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Image of the Month—Diagnosis
Arch Surg. 2004;139:224.
Answer: Desmoid Tumor
Figure 1. Computed tomographic scan of the abdomen and pelvis demonstrating a large, homogeneous mass of approximately 23 x 15 cm.
Figure 2. Biopsy specimen taken from the intra-abdominal tumor showing the same appearance as the incisional biopsy specimens taken from the mesentery: a paucicellular to cellular proliferation of bland-looking fibroblasts, infiltrating muscle and engulfing muscle fibers (black arrowheads). No mitoses are seen. Immunohistochemical analysis was negative for CD117 (poly C-kit) and estrogen receptors but weakly positive for progesterone receptors.
Desmoid tumors are benign in origin and do not metastasize. However, they can be aggressive locally and have a tendency for local recurrence after excision.1-2 Their ability for local infiltration can result in significant deformity, morbidity, and mortality. Desmoid tumors in the general population are rare, accounting for 0.03% of all neoplasms.3-4 There is an association between the occurrence of desmoid tumors and familial adenomatous polyposis coli, especially in patients with Gardner syndrome.3-4 Both intra- and extra-abdominal desmoid tumors occur more frequently in patients with familial adenomatous polyposis coli, with an incidence of 3.5% to 32%.4-5
The treatment of choice for desmoid tumors is radical excision, both for primary tumors and recurrent lesions. However, a radical resection of mesenteric desmoid tumors is often not feasible owing to the inevitable preservation of vital anatomic structures, as was the case in our patient. In such cases, treatment with sulindac, tamoxifen, or toremifene citrate may alleviate symptoms.5-7 Radiotherapy has only a minor role, with some reports of complete and partial remission following adjuvant radiotherapy.2, 8 Some attempts have been made to use chemotherapy, but, with a partial response in 50% to 60% of cases, the results have been inconclusive.9-10
In the patient described, diagnoses other than desmoid tumor were also considered, including gastrointestinal stromal tumor, fibrosarcoma, and ovarian carcinoma. We considered gastrointestinal stromal tumor because of the rectal blood loss. These tumors occur in the entire gastrointestinal tract and may also arise from the omentum, mesenteries, and retroperitoneum. Such tumors may become large and occur more often in men than women, with the highest incidence around 50 to 60 years of age. Unlike the specimen described, they are positive for CD117 on histologic examination.11 Fibrosarcoma seemed plausible because of the tumor's rapid growth and the fact that it is usually difficult to distinguish between low-grade fibrosarcoma and desmoid tumor. Ovarian carcinoma was considered because of the tumor's enormous size; however, ovarian carcinoma is often cystic. In accordance with the patient's history of Gardner syndrome, a desmoid tumor was thought to be the most likely option and turned out to be the case.
AUTHOR INFORMATION
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Corresponding author: Joost M. Klaase, MD, PhD, Department of Surgery, Medisch Spectrum Twente, PO Box 50.000, 7500 KA Enschede, the Netherlands (e-mail: j.klaase{at}ziekenhuis-mst.nl).
Accepted for publication March 15, 2003.
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SECTION EDITOR: GRACE S. ROZYCKI, MD
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