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Clinical presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma

Authors
Christopher W Ryan, MD
Janelle Meyer, MD
Section Editors
Robert Maki, MD, PhD
Raphael E Pollock, MD
Deputy Editor
Diane MF Savarese, MD

INTRODUCTION

Sarcomas are a rare and heterogeneous group of malignant tumors of mesenchymal origin that comprise less than 1 percent of all adult malignancies and 12 percent of pediatric cancers [1-3]. Approximately 80 percent of sarcomas originate from soft tissue and the rest from bone. There are about 12,310 new cases of soft tissue sarcoma diagnosed each year in the United States, with 4990 deaths [3].

The histopathologic spectrum of sarcomas is broad, presumably because the embryonic mesenchymal cells from which they arise have the capacity to mature into striated skeletal and smooth muscle, adipose and fibrous tissue, bone, and cartilage. Although ectodermal in origin, malignant tumors affecting peripheral nerves are included because of similarities in their clinical behavior, management, and outcome.

This topic review will cover the clinical presentation, diagnostic evaluation, and staging of soft tissue sarcoma other than gastrointestinal stromal tumor (GIST), the most common sarcoma, discussed in detail elsewhere. Issues specific to soft tissue sarcomas arising in the head and neck, retroperitoneum, and in the breast are discussed elsewhere, as are bone sarcomas and Kaposi’s sarcoma. (See "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 "Osteosarcoma: Epidemiology, pathogenesis, clinical presentation, diagnosis, and histology" and "AIDS-related Kaposi sarcoma: Staging and treatment" and "Classic Kaposi sarcoma: Clinical features, staging, diagnosis, and treatment".)

HISTOPATHOLOGY

As classified by the World Health Organization (WHO), the group of soft tissue sarcomas includes more than 100 different histologic subtypes [2]. The most common subtypes that arise in adults are outlined in the table (table 1).

The WHO classifies most soft tissue sarcomas according to the presumptive tissue of origin (ie, the normal tissues the tumor most closely resembles) [2]. Examples include liposarcoma, synovial sarcoma, leiomyosarcoma (LMS), rhabdomyosarcoma (RMS), fibrosarcoma, and angiosarcoma. In some cases, histogenesis is uncertain and the designation reflects the morphologic appearance of the cells or the architectural pattern (eg, alveolar sarcoma of soft parts, epithelioid sarcoma, clear cell sarcoma, Ewing sarcoma).

                              

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Literature review current through: Nov 2016. | This topic last updated: Thu Aug 11 00:00:00 GMT+00:00 2016.
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