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Radiologic-Pathologic Conferences of The University of Texas M. D. Anderson Cancer Center |
1 Division of Diagnostic Imaging, The University of Texas M. D. Anderson Cancer
Center, 1515 Holcombe Blvd., Box 57, Houston, TX 77030.
2 Division of Pathology, The University of Texas M. D. Anderson Cancer Center,
Houston, TX 77030.
3 Division of Surgery, The University of Texas M. D. Anderson Cancer Center,
Houston, TX 77030.
Received October 7, 2003;
accepted after revision October 8, 2003.
S. C. Faria was supported by a grant from CAPES (Coordenacao de
Aperfeicoamento de Pessoal de Nivel Superior) Brazil.
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Desmoid tumors, which are included in a group of disorders called fibromatosis, are benign tumors composed of fibrous elements. Desmoid tumors have an estimated incidence of 3.7 new cases per million people per year [1]. Desmoids may occur in the abdominal wall, the mesentery, or the retroperitoneum. Extraabdominal desmoids may involve the shoulder, the thigh, the buttock, or the trunk [1].
Most intraabdominal desmoids arise in the mesentery, usually in the mesentery of the small bowel. Desmoids are the most common primary tumor of the mesentery [2]. Most of these tumors occur sporadically; however, patients with Gardner's syndrome are at higher risk than others. The incidence of abdominal wall and mesenteric desmoids in patients with Gardner's syndrome ranges between 4% and 29%, and the tumors typically occur after abdominal surgery [1]. Desmoid tumors may be also associated with trauma and estrogen therapy [2].
Desmoids may occur in all age groups but they are typically seen in the third and fourth decades of life [1, 3]. Women are reported to be affected more commonly than men are [1, 3]. However, in the largest study in the literature, with 130 cases, 55% of the cases occurred in men [2].
Most patients with desmoid tumors are clinically asymptomatic, and the tumors cause little or no focal symptoms until late in their course. Patients may present to a physician because of a palpable mass, abdominal pain, or gastrointestinal bleeding [2]. Desmoids can be locally aggressive and may invade contiguous structures. Some complications that have been reported include small-bowel obstruction and hydronephrosis [4].
On gross histopathologic examination, desmoid tumors are usually circumscribed lesions, but they may have irregular or infiltrating borders. On the cut surface, they are white and coarsely trabeculated, resembling scar tissue. Desmoids are usually larger than 5 cm when they are discovered, and they may be larger than 15 cm. In 10–15% of cases, desmoids are multiple [5].
Histologically, desmoid tumors are lesions composed of bland spindled or stellate fibroblastic cells embedded in a collagenous stroma, without evidence of muscular or neural differentiation and with little or no inflammatory component. The tumor may infiltrate adjacent viscera and tissues at the periphery [2].
The imaging appearance of these tumors is variable and depends on fibroblastic proliferation, fibrosis, collagen content, and vascularity. On sonography, desmoid tumors have variable echogenicity, appearing as masses of low, medium, or high echogenicity with smooth, defined margins [3]. On CT, most desmoid tumors appear as well-circumscribed homogeneous masses that may be isodense or hyperdense relative to muscle. Some cases of heterogeneous masses with an infiltrative outer margins are seen. Desmoid tumors may enhance after injection of IV contrast material [1]. On MRI, desmoid tumors appear as masses of low signal intensity relative to muscle on T1-weighted images. On T2-weighted images, desmoid tumors show variable signal intensity relative to muscle [3].
Desmoid tumors have a tendency to recur, even after complete surgical resection [1, 4, 5]. The high recurrence rate favors the use of nonsurgical therapy, such as nonsteroidal antiinflammatory drugs and antiestrogens [1]. Distant metastases have not been reported [2].
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  A. D. Levy, J. Rimola, A. K. Mehrotra, and L. H. Sobin From the Archives of the AFIP: Benign Fibrous Tumors and Tumorlike Lesions of the Mesentery: Radiologic-Pathologic Correlation RadioGraphics, January 1, 2006; 26(1): 245 - 264. [Abstract] [Full Text] [PDF]
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