Zur Klinik und Klassifikation der juvenilen Fibromatosen

Abstract

From 1963 through 198324 infants and children (mean age 3.2 years) were operated on for extra-abdominal desmoid fi bromatosis (F). The patients were classified according to En ::illger and Wciss (1983). 14 children presented with infantile or juvenile F, 3 with digital F, and 7 with F colli. The lesion recurred after local excision in 50(\\) of the patients. 37.5% had a second local recurrence, and 16.6 % more than 2 (up to 7) local recurrences. Therapy of F consists of radical local excislOn of the tumour. Local irradiation or local administra tion ofcorticosteroids may be helpful in special cases, as may be systemic administration of chemotherapy, corticosteroids or inhibitors of cAM P. Spontaneous regression of F has been observed.