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Soft Tissue Tumors

Introduction

Sarcomas-general


Reviewer: Komal Arora, M.D. (see Reviewers page)
Revised: 6 July 2012, last major update July 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● 9,500 new cases per year in US
● Classified based on type of cellular differentiation, although most arise from multipotent mesenchymal cells that then differentiate along various cell lines
● Diagnostic errors are often due to lack of familiarity with rare lesions, not failure to perform immunostains (Am J Clin Pathol 2001;116:473)

WHO classification:
● Last revised in 2002 (Histopathology 2006;48:3), Table of Contents of WHO book-2002, World Health Organization classification with comments (Atlas of Genetics and Cytogenetics)

Clinical features
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● Usually arise de novo, not from benign tumors (MPNST may be an exception)
● Do not appear to arise from trauma
● May be caused by radiation therapy (MFH, extraskeletal osteosarcoma), foreign bodies (MFH, angiosarcoma) or chemical carcinogens (angiosarcoma)
● Recommended to diagnose with FNA, core biopsy or incisional biopsy so appropriate treatment can be determined in advance
● Congenital soft tissue tumors, even with high grade features, rarely have malignant behavior
● Nodal involvement uncommon
● Local recurrences show increased number of genetic changes, including loss at 9p, gains at 5p and 20q (Mod Pathol 2001;14:978)

Poor prognostic factors:
● Large size, deep seated versus superficial, retroperitoneum versus extremities, high grade, high stage, positive margins (associated with local recurrence)

Treatment
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● Ideally wide local excision with 1-2 cm margin of normal tissue
● If not possible due to anatomic constraints (neurovascular bundle, bone), a marginal excision (through pseudocapsule) or focally positive margin is acceptable
● Surgery should also remove site of prior incision
● Pre- or postoperative radiotherapy are routinely used for large, high-grade tumors or disseminated disease
● Amputation is reserved for unresectable tumors
● Surgical resection of pulmonary metastases often has value

Fine needle aspiration
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● Most specimens are recognized as sarcoma, and subtyping is most accurate in bone sarcomas and pediatric sarcomas
● Subtyping is often not possible in adult specimens, but this usually does not affect initial therapy (Am J Clin Pathol 2001;115:59)
● For staging information, click here

End of Soft Tissue Tumors > Introduction > Sarcomas-general


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