Challenges in the treatment of childhood fibromatosis
B. N. Rao, M. E. Horowitz, D. M. Parham, E. E. Etcubanas, I. D. Fleming, C. B. Pratt, H. O. Hustu, A. A. Green and L. E. Kun
Division of Surgery, St Jude Children's Research Hospital, Memphis, TN 38101.
Between 1968 and 1985, we treated 20 children for fibromatosis (also called
desmoid tumor and aggressive fibromatosis). The primary sites included head
and neck (seven patients), extremity (seven patients), and trunk (six
patients). Lesions ranged from 3 to 18 cm in diameter. The tumors were
smaller than 5 cm in 13 patients, and in seven patients they were larger
than 5 cm. A total resection was not feasible in any of the patients with
lesions larger than 5 cm. Ten of the 11 patients treated with wide local
resection, in whom the margins were clearly negative or close, remained
free of disease for six to 16 years. Nine patients required additional
treatment with radiotherapy (nine patients) and chemotherapy (five
patients). Two died of local disease progression. In the remaining seven
children, the disease was controlled. We describe our strategies for
managing this disease in a pediatric population.
