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婴儿肌纤维瘤病:临床病理学综述及新治疗选择展望

Infantile myofibromatosis: a review of clinicopathology with perspectives on new treatment choices.

作者信息

Goldberg N S, Bauer B S, Kraus H, Crussi F G, Esterly N B

机构信息

New York Medical College, Valhalla.

出版信息

Pediatr Dermatol. 1988 Feb;5(1):37-46. doi: 10.1111/j.1525-1470.1988.tb00882.x.

Abstract

The fibromatoses are a heterogeneous group of disorders characterized by proliferation of fibroblasts. Infantile myofibromatosis is a variant that is distinctive because of its multicentric origin, appearance at birth, and cellular composition, which is predominantly myofibroblasts. We treated a patient with infantile myofibromatosis with the interesting clinical presentation of a linear lesion involving the left arm and shoulder, and aggressive hepatomegaly with jaundice secondary to fibroblastic infiltration of the common bile duct and gallbladder. Diagnosis was confirmed histologically and ultrastructurally. Excision of the cutaneous lesion was facilitated by tissue expansion of uninvolved regional tissue.

摘要

纤维瘤病是一组异质性疾病,其特征为成纤维细胞增殖。婴儿肌纤维瘤病是一种变体,因其多中心起源、出生时出现以及细胞组成(主要是肌成纤维细胞)而独具特色。我们治疗了一名患有婴儿肌纤维瘤病的患者,该患者临床表现有趣,左臂和肩部出现线状病变,因胆总管和胆囊成纤维细胞浸润继发严重肝肿大并伴有黄疸。通过组织学和超微结构检查确诊。未受累区域组织的组织扩张有助于切除皮肤病变。

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