Gardner syndrome with extra and intra-abdominal desmoid tumors and adrenal involvement: PET/CT findings

Gardner syndrome, also known as familial colorectal polyposis, is an autosomal dominant disease and part of the spectrum of familial adenomatous polyposis (FAP). It is characterized by the presence of multiple polyps in the colon together with neoplasms outside the colon. Gardner syndrome is also associated with desmoid tumors in approximately 15% of affected individuals. Desmoid tumors are rare benign slow-growing neoplasm's, arising from connective tissue (musculoaponurotic fibrous) tissue. Due to their locally aggressive nature, the term aggressive fibromatoses have been used to describe these tumors. 7% of patients with FAP or its variants may also have adrenal masses, most are benign and asymptomatic. We report the PET/CT of a 42 year old diagnosed with Gardner syndrome demonstrating on PET/CT the rare coexistence of the intestinal polyps, extra and intra abdominal desmoids and adrenal adenoma. In addition the characteristic cutaneous lesions and osteomas of the skull were also noted.