Successful combined treatment for giant mesenteric desmoid tumor: case report and review of the literature

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• 作者：Silvana Federici^a ; Maurizio Mordenti^a ; Vincenzo Domenichelli^a ; ^{zodott@me.com} ; Gabriella Pelusi^a ; Simona Straziuso^a ; Roberta Pericoli^b
• 关键词：Desmoid tumor ; Mesenteric fibromatosis ; Low-dose chemotherapy
• 刊名：Journal of Pediatric Surgery
• 出版年：2012
• 期刊代码：190_00223468
• 类别：med
• 出版时间：June, 2012
• 卷：47
• 期：6
• 页码：e25-e30
• 文件大小：622 K

摘要

Mesenteric aggressive fibromatosis, also known as abdominal desmoid tumor (DT), is a rare monoclonal neoplasm arising from muscoloaponeurotic structures, caused by a generalized defect in growth regulation of the connective tissue. Childhood abdominal DT is very rare (1), and the mesenteric localization is one of the rarest forms (approximately 5%of total cases). Despite its benign microscopic appearance and nonmetastasizing behavior, abdominal DT shows a high risk of recurrence (30%-80%) (2) and local aggressive growth.

We report a case of a 7-year-old girl with a giant mesenteric fibromatosis, with multiple recurrence after surgical resections, successfully treated with low-dose of vinblastine (3-6 mg/m² per week) and methotrexate (20-30 mg/m² per week) for 24 months (every 7 days for 11 months and every 2 weeks for the last 13 months).

After a follow-up of 47 months from the end of treatment, the child is in good health and in complete remission. Prolonged therapy with low-dose methotrexate and vinblastine appears to control abdominal DT and is associated with stable disease in patients with tumor unresponsive to surgery.