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Desmoid tumors (DTs) are a rare and biologically heterogeneous group of locally aggressive fibroblastic neoplasm: their biological behavior spectrum ranges from indolent to aggressive tumors. DTs are classified as intra-abdominal, extra-abdominal, and within the abdominal wall lesions.  It is well known that abdominal and extra-abdominal DTs are associated with familial adenomatous polyposis (FAP) and Gardner syndrome. Possible risk factors are prior trauma/surgery, pregnancy, and oral contraceptives.  There was a real revolution in the management of DT: from aggressive first-line approach (surgery and radiation therapy) to a more conservative one (systemic treatment and “wait-and-see policy”).  In these clinical settings, radiologists play an important role for assessing lesion resectability, evaluating recurrence, monitoring the biological behavior if an expectant management is chosen, and assessing response to systemic treatment as well as to radiation therapy.  Awareness of common locations, risk factors, and imaging features is fundamental for a correct diagnosis and an adequate patient management.

Multimodality imaging features of desmoid tumors: a head-to-toe spectrum