[Clinical aspects, classification and prognosis of 7 cases of pediatric fibromatosis]

Abstract

Seven cases of fibromatoses in infancy and childhood serve as examples to demonstrate this group of rare tumours, taking especially the aggressive forms of juvenile fibromatoses into account. There is a tendency to locally invasive destructive growth without metastasising at any time and a markedly high relapse rate after surgery--a tendency that showed up clearly in our patients, too. Only one child out of four having infantile (desmoid-type) fibromatosis of different localisation did not show any recurrence. The article reports, among other cases, on a desmoid of the stomach and oesophagus in a 15-year-old girl, not described in the literature so far, and on a congenital fibromatosis of the pancreas. Several classifications had to be performed to properly grade the individual cases (1, 6), since no generally valid systematic description of these tumours exists.