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July 1988

Juvenile Aggressive Fibromatosis of the Head and Neck

Author Affiliations

Charleston, SC

Arch Otolaryngol Head Neck Surg. 1988;114(7):713. doi:10.1001/archotol.1988.01860190017007

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David H. Thompson and coworkers at the US Naval Hospital, Bethesda, Md, reported their experience with three children with aggressive fibromatosis to the Southern Section meeting of the Triological Society recently held in Birmingham, Ala. All three patients presented with a rapidly growing, painless jaw mass (two of the mandible and one of the maxilla) and began to develop airway compromise. The recommended evaluation included enhanced computed tomographic scanning to document the bony destruction and tumor vascularity, both prominent features in the patients described. Wide extirpation was curative, with follow-up from 12 to 33 months. For incompletely excised or unresectable tumors, radiation therapy has been helpful according to some reports, and intravenously administered doxorubicin (Adriamycin) in others. The authors postulated a genetic predisposition to fibromatosis, and prior trauma also has been implicated in some cases.

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