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Chapter-18 Soft Tissue Sarcoma

BOOK TITLE: Surgical Oncology: Fundamentals, Evidence-based Approaches and New Technology

Author
1. Choudry Haroon A
2. Alcala Marco A
3. Narang Rahul
ISBN
9789350250518
DOI
10.5005/jp/books/11193_17
Edition
1/e
Publishing Year
2011
Pages
11
Author Affiliations
1. University of Pittsburgh, USA, University of Pittsburgh Medical Center and Research Institute, Pittsburgh, Pennsylvania, USA
2. University of Pittsburgh, USA
3. University of Pittsburgh, USA, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York, USA
Chapter keywords

Abstract

Soft tissue sarcomas are rare tumors of mesenchymal origin that account for < 1% of adult malignancies and 15% of childhood tumors. In 2008, there were approximately 9,400 new cases of soft tissue sarcoma in the United States, with a mortality of 3,400 patients. They present in all age groups but usually occur in the fifth or sixth decade of life, with a slight male predominance in a ratio of 1.4:1. Soft tissue sarcomas most frequently arise in the extremities (60%), followed by trunk (20%), retroperitoneum and abdomen (15%) and head and neck (5-10%). There are over 50 histologic sub-types of soft tissue sarcomas that widely differ from each other in biology, aggressiveness, location, patterns of recurrence and spread and ultimately treatment and prognosis. Overall, the most common types are malignant fibrous histiocytoma (28%), liposarcoma (15%), leiomyosarcoma (12%), synovial sarcoma (10%), malignant peripheral nerve sheath tumor (6%) and rhabdomyosarcoma (5%). However, the frequency of histologic sub-types also varies by anatomic site of origin. The most common soft tissue sarcoma in the extremities is malignant fibrous histiocytoma, whereas, liposarcomas are the predominant sarcoma in the retroperitoneum and rhabdomyosarcomas comprise the majority of childhood soft tissue sarcomas. In the distal extremities epithelioid sarcomas are the most common sub-type in the hands, while synovial sarcoma and clear cell sarcoma comprise the majority in the feet. Current National Comprehensive Cancer Network (NCCN) surveillance guidelines recommend history and physical examination on every 3-6 months for the first 2-3 years, then on every 6 months for the subsequent 2 years, then annually. Chest imaging should be performed on every 6-12 months for stage I and on every 3-6 months for stage II/III tumors for the first 5 years and then annually. Primary site surveillance imaging with CT scan or MRI is advisable for anatomically deep seated tumors not amenable to physical examination. Soft tissue sarcoma recurrence is rare after 10 years, therefore, further surveillance is not essential at this time.

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