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Desmoid tumors: Systemic therapy

Authors
Vinod Ravi, MD
Shreyaskumar R Patel, MD
Section Editor
Thomas F DeLaney, MD
Deputy Editor
Diane MF Savarese, MD

INTRODUCTION

Desmoid tumors (also called aggressive fibromatosis, deep musculoaponeurotic fibromatosis, and fibrosarcoma grade I of the desmoid type) are locally aggressive tumors with no known potential for metastasis or dedifferentiation. However, tumor invasion into vital structures and/or organs can result in substantial morbidity and may be fatal, especially in the case of intraabdominal desmoids arising in patients with familial adenomatous polyposis (FAP). Desmoid tumors are responsible for 9 to 11 percent of deaths among patients with FAP. (See "Desmoid tumors: Epidemiology, risk factors, molecular pathogenesis, clinical presentation, diagnosis, and local therapy", section on 'FAP and Gardner's syndrome'.)

Because desmoid tumors lack the ability to metastasize, local control using surgery and radiation has traditionally been the mainstay of therapy for these tumors. However, there is a significant risk of local recurrence, even after complete surgical resection. Increasingly, a multidisciplinary approach to management is being used, particularly among patients with an intraabdominal desmoid. Multimodality therapy can result in effective disease control in up to 96 percent of such patients with both FAP-associated and sporadic desmoid tumors, even when the disease occurs at a difficult location such as the intestinal mesentery [1,2].

This topic review will discuss systemic therapy for desmoid tumors. Epidemiology, risk factors, molecular pathogenesis, clinical presentation, and local treatment options for desmoids are discussed elsewhere. (See "Desmoid tumors: Epidemiology, risk factors, molecular pathogenesis, clinical presentation, diagnosis, and local therapy".)

GENERAL ASPECTS

Desmoid tumors lack the ability to metastasize, and therefore local control using surgery and radiation has traditionally been the mainstay of therapy for these tumors. (See "Desmoid tumors: Epidemiology, risk factors, molecular pathogenesis, clinical presentation, diagnosis, and local therapy", section on 'Treatment'.)

There are no evidence-based or widely accepted guidelines for management of unresectable desmoids. At our institution [1] and others [2], systemic therapy is increasingly being integrated into a multidisciplinary approach for selected patients with unresectable or intraabdominal desmoids for which local therapy options may cause unacceptable morbidity. Early and aggressive systemic therapy in well-selected patients has the potential to prevent complications that might adversely affect survival.

                        

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Literature review current through: Nov 2016. | This topic last updated: Fri Jan 15 00:00:00 GMT+00:00 2016.
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