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Desmoid tumors

INTRODUCTION

Desmoid tumors are deep-seated, benign, slowly growing fibroblastic neoplasms that arise from musculoaponeurotic stromal elements. The term "desmoid" originates from the Greek word "desmos", meaning band or tendon-like, and was first applied in the 1800s to describe tumors with a tendon-like consistency.

Desmoids do not have the capacity to establish metastatic lesions, yet they are locally aggressive and have a high rate of recurrence even after complete resection. Tumor-related destruction of vital structures and/or organs can be fatal, particularly when these tumors arise in patients with familial adenomatous polyposis (FAP, Gardner's syndrome). The intraabdominal desmoids that develop in this setting are often unresectable because they diffusely infiltrate the mesentery. In addition, recurrences tend to become more frequent and aggressive with each surgical intervention.

This topic review will discuss the epidemiology, risk factors, molecular pathogenesis, clinical presentation, and treatment of desmoid tumors. Other miscellaneous benign diseases affecting soft tissue and bone including Gorham's disease, desmoplastic fibroma of bone, and pigmented villonodular synovitis are discussed elsewhere as are keloids and ganglia. (See "Miscellaneous benign diseases affecting soft tissue and bone" and "Keloids" and "Ganglia and nodules".)

EPIDEMIOLOGY AND RISK FACTORS

Desmoid tumors are rare; they account for about 0.03 percent of all neoplasms and less than 3 percent of all soft tissue tumors. The estimated incidence in the general population is two to four per million population per year, which in the United States translates into approximately 900 new cases annually [1].

Individuals between the ages of 15 and 60 can be affected; desmoids are rare in the young and in the elderly. They are slightly more common in women than in men, and there is no significant racial or ethnic predilection.
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