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| Home > Publications database > HGNET-BCOR Tumors of the Cerebellum: Clinicopathologic and Molecular Characterization of 3 Cases. > print |
| 001 | 131488 | ||
| 005 | 20240228145603.0 | ||
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| 082 | _ | _ | |a 610 |
| 100 | 1 | _ | |a Appay, Romain |b 0 |
| 245 | _ | _ | |a HGNET-BCOR Tumors of the Cerebellum: Clinicopathologic and Molecular Characterization of 3 Cases. |
| 260 | _ | _ | |a Philadelphia, Pa. |c 2017 |b Lippincott Williams & Wilkins |
| 336 | 7 | _ | |a article |2 DRIVER |
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| 520 | _ | _ | |a The 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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| 650 | _ | 7 | |a BCOR protein, human |2 NLM Chemicals |
| 650 | _ | 7 | |a Proto-Oncogene Proteins |2 NLM Chemicals |
| 650 | _ | 7 | |a Repressor Proteins |2 NLM Chemicals |
| 700 | 1 | _ | |a Macagno, Nicolas |b 1 |
| 700 | 1 | _ | |a Padovani, Laetitia |b 2 |
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| 700 | 1 | _ | |a André, Nicolas |b 5 |
| 700 | 1 | _ | |a Scavarda, Didier |b 6 |
| 700 | 1 | _ | |a Pietsch, Torsten |b 7 |
| 700 | 1 | _ | |a Figarella-Branger, Dominique |b 8 |
| 773 | _ | _ | |a 10.1097/PAS.0000000000000866 |g Vol. 41, no. 9, p. 1254 - 1260 |0 PERI:(DE-600)2029143-7 |n 9 |p 1254 - 1260 |t The @American journal of surgical pathology |v 41 |y 2017 |x 0147-5185 |
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