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[Desmoid tumor sensitive to tamoxifen]
1Maison Médicale de Lunesse, Isle d'Espagnac.
Background:
Desmoid tumors are the second leading cause of mortality in patients with familial adenomatous polyposis who have undergone proctocolemy. Both medical and surgical treatment of these non-metastasizing but locally aggressive tumors are difficult.
Case Report:
A 20-years-old woman consulted 6 years after coloproctectomy for familial adenomatous polyposis. A mesenteric desmoid tumor was found. Resection was not possible. The tumor progressed under sulindac treatment and subocclusion developed. The patient was given tamoxifen, 20 mg/d and the size of the tumor began to regress within a few days together with pain and signs of compression. The treatment was well tolerated for 4.5 years and tumor volume has remained unchanged after a 20-fold reduction compared with the size prior to treatment. The patient has remained symptom-free.
Discussion:
In this critical clinical situation, tamoxifen provided rapid and prolonged effect and was well tolerated.