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Overview of multimodality treatment for primary soft tissue sarcoma of the extremities and chest wall

Authors
Thomas F DeLaney, MD
Mark C Gebhardt, MD
Christopher W Ryan, MD
Section Editors
Robert Maki, MD, PhD
Russell S Berman, MD
Raphael E Pollock, MD
Deputy Editors
Diane MF Savarese, MD
Kathryn A Collins, MD, PhD, FACS

INTRODUCTION

Sarcomas are malignant tumors that arise from skeletal and extraskeletal connective tissues, including the peripheral nervous system. They can arise from mesenchymal tissue at any body site. Soft tissue sarcomas (STS) are uncommon. In the United States, approximately 12,390 cases are diagnosed annually, representing less than 1 percent of all newly diagnosed malignant tumors [1]. Although surgical resection is, as a general rule, a necessary prerequisite for cure, the addition of radiation therapy is recommended for most patients with an STS of the extremities or chest wall to minimize the risk of a local recurrence, and to maximize function and long-term survival.

This topic will provide an overview of multimodality treatment of STS arising in the extremities and chest wall. The following issues are discussed separately: classification, diagnosis, and staging of STS; radiation-related sarcomas; STS arising in the head and neck, retroperitoneum, and breast; surgical resection of potentially resectable STS of the extremities; management of locally advanced, unresectable STS; and adjuvant as well as neoadjuvant chemotherapy for the types of STS that typically arise in adults. (See "Clinical presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma" and "Radiation-associated sarcomas" and "Head and neck sarcomas" and "Clinical features, evaluation, and treatment of retroperitoneal soft tissue sarcoma" and "Breast sarcoma: Epidemiology, risk factors, clinical presentation, diagnosis, and staging" and "Breast sarcoma: Treatment" and "Surgical resection of primary soft tissue sarcoma of the extremities" and "Treatment of locally recurrent and unresectable, locally advanced soft tissue sarcoma of the extremities" and "Adjuvant and neoadjuvant chemotherapy for soft tissue sarcoma of the extremities".)

IMPORTANCE OF MULTIDISCIPLINARY EVALUATION AND MANAGEMENT

Because of their rarity and the frequent need for multimodality treatment, evaluation and management of soft tissue sarcomas (STS) should ideally be carried out in a center with expertise in the treatment of sarcomas, including surgical oncology, orthopedic surgery, plastic surgery, adult or pediatric medical oncology, and radiation oncology. The multidisciplinary team approach to care of STS optimizes treatment planning, minimizes duplication of diagnostic studies, and reduces the time to implementation of the definitive therapeutic protocol.

Moreover, the expertise gained by dedicated subspecialists improves clinical outcomes. This was illustrated by one study that examined outcomes of 375 patients with STS of the extremities and torso according to the time of referral to a tumor center in the South Sweden Health Care Region [2]. Compared with patients referred preoperatively, local recurrence rates were 2.4-fold higher in patients not referred at any time and 1.3-fold higher in those referred only after definitive surgery.

EXTREMITY SARCOMAS

General approach to treatment — In treating extremity soft tissue sarcoma (STS), the major therapeutic goals are survival, avoidance of a local recurrence, maximizing function, and minimizing morbidity. There are a wide variety of clinical situations that arise from involvement of a variety of different anatomic sites, the range of histologies, and variability in grade and tumor size. As a result, for most patients, treatment must be individualized. The following suggestions serve as a general guide to therapy:

                             

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Literature review current through: Dec 2016. | This topic last updated: Thu Jan 19 00:00:00 GMT+00:00 2017.
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