Desmoid (meaning tendon-like) tumors are a heterogeneous group of rare connective tissue neoplasms, which can occur at almost any anatomical location.
Desmoids have been classified as fibromatoses, along with pathologies such as palmar fasciitis, which are due to proliferation of well-differentiated fibroblasts and are locally infiltrative and tend to recur after excision but do not metastasize.
Desmoids are rare, accounting for less than 0.1 % of all tumors, and have an annual incidence of two to four per million. While most occur sporadically, 2 % are associated with familial adenomatous polyposis (FAP), an autosomal dominantly inherited cancer predisposition syndrome due to mutation of the tumor suppressor gene APC (APC gene in familial adenomatous polyposis). Desmoids are over 1,000 times more common in individuals with FAP than in the population in general, occurring in about...