Journal Article10.1111/NEUP.12325
Meningeal Ewing Sarcoma/Peripheral PNET: Clinicopathological, Immunohistochemical and FISH study of four cases.
Changshu Ke,Qiuhong Duan,Hui Yang,Feng Zhu,Meng Yan,Sanpeng Xu,Sheng Zhou,Feng Wan,Kai Shu,Ting Lei,Li-ming Xia +10 more
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TL;DR: Results suggest that using a panel of immunohistochemical markers, including NKX2.2, CD99, FLI‐1, EMA, GFAP and synaptophysin, combined with the supplementary EWSR1 FISH test, helps to define the diagnosis of meningeal ES/pPNET of CNS.
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Abstract: Meningeal Ewing Sarcoma (ES)/peripheral primitive neuroectodermal tumor (pPNET) is a rare diagnostically challenging small round cell tumor in the CNS. This study investigates the clinical pathological features of four cases of this tumor from archives of 6 years in our hospital. Patients were within the median age of 21.5 years and male to female ratio was 1:1. The tumors distributed at the supra-tentorial location, posterior fossa and lumbar vertebral canal, usually presenting as the dura-sited nodule or having close connection with the meninges within the cranium or vertebral canal. Histopathologically, small round undifferentiated tumor cells with hypercellularities, scant cytoplasm and inconspicuous nucleoli were observed, although some components such as atypical larger vesicular nuclei, prominent nucleoli of tumor cells, necrotic foci and mesenchymal collagen proliferation forming the lobular structure, were also appreciated. Immunohistochemally, tumor cells displayed membranous positivity of CD99 (4/4), nuclear positivity of FLI-1 (4/4) and NKX2.2 (4/4), negativity of EMA, GFAP and synaptophysin expression. The histochemical PAS staining showed weak positivity in one case. Fluorescence in situ hybridization (FISH) test using EWSR1 (22q12) dual color break apart rearrangement probe showed positive results in two cases. Results suggest that using a panel of immunohistochemical markers, including NKX2.2, CD99, FLI-1, EMA, GFAP and synaptophysin, combined with the supplementary EWSR1 FISH test, helps to define the diagnosis of meningeal ES/pPNET of CNS.
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Citations
Clinical Features and Long-Term Outcome of Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumors: 14 Cases From a Single Institution.
Jun Chen,Qian Jiang,Yu Zhang,Yufei Yu,Yifeng Zheng,Juan Chen,Yiqing Zhao,Zhuangzhuang Miao,Fanfan Fan,Yu Wang +9 more
TL;DR: Primary intracranial ES/pPNETs have an aggressive clinical course, with a high tendency for local recurrence and distant metastasis, and postoperative radiotherapy was a significant prognostic factor for longer MST.
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Ewing Sarcoma With Emphasis on Extra-skeletal Ewing Sarcoma: A Decade's Experience From a Single Centre in India.
Sudha S Murthy,Sundaram Challa,Kvvn Raju,Senthil Rajappa,Daphne Fonseca,Sandhya Devi Gundimeda,B V Rao,Faiq Ahmed,Suseela Kodandapani,Lavanya Nambaru,Manasi C. Mundada,Rakesh Sharma,Krishna Mohan Mallavarapu,Veeraiah Koppula,T. S. Rao +14 more
- 11 Nov 2020
TL;DR: Molecular tests were performed more frequently in extra-skeletal tumours, especially in visceral tumours to establish the diagnosis of Ewing sarcoma family of tumours with emphasis on sex, age, and location.
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Primary and Metastatic Intracranial Ewing Sarcoma at Diagnosis: Retrospective International Study and Systematic Review.
Lianne M. Haveman,Andreas Ranft,Henk van den Berg,Stephanie Klco-Brosius,Ruth Ladenstein,Michael Paulussen,Heribert Juergens,Uta Dirksen,Johannes H. M. Merks +8 more
TL;DR: Primary intracranial EwS and extracranial ewing sarcoma with cerebral metastases at diagnosis is rare, yet survival is comparable with local and metastatic EWS elsewhere in the body.
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Treatment Outcomes and Prognostic Factors of Patients With Primary Spinal Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumors.
TL;DR: Total en bloc resection and adjuvant radiotherapy considerably improve OS for patients with primary spinal ES/pPNETs and adjutant radiotherapy was a favorable factor for PFS.
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References
Small supratentorial, extraaxial primitive neuroectodermal tumor causing large intracerebral hematoma.
Jan-Karl Burkhardt,Ralf A. Kockro,Hildegard Dohmen-Scheufler,Christoph M. Woernle,David Bellut,Spyros Kollias,Helmut Bertalanffy +6 more
TL;DR: This case represents an important example of atypical extraaxial appearance of thisLesion, which should be considered in the differential diagnosis of cortical or subcortical hemorrhage, since complete resection of this lesion is critical for the successful treatment and outcome.
MIC2 is a specific marker for Ewing's sarcoma and peripheral primitive neuroectodermal tumors. Evidence for a common histogenesis of Ewing's sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration.
Inge M. Ambros,Peter F. Ambros,Sabine Strehl,Heinrich Kovar,Helmut Gadner,Mechthild Salzer-Kuntschik +5 more
TL;DR: Both ES and pPNET cells express the MIC2 gene in very high amounts, which represents a highly selective and almost unique feature of these cells, making an assignment of these tumors in one entity even more likely.