Multifocal Medulloblastoma in an Adult Patient: Description of a Rare Presentation and Review of the Literature.

IF 0.7 Q4 PATHOLOGY
Case Reports in Pathology Pub Date : 2020-09-11 eCollection Date: 2020-01-01 DOI:10.1155/2020/4502878
Irene Troncon, Angela Guerriero, Sabrina Rossi, Monica Ronzon, Marta Padovan, Caccese Mario, Lucia Zanatta, Luisa Toffolatti, Elisabetta Marton, Giuseppe Lombardi, Angelo Paolo Dei Tos, Giuseppe Canova
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引用次数: 1

Abstract

Medulloblastoma is an embryonal neuroepithelial tumor that affects mainly childhood and more rarely adults. Medulloblastoma occurring as multiple nodules at diagnosis is a rare and tricky presentation. Here, we describe the case of a previously healthy 47-year-old woman with multiple posterior fossa cerebellar tumors. Histological, immunohistochemical, and molecular analyses were performed to best characterize the two excised lesions. The histopathological analysis revealed different variants of medulloblastoma in the excised nodules, one being extensive nodularity, rare in adults, and the other desmoplastic/nodular with areas of anaplasia. Immunostains and molecular analysis classified both nodules as SHH medulloblastoma. Adult medulloblastoma is extremely rare. Important differences exist between adult medulloblastoma and medulloblastoma arising in children and infants. Such differences are in location, distribution of histological variants and of molecular subgroups, survival rates, and therapeutic options. An extensive morphological and molecular characterization of such rare tumors is necessary to choice the best-tailored therapy.

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一例成人多灶性髓母细胞瘤:罕见病例描述及文献回顾。
髓母细胞瘤是一种胚胎性神经上皮肿瘤,主要影响儿童,很少影响成人。髓母细胞瘤在诊断时表现为多发结节是一种罕见且棘手的表现。在这里,我们描述一个先前健康的47岁女性患有多发性后窝小脑肿瘤的病例。组织学,免疫组织化学和分子分析进行了最好的表征两个切除病变。组织病理学分析显示,在切除的结节中,髓母细胞瘤有不同的变体,一种是广泛的结节性,在成人中很少见,另一种是伴有间变性的结缔组织增生/结节性。免疫染色和分子分析将这两个结节归类为SHH髓母细胞瘤。成人成神经管细胞瘤极为罕见。成人成神经管细胞瘤与发生于儿童和婴儿的成神经管细胞瘤存在重要差异。这些差异体现在位置、组织变异和分子亚群的分布、存活率和治疗选择上。对这种罕见肿瘤进行广泛的形态学和分子表征是选择最佳治疗方法的必要条件。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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12 weeks
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