Clinical presentation of desmoid tumors

Abstract

Desmoid tumors (DT) constitute a rare fibroblastic proliferative disease. They present sporadically or as a manifestation of a hereditary syndrome such as Familial Adenomatous Polyposis (FAP). Despite the absence of metastatic potential, DT may cause debilitating symptoms and in some cases life-threatening organ damage because of their locally invasive nature. DT may range from small slow-growing masses to rapidly enlarging aggressive tumors. The clinical course of the disease is unpredictable but available data suggest an initial phase of growth may be followed by a long period of growth arrest with tumor stabilization or even regression. FAP-related DT are preferentially located in the abdomen whereas sporadic DT tend to involve mostly the extremities, although the abdomen and the thorax may also be affected. Antecedent trauma, pregnancy and estrogens play a role in the genesis of some desmoid tumors. Surgery is the favored current approach in the treatment of most desmoid tumors. Definitive protocols are not available as most studies have been retrospective, small and comprised of mixed populations of FAP and non-FAP as well as of mixed populations of extra-abdominal and intra-abdominal patients. © Springer Science+Business Media B.V. 2012.