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先天性全身性纤维瘤病

Congenital generalized fibromatosis.

作者信息

Aguirre Vila-Coro A, Dominguez R, Calk J B, Gallardo G

机构信息

University of Texas Health Science Center, Houston.

出版信息

Ann Ophthalmol. 1990 Jun;22(6):217-21.

PMID:2369033
Abstract

Congenital fibromatosis is a rare form of fibroblastic proliferation, which may appear similar to fibrosarcoma; it is multicentric, involving soft tissues, viscera, and bones. The larger soft tissue tumors are very hemorrhagic and necrotic, and they may contain calcifications. Because these characteristics are also present in the more common hemangiomas of infancy, congenital fibromatosis can be diagnosed erroneously as hemangioma. We report a case of congenital generalized fibromatosis erroneously diagnosed initially as hemangioma. The multicentric tumor was aggressive, ultimately resulting in death.

摘要

先天性纤维瘤病是一种罕见的成纤维细胞增殖形式,可能类似于纤维肉瘤;它是多中心的,累及软组织、内脏和骨骼。较大的软组织肿瘤有大量出血和坏死,可能含有钙化。由于这些特征也见于婴儿期较常见的血管瘤,先天性纤维瘤病可能被误诊为血管瘤。我们报告一例最初被误诊为血管瘤的先天性全身性纤维瘤病病例。这个多中心肿瘤具有侵袭性,最终导致死亡。

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