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先天性全身性纤维瘤病:一例伴有骨骼X线表现的病例报告

Congenital generalized fibromatosis: a case report with roentgen manifestations of the skeleton.

作者信息

Schlangen J T

出版信息

Radiol Clin (Basel). 1976;45(1):18-26.

PMID:819961
Abstract

Congenital generalized fibromatosis is a rare disease. Since the features were described in 1954, 23 cases have been mentioned in the literature. 4 cases that had been reported before that time may retrospectively be said to belong to this syndrome. Congenital generalized fibromatosis resembles clinically as well as roentgenologically neurofibromatosis and may therefore be classified as a hamartomatosis, although the histological distinction of the congenital fibrosarcoma may sometimes be difficult. In this case report, a young patient is described with clinical, roentgenological and histological features of congenital generalized fibromatosis.

摘要

先天性全身性纤维瘤病是一种罕见疾病。自1954年对其特征进行描述以来,文献中提及了23例病例。在该时间之前报道的4例病例经回顾性分析可认为属于此综合征。先天性全身性纤维瘤病在临床及放射学上与神经纤维瘤病相似,因此可归类为错构瘤病,尽管先天性纤维肉瘤的组织学鉴别有时可能存在困难。在本病例报告中,描述了一名具有先天性全身性纤维瘤病临床、放射学及组织学特征的年轻患者。

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