[The desmoid problem]

Abstract

Desmoid tumors (DTs) are rare nonmetastasizing neoplasms, occurring both sporadically and in the context of familial adenomatous polyposis (FAP). DTs show an unpredictable natural course with approximately 10% demonstrating a severely aggressive growth pattern, whereas others regress spontaneously. Surgery appears to have a triggering effect on desmoid growth, but is unavoidable in patients with FAP requiring prophylactic colectomy. Treatment recommendations remain controversial. Surgery may only be regarded as an option if a safety margin of at least 2 cm is guaranteed. Even under these circumstances recurrence rates are very high. Debulking operations invariably lead to a boost towards more aggressive growth. Most issues regarding DTs are contradictory, reflecting the lack of any systematic approach to the disease. One way out of this dilemma is the establishment of a clinical classification as a first step towards standardized therapy.