Juvenile aggressive fibromatosis: report of three cases and review of the literature.
AF is a non-encapsulated, non-metastasizing fibrous tumor that has a tendency for local recurrence in up to 70% of cases. We suspect possible genetic disposition. Microscopically, AF is a poorly circumscribed fibrous tumor that infiltrates surrounding tissues. It presents as a painless, rapidly growing mass with a predilection for bone destruction. Treatment consists of a carefully planned en bloc resection after biopsy and accurate radiologic evaluation of the extent of the tumor. Radiation therapy and chemotherapy can be used as adjuvant therapy in selected patients. Three patients with AF of the head and neck are described, of which two were found to have congenital disorders of mesodermal development.
Department of Otolaryngology and Oral Pathology, National Naval Medical Center, Bethesda, MD.
D H
DH
Thompson
A
A
Khan
C
C
Gonzalez
P
P
Auclair
D000293:Adolescent; D001706:Biopsy; D002675:Child, Preschool; D005260:Female; D005350:Fibroma / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D007573:Jaw Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D008297:Male; D014057:Tomography, X-Ray Computed