Japanese Journal of Clinical Oncology Advance Access originally published online on February 26, 2008
Japanese Journal of Clinical Oncology 2008 38(3):222-226; doi:10.1093/jjco/hyn004
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Dacarbazine-Doxorubicin Therapy Ameliorated an Extremely Aggressive Mesenteric Desmoid Tumor Associated with Familial Adenomatous Polyposis: Report of a Case
Department of Surgery, Gastroenterological Surgery, Graduate School of Medicine, Osaka University, Osaka, Japan
For reprints and all correspondence: Hirofumi Yamamoto, Department of Surgery, Gastroenterological Surgery, Graduate School of Medicine, Osaka University, 2-2 Yamada-oka, Suita-City, Osaka 565-0871, Japan. E-mail: kobunyam{at}surg2.med.osaka-u.ac.jp
Received September 24, 2007; accepted January 7, 2008
A 30-year-old man with familial adenomatous polyposis (FAP) underwent prophylactic proctocolectomy by laparoscopy-assisted surgery. After 10 months, we found an intra-abdominal tumor, which grew rapidly to 25 cm in diameter. We performed an emergency operation, which revealed that it was a desmoid tumor derived mainly from colorectal mesenterium. The tumor was removed with three short segments of intestine and the left ureter. A computed tomography (CT) scan done 3 months later showed a 10 cm mesenteric desmoid tumor at the beginning of jejunum, approaching the root of the superior mesenteric artery (SMA). Fortunately, we were able to remove the tumor without injuring the SMA. To our distress, however, another recurrent mesenteric desmoid tumor was discovered in the pelvis one month later, which grew rapidly from 5 cm to 16 cm within 4 months. During this period, we gave the patient several regimens, including antiestrogen (tamoxifen), a nonsteroidal anti-inflammtory drug and imatinib mesylate (Gleevec), which had little or no effect. Finally, when the desmoid occupied the pelvic space, we gave the patient dacarbazine (DTIC) and doxorubicin (DOX). After seven courses, the mesenteric tumor showed an almost complete response (CR). The chemotherapy caused grade 3 to 4 leukocytopenia, but without any hazardous events. No evidence of further recurrence of mesenteric desmoid has been seen for 4 years. This combination chemotherapy is a promising strategy, even against an extremely aggressive, life-threatening mesenteric desmoid associated with FAP.
Key Words: mesenteric desmoid • familial adenomatous polyposis • doxorubicin • dacarbazine
Abbreviations: APC, adenomatous polyposis coli; CT, computed tomography; DOX, doxorubicin; DTIC, dacarbazine; FAP, familial adenomatous polyposis; NSAID, nonsteroidal anti-inflammtory drug; SMA, superior mesenteric artery.