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Chap 27 - PRIMARY SOFT TISSUE TUMORS WITH EPITHELIAL DIFFERENTIATION

Published online by Cambridge University Press:  01 March 2011

By
Markku Miettinen
Edited by
Markku Miettinen
Markku Miettinen
Affiliation:
Armed Forces Institute of Pathology, Washington DC
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Summary

Epithelial differentiation is often easily detected histologically and is almost always highlighted with immunohistochemical markers (i.e., keratins, epithelial membrane antigen [EMA], and others). Mesothelial proliferations and tumors are discussed in Chapter 28, and Merkel cell carcinoma and metastatic carcinomas in Chapter 29.

Some other nonepithelial cells and tumors also can express keratins and other epithelial markers. Examples of normal varyingly keratin-positive mesenchymal cells include endothelial and smooth muscle cells and some myofibroblasts. Many nonepithelial tumors, such as melanomas and many sarcomas, occasionally are keratin positive. These are discussed in the appropriate chapters.

Synovial sarcoma and epithelioid sarcoma are epithelial tumors of soft tissue of unknown histogenesis. These tumors are not thought to be closely related.

Mixed tumors and related skin adnexal tumors, and chordoma, a tumor mimicking the features of notochord cells, are also reviewed here.

SYNOVIAL SARCOMA

A synovial sarcoma is a specific soft tissue sarcoma with dual epithelial and mesenchymal differentiation. The tumor is not related to synovial lining cells, and its association with synovia, if any, is only incidental. The name is based on historical reasons, because the epithelial component of the biphasic tumors was once thought to replicate synovial slits. The typical t(X;18) translocation with SS18-SSX gene fusion is a useful diagnostic marker.

Demographics of Synovial Sarcoma

Synovial sarcomas are relatively common sarcomas, constituting from 7% to 10% of all soft tissue sarcomas. This tumor typically presents in young adults, with a median age of approximately 30 years and a mild male predominance.

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Modern Soft Tissue Pathology
Tumors and Non-Neoplastic Conditions
 , pp. 777 - 810
Publisher: Cambridge University Press
Print publication year: 2010

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