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

James B. Moore, MD; Nancy Waldenmaier; E. James Potchen, MD
Am J Dis Child. 1987;141(7):714-716. doi:10.1001/archpedi.1987.04460070016004.
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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


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