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Desmoid tumors: Epidemiology, risk factors, molecular pathogenesis, clinical presentation, diagnosis, and local therapy

Authors
Vinod Ravi, MD
Shreyaskumar R Patel, MD
Chandrajit P Raut, MD, MSc, FACS
Thomas F DeLaney, MD
Section Editors
Robert Maki, MD, PhD
Russell S Berman, MD
Raphael E Pollock, MD
Deputy Editor
Diane MF Savarese, MD

INTRODUCTION

Desmoid tumors (also called aggressive fibromatosis, deep musculoaponeurotic fibromatosis, and also formerly termed fibrosarcoma grade I of the desmoid type) are locally aggressive tumors with no known potential for metastasis or dedifferentiation. 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.

Although they lack the capacity to establish metastases, desmoids 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).

This topic review will discuss the epidemiology, risk factors, molecular pathogenesis, clinical presentation, and local treatment options for desmoid tumors. Systemic therapy is discussed elsewhere, as are other miscellaneous benign diseases affecting soft tissue and bone, such as Gorham's disease, desmoplastic fibroma of bone, pigmented villonodular synovitis, keloids, and ganglion cysts. (See "Desmoid tumors: Systemic therapy" and "Antineoplastic therapy for miscellaneous benign diseases affecting soft tissue and bone" and "Keloids and hypertrophic scars" and "Ganglion cysts of the wrist and hand".)

EPIDEMIOLOGY AND RISK FACTORS

Desmoid tumors are rare; they account for approximately 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 [1].

Individuals between the ages of 15 and 60 are most commonly affected; desmoids are rare in the young and in the elderly. They are slightly more common in women than in men [2], and there is no significant racial or ethnic predilection.

                                     

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