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先天性软组织孤立性纤维瘤病,先天性全身性纤维瘤病的一种变异型。2例报告。

Congenital solitary fibromatosis of soft tissues, a variant of congenital generalized fibromatosis. 2 cases reports.

作者信息

Kindblom L G, Termén G, Säve-Söderbergh J, Angervall L

出版信息

Acta Pathol Microbiol Scand A. 1977 Sep;85(5):640-8. doi: 10.1111/j.1699-0463.1977.tb00454.x.

Abstract

A report of 2 cases of solitary fibromatosis in a 10-day-old boy and a girl 3 years and 10 months old is presented. Both lesions were deep-seated and showed a nodular and infiltrating growth, predominantly buil-up by immature fibroblast-like cells and including hemangiopericytoma-like areas. One of the lesions also showed leiomyoma-like areas. An ultrastructural study however, revealed no intra-cytoplasmatic myofilaments. At follow-up examinations after 21 years and 1 year, respectively, there were no signs of recurrences or metastases. These 2 cases are considered to represent a solitary form of congenital generalized fibromatosis. The differential diagnosis from infantile hemangiopericytoma and fibrous lesions seen in infancy and early childhood, such as infantile fibrosarcoma, diffuse infantile fibromatosis, extra-abdominal desmoid, fibrous hamartoma of infancy and juvenile aponeurotic fibroma, is discussed.

摘要

本文报告了1例10日龄男婴和1例3岁10个月女童的孤立性纤维瘤病。两个病变均为深部病变,呈结节状浸润性生长,主要由未成熟的成纤维细胞样细胞构成,包括血管外皮细胞瘤样区域。其中一个病变还显示有平滑肌瘤样区域。然而,超微结构研究未发现胞浆内肌丝。分别在21年和1年后的随访检查中,均未发现复发或转移迹象。这2例病例被认为代表先天性全身性纤维瘤病的一种孤立形式。文中还讨论了与婴儿血管外皮细胞瘤以及婴儿期和儿童早期所见纤维性病变(如婴儿纤维肉瘤、弥漫性婴儿纤维瘤病、腹外硬纤维瘤、婴儿纤维性错构瘤和青少年腱膜纤维瘤)的鉴别诊断。

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