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[成人髓母细胞瘤。17例临床、解剖及治疗研究(作者译)]

[Adult medulloblastomas. Clinical, anatomical and therapeutical study on seventeen cases (author's transl)].

作者信息

Paillas J E, Hassoun J, Torres-Garcia P, Miihotey P, Vialet G

出版信息

Sem Hop. 1979;55(35-36):1590-6.

PMID:231828
Abstract

Medulloblastoma is classically a childhood tumor, but one third of the cases occurs in adulthood. 17 adult cases are reported here (12 men, 5 women). The average age was 26 years 6 m (from 16 to 47 years). Pre-surgical clinical course is shorter before 30 years (7 months) than after (up to 24 months). Initial symptoms were intracranial hypertension with signs of lateralisation in 2:3 of the cases (cranial nerve and pontocerebellar syndroms). Vertebral angiography, scintigraphy, tomodensitometry helped to the diagnosis. Morphologically, two variants of tumors were found: soft and infiltrative classical medulloblastoma (12 cases) and desmoplastic medulloblastoma which was firmer and better limited (5 cases). The treatment consisted of a surgical removal, as complete as possible, then of an entire neuraxis radiation. Three operated patients died before 6 weeks. Among the 14 other cases, six patients are still alive three years, four other, five years and one, 22 years after the operation. This histological type was of no influence on the post-operative course. Remote metastasis were noted in cauda equina (3 cases), in spinal cord (1 case), in bones and lymphatic nodes (3 cases).

摘要

髓母细胞瘤通常是一种儿童肿瘤,但三分之一的病例发生在成人期。本文报告了17例成人病例(12例男性,5例女性)。平均年龄为26岁6个月(16至47岁)。30岁之前手术前的临床病程(7个月)比之后(长达24个月)短。2/3的病例初始症状为伴有定位体征的颅内高压(颅神经和桥小脑综合征)。椎动脉血管造影、闪烁扫描、计算机断层扫描有助于诊断。在形态学上,发现了两种肿瘤变体:柔软且浸润性的经典髓母细胞瘤(12例)和更坚实且边界更清晰的促纤维增生性髓母细胞瘤(5例)。治疗包括尽可能彻底的手术切除,然后进行全脑脊髓放疗。3例接受手术的患者在6周前死亡。在其他14例病例中,6例患者术后3年仍存活,另外4例术后5年存活,1例术后22年存活。这种组织学类型对术后病程没有影响。远处转移见于马尾(3例)、脊髓(1例)、骨骼和淋巴结(3例)。

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