GENERALIZED HAMARTOMATOSIS

(CONGENITAL GENERALIZED FIBROMATOSIS)

¹ Associate Professor of Radiology, University of Texas Medical Branch, Galveston, Texas.
² Resident, Department of Pathology, University of Texas Medical Branch, Galveston, Texas.
³ Professor of Radiology, University of Texas Medical Branch, Galveston, Texas.

A review of the literature and an analysis of the 20 previously reported cases of congenital generalized fibromatosis and 4 cases reported under other titles are presented.

The 21st proven case is described. The lesions were visceral and somatic in location.

The personal case reported revealed the following remarkable features: (1) it is the first case reported in a Negro; (2) it is the first case studied with electron microscopy, which added proof to the previous hypothesis that the lesion has a smooth muscle component in addition to the fibroblastic element; and (3) it is the first case in which periosteal reaction was apparent.

It is proposed that the term "generalized hamartomatosis" is better suited to define this entity, without reference to its unproven congenital nature or exclusion of forms of later onset. A new classification is presented which takes into consideration the distribution of the lesions and clinical evolution.

The roentgenographic findings and differential diagnosis are discussed.

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

This article has been cited by other articles:

				M. R. Robbin, M. D. Murphey, H. T. Temple, M. J. Kransdorf, and J. J. Choi Imaging of Musculoskeletal Fibromatosis RadioGraphics, May 1, 2001; 21(3): 585 - 600. [Abstract] [Full Text] [PDF]