evaluated at a single institution were retrospectively reviewed. Using multiple regression analysis the patient characteristics and treatments Keywords: were correlated with seizure outcome using Engel’s classification.
    [Show full text]
  • Clinical, Radiological, and Pathological Features in 43 Cases of Intracranial Subependymoma
    CLINICAL ARTICLE J Neurosurg 122:49–60, 2015 Clinical, radiological, and pathological features in 43 cases of intracranial subependymoma Zhiyong Bi, MD, Xiaohui Ren, MD, Junting Zhang, MD, and Wang Jia, MD Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China ObjECT Intracranial subependymomas are rarely reported due to their extremely low incidence. Knowledge about sub- ependymomas is therefore poor. This study aimed to analyze the incidence and clinical, radiological, and pathological features of intracranial subependymomas. METHodS Approximately 60,000 intracranial tumors were surgically treated at Beijing Tiantan Hospital between 2003 and 2013. The authors identified all cases in which patients underwent resection of an intracranial tumor that was found to be pathological examination demonstrated to be subependymoma and analyzed the data from these cases. RESULTS Forty-three cases of pathologically confirmed, surgically treated intracranial subependymoma were identi- fied. Thus in this patient population, subependymomas accounted for approximately 0.07% of intracranial tumors (43 of an estimated 60,000). Radiologically, 79.1% (34/43) of intracranial subependymomas were misdiagnosed as other dis- eases. Pathologically, 34 were confirmed as pure subependymomas, 8 were mixed with ependymoma, and 1 was mixed with astrocytoma. Thirty-five patients were followed up for 3.0 to 120 months after surgery. Three of these patients expe- rienced tumor recurrence, and one died of tumor recurrence. Univariate analysis revealed that shorter progression-free survival (PFS) was significantly associated with poorly defined borders. The association between shorter PFS and age < 14 years was almost significant (p = 0.51), and this variable was also included in the multivariate analysis.
    [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]
  • Genomic Landscape of Intramedullary Spinal Cord Gliomas Ming Zhang1,10, Rajiv R
    www.nature.com/scientificreports OPEN Genomic Landscape of Intramedullary Spinal Cord Gliomas Ming Zhang1,10, Rajiv R. Iyer2,10, Tej D. Azad2,3,10, Qing Wang1, Tomas Garzon-Muvdi2,4, Joanna Wang5, Ann Liu2, Peter Burger6, Charles Eberhart6, Fausto J. Rodriguez 6, Daniel M. Sciubba2, Jean-Paul Wolinsky2,7, Ziya Gokaslan2,8, Mari L. Groves2, George I. Jallo2,9* & Chetan Bettegowda1,2* Intramedullary spinal cord tumors (IMSCTs) are rare neoplasms that have limited treatment options and are associated with high rates of morbidity and mortality. To better understand the genetic basis of these tumors we performed whole exome sequencing on 45 tumors and matched germline DNA, including twenty-nine spinal cord ependymomas and sixteen astrocytomas. Though recurrent somatic mutations in IMSCTs were rare, we identifed NF2 mutations in 15.7% of tumors (ependymoma, N = 7; astrocytoma, N = 1), RP1 mutations in 5.9% of tumors (ependymoma, N = 3), and ESX1 mutations in 5.9% of tumors (ependymoma, N = 3). We further identifed copy number amplifcations in CTU1 in 25% of myxopapillary ependymomas. Given the paucity of somatic driver mutations, we further performed whole-genome sequencing of 12 tumors (ependymoma, N = 9; astrocytoma, N = 3). Overall, we observed that IMSCTs with intracranial histologic counterparts (e.g. glioblastoma) did not harbor the canonical mutations associated with their intracranial counterparts. Our fndings suggest that the origin of IMSCTs may be distinct from tumors arising within other compartments of the central nervous system and provides the framework to begin more biologically based therapeutic strategies. Intramedullary spinal cord tumors (IMSCTs) are a heterogeneous group of rare lesions that can cause signifcant morbidity in both children and adults.
    [Show full text]
  • REPORTABILITY Determining Reportability the Following
    SECTION II - REPORTABILITY Determining Reportability The following supercedes any previous MCR reportability rules appearing in our manuals. These rules attempt to cover ALL years of case reportability to the MCR, but most specifically refer to diagnoses made beginning in 2003. If there are changes needed in these rules in the future, notification will be sent to reporting facilities and software vendors and replacement pages will be issued for this manual. The MCR requires reporting facilities to submit all cases seen at that facility with neoplasms classified as invasive or in situ in the "Morphology of Neoplasms" section of ICD-O-3* (for cases diagnosed in 2001 and thereafter), ICD-O-2 (for diagnoses made between 1992 and 2000), or ICD-O[-1] (for 1982-1991 diagnoses). (This MCR Manual applies to diagnoses made beginning in 2003, so only ICD-O-3 codes appear here.) If you've changed a listed behavior in an ICD-O book to /2 or /3, that case is also reportable (the "matrix" rule, ICD-O-3 Rule F). The only exceptions to these /2 and /3 reportability rules are the site/morphology combinations that follow: morphology 8000-8005 malignant neoplasms, NOS, of the skin (C44.0-C44.9) 8010-8046 epithelial carcinomas of the skin (C44.0-C44.9) 8050-8084 papillary and squamous cell carcinomas of the skin (C44.0-C44.9) 8090-8110 basal cell carcinomas of the skin (C44.0-C44.9) Note: The above morphologies of any non-C44 primary site will be reportable to the MCR as of January 1, 2004.
    [Show full text]
  • Coexistent Subependymoma and Psammomatous Meningioma
    International Medicine 2019; 1(3): 176-177 International Medicine https://www.theinternationalmedicine.com/ Clinical Image Coexistent subependymoma and psammomatous meningioma Richard A. Prayson Department of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio, USA Received: 12 April 2019 / Accepted: 10 May 2019 A 74-year-old female with a past medical history of hypertension, congestive heart failure, atrial fibrillation, and type II diabetes mellitus presented most recently with somnolence and nonresponsiveness. She had a known history of a multilobular intraventricular mass within the frontal horn and anterior body of the right lateral ventricle that was discovered on a magnetic resonance imaging (MRI) study done six and a half years earlier for complaints at that time of intermittent head pain. The intraventricular lesion was thought to represent a possible central neurocytoma. Also noted at that time were multiple extra-axial masses, presumed to be meningiomas, located at the planum sphenoidale, left clinoid, bilateral falx, and right lateral convexity. Given the patient’s age, medical risk factors, and lack of neurological symptoms and findings directly attributable to the brain masses, it was decided that surgical intervention was not warranted and close follow-up with imaging studies would be done. At the time of her most recent presentation, computed tomography (CT) showed an intraparenchymal hemorrhage in the midline region of the corpus callosum with moderate hydrocephalus and extension of the bleed into the ventricle, proximal to the intraventricular tumor. Surgery was undertaken to remove the blood clot, the intraventricular neoplasm, and a 0.5 cm calcified mass overlying the right frontal gyrus. Figure 1. Psammomatous meningioma arising over the right frontal gyrus (left) and subependymoma situated within the lateral ventricle (right) (hematoxylin and eosin, original magnifications for both images 200X).
    [Show full text]