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000131488 1001_ $$aAppay, Romain$$b0
000131488 245__ $$aHGNET-BCOR Tumors of the Cerebellum: Clinicopathologic and Molecular Characterization of 3 Cases.
000131488 260__ $$aPhiladelphia, Pa.$$bLippincott Williams & Wilkins$$c2017
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000131488 520__ $$aThe central nervous system (CNS) high-grade neuroepithelial tumor with BCOR alteration (CNS HGNET-BCOR) is a recently described molecular entity. We report 3 new CNS HGNET-BCOR cases sharing common clinical presentation and pathologic features. The 3 cases concerned children aged 3 to 7 years who presented with a voluminous mass of the cerebellum. Pathologic features included proliferation of uniform spindle to ovoid cells with fine chromatin associated with a rich arborizing capillary network. Methylation profiling classified these cases as CNS HGNET-BCOR tumors. Polymerase chain reaction analysis confirmed the presence of internal tandem duplications in the C-terminus of BCOR (BCOR-ITD), a characteristic of these tumors, in all 3 cases. Immunohistochemistry showed a strong nuclear BCOR expression. In 2 cases, local recurrence occurred within 6 months. The third case, a patient who received a craniospinal irradiation after total surgical removal followed by a metronomics maintenance with irinotecan, temozolomide, and itraconazole, is still free of disease 14 months after diagnosis. In summary, CNS HGNET-BCOR represents a rare tumor occurring in young patients with dismal prognosis. BCOR nuclear immunoreactivity is highly suggestive of a BCOR-ITD. Whether CNS HGNET-BCOR should be classified among the category of 'embryonal tumors' or within the category of 'mesenchymal, nonmeningothelial tumors' remains to be clarified. Because CNS HGNET-BCOR share pathologic features and characteristic BCOR-ITD with clear cell sarcoma of the kidney, these tumors may represent local variants of the same entity.
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000131488 650_7 $$2NLM Chemicals$$aBCOR protein, human
000131488 650_7 $$2NLM Chemicals$$aProto-Oncogene Proteins
000131488 650_7 $$2NLM Chemicals$$aRepressor Proteins
000131488 7001_ $$aMacagno, Nicolas$$b1
000131488 7001_ $$aPadovani, Laetitia$$b2
000131488 7001_ $$0P:(DE-He78)8d9c904a6cea14d4c99c78ba46e41f93$$aKorshunov, Andrey$$b3$$udkfz
000131488 7001_ $$0P:(DE-He78)4c28e2aade5f44d8eca9dd8e97638ec8$$aKool, Marcel$$b4$$udkfz
000131488 7001_ $$aAndré, Nicolas$$b5
000131488 7001_ $$aScavarda, Didier$$b6
000131488 7001_ $$aPietsch, Torsten$$b7
000131488 7001_ $$aFigarella-Branger, Dominique$$b8
000131488 773__ $$0PERI:(DE-600)2029143-7$$a10.1097/PAS.0000000000000866$$gVol. 41, no. 9, p. 1254 - 1260$$n9$$p1254 - 1260$$tThe @American journal of surgical pathology$$v41$$x0147-5185$$y2017
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