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先天性内脏纤维瘤病中的中枢神经系统受累

Central nervous system involvement in congenital visceral fibromatosis.

作者信息

Adickes E D, Goodrich P, AuchMoedy J, Bickers G, Bowden B, Koh J, Nelson R M, Shuman R M, Wilson R B

出版信息

Pediatr Pathol. 1985;3(2-4):329-40. doi: 10.3109/15513818509078793.

Abstract

Congenital visceral myofibromatosis is an uncommon disorder characterized by multiple tumors of myofibroblastic origin in the neonatal period. The natural history of the disorder has been well delineated. The myofibroblast is the cell of origin of the tumor. This is a report of a patient in whom multiple mesenchymal tumors occurred in the CNS as well as in other organs. Light and electron microscopic findings of the CNS lesions are similar to those of the somites and viscera.

摘要

先天性内脏肌纤维瘤病是一种罕见的疾病,其特征为新生儿期出现多个肌成纤维细胞起源的肿瘤。该疾病的自然病程已得到明确描述。肌成纤维细胞是肿瘤的起源细胞。本文报告了一名患者,其在中枢神经系统以及其他器官中出现了多个间充质肿瘤。中枢神经系统病变的光镜和电镜检查结果与体节和内脏的病变相似。

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