A 45-year-old man with familial adenomatous polyposis (FAP) and > 1000 colorectal adenomas undergoes total colectomy. He has a desmoid tumor in the mesentery growing at 3 cm/year. What is the first-line medical management for mesenteric desmoid tumors in FAP?

• A Imatinib (tyrosine kinase inhibitor)
• B High-dose sulindac + anti-estrogen therapy (tamoxifen) ✓
• C Sorafenib (multikinase inhibitor)
• D Pembrolizumab (PD-1 inhibitor)

Explanation

Mesenteric desmoid tumors in FAP are first managed with sulindac (COX inhibitor) combined with anti-estrogen therapy (tamoxifen or toremifene), which can induce regression in approximately 50% of cases. Desmoid tumors express estrogen receptors and COX-2, explaining this dual approach. Imatinib is used for PDGFR-mutant sporadic desmoids that fail first-line therapy. Surgery carries very high recurrence rates and significant morbidity in mesenteric desmoids, making medical management preferable as initial treatment.

Reference: Bailey & Love's Short Practice of Surgery, 27th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.