Surgery, desmoid tumors, and familial adenomatous polyposis: Case report and literature review

Objectives: To demonstrate the role of surgical trauma in desmoid tumor formation in Gardner's syndrome. Methods: Literature review indicates that desmoid tumors are exceedingly common in familial adenomatous polyposis (FAP) where the comparative risk is 852 times that of the general population. Prior abdominal surgery has been found in as many as 68% of FAP patients with abdominal desmoids. Fifty-five percent develop these lesions within 5 yr of their surgery. We describe a patient with Gardner's syndrome complicated by a desmoid tumor. Results: This patient underwent a prophylactic colectomy at age 14, and 3 yr later developed intra-abdominal desmoid tumors unresponsive to radiation therapy, surgical excision, Sulindac, and tamoxifen therapy. The desmoids became massive and were inoperable. However, they showed an apparent complete response to a chemotherapeutic regimen of doxorubicin (90 mg/m²) and dacarbazine (900 mg/m²) in divided doses over 4 days of continuous infusion every 28 days. Because CT scans could not confirm this complete response, laparoscopy was performed. However, within a matter of only several months, desmoid tumors began developing in each of the three trocar sites, became massive and inoperable, and led to the death of this patient. Conclusion: These findings provide convincing evidence of the association between surgical trauma and the occurrence of desmoid tumors in patients with Gardner's syndrome.