Two cases of aggressive fibromatosis in children presenting as abdominal tumor

Abstract

During the past 7 years we have operated upon two boys 4${\raise0.5ex\hbox{$\scriptstyle 1$}\kern-0.1em/\kern-0.15em\lower0.25ex\hbox{$\scriptstyle 2$}}$ and 5 years old who both had an orange-sized, hard, tumorlike lesion of the abdominal wall. In both cases a history of rapid growth and the clinical presentation suggested a malignant tumor. Histologically, the lesions were classified as aggressive fibromatosis, a histological variant of infantile fibromatosis that may be difficult to distinguish from infantile fibrosrcoma. However, the fibromatoses are benign although their growth pattern may appear invasive. If the tumor is not completely excised, recurrence is often seen but the lesion does not metastasize. The interpretation of frozen sections is difficult and may thus be misleading, leading to unnecessary radical surgery.