The enigma of desmoid tumors

Abstract

Objective: To analyze patients with recurrent extremity desmoids, in whom the surgical therapeutic option was either major amputation or observation.

Summary background data: The biology and natural history of desmoid tumors are an enigma. These tumors invade surrounding structures and recur locally but do not metastasize. The morbidity of treating these tumors in the context of their relatively benign biology is uncertain.

Methods: Between July 1982 and June 1998, the authors treated and prospectively followed 206 patients with extremity desmoid tumors. All patients underwent standardized surgical resection, the surgical goal always being complete resection with negative margins. When tumors recurred, they were evaluated for reresection. Amputation was considered when resection was not possible because of neurovascular or major bone involvement, or in the presence of a functionless, painful extremity.

Results: During this period, 22 patients had disease that was not resectable without amputation. This was out of a total of 115 patients with primary disease and 91 patients with recurrent disease. All recurrences were local; in no patient did metastasis develop. In this group of 22 patients with unresectable disease, 7 underwent amputation and 15 did not. These 15 patients were followed, alive with disease, having no surgical resection. Four patients received systemic treatment with tamoxifen and nonsteroidal antiinflammatories, three received systemic cytotoxic chemotherapy, and two received both tamoxifen and chemotherapy. Six patients received no systemic treatment. The range of follow-up was 25 to 92 months. In all patients, there was no or insignificant tumor progression; in three patients who underwent observation alone, there was some regression of tumor. During follow-up, no patient has required subsequent amputation, and no patient has died from disease.

Conclusions: In desmoid tumors, aggressive attempts at achieving negative resection margins may result in unnecessary morbidity. Function- and structure-preserving procedures should be the primary goal. In select patients, whose only option is amputation, it may be prudent to observe them with their limb and tumor intact.

Figure 1. Distribution algorithm of 206 patients treated and followed with desmoid tumors of the extremity. Of these, 22 tumors were resectable only with amputation, and 15 patients underwent no resection and no amputation.

Figure 2. Axial T1-weighted magnetic resonance imaging scan of shoulder in patient 3. The right image was taken 12 months after the left one. This patient underwent no treatment, and the tumor remained stable during this time.

Figure 3. Axial Fat Sat magnetic resonance imaging scan of thigh in patient 7. The right image was taken 23 months after the left one. The patient underwent no treatment, and the tumor remained stable during this time.

Figure 4. Sagittal T1-weighted magnetic resonance imaging scan of knee in patient 13. The right image was taken 15 months after the left one. The patient underwent no treatment and the tumor remained stable during this time.