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Congenital Generalized Fibromatosis: A New Management Strategy Provided by Magnetic Resonance Imaging

Authors :
James B. Moore
E. James Potchen
Nancy Waldenmaier
Source :
Archives of Pediatrics & Adolescent Medicine. 141:714
Publication Year :
1987
Publisher :
American Medical Association (AMA), 1987.

Abstract

Sir .—Congenital generalized fibromatosis (CGF) is a rare disease characterized by multiple fibromatous tumors that are present at birth, often showing proliferation and growth during infancy, with subsequent spontaneous regression if the patient survives. This peculiar natural history presents serious management dilemmas for the clinician, with uncertainty regarding the distribution of lesions and probable course. Herein, we communicate the ability of magnetic resonance imaging (MRI) to clearly delineate the precise location of fibromas in all parts of the body, and we present evidence that this new imaging modality may provide insights into the biologic stage (proliferation/regression) of individual lesions in critical areas. This represents a significant advance in the management of infants with CGF and allows frequent follow-up without exposure to ionizing radiation. In addition, we suggest that previous prognostic subclassifications of the disease were in error. Patient Report .—The patient was a vigorous boy with CGF who was originally

Details

ISSN :
10724710
Volume :
141
Database :
OpenAIRE
Journal :
Archives of Pediatrics & Adolescent Medicine
Accession number :
edsair.doi...........b2adce2e714e8ac26a8dd5b0ef94a05b
Full Text :
https://doi.org/10.1001/archpedi.1987.04460070016004