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Chap 15 - ATYPICAL LIPOMATOUS TUMOR AND LIPOSARCOMAS

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

Atypical lipomatous tumors and four clinicopathologically or genetically distinct types of liposarcomas, sarcomas with fatty differentiation, are discussed in this chapter. Table 15.1 reviews their clinicopathologic features. The diagnosis of liposarcoma is based on recognition of adipocytic atypia and the characteristic histologic patterns for each type of liposarcoma. Fat stains are not useful, because fat is present in other tumors, for example in malignant fibrous histiocytoma (MFH, undifferentiated sarcoma) and in nonsarcomatous tumors, such as renal cell carcinoma. Immunostains are of limited value. The present classification of liposarcoma has evolved especially by work by Enterline et al., Enzinger et al., and Evans.

Well-differentiated liposarcoma and atypical lipomatous tumor are considered synonymous in the World Health Organization (WHO) classification. In this text, however, their clinical discussion is separated so that subcutaneous lesions are designated as atypical lipomatous tumors, whereas morphologically similar intramuscular, inguinal, mediastinal, and intraabdominal (retroperitoneal) tumors are designated as well-differentiated liposarcomas because of their greater tendency to uncontrollable local recurrences and dedifferentiation. The latter refers to their evolution into a nonlipomatous sarcoma phenotype. Despite some tendencies for different morphology in subcutaneous versus deep tumors, there are no criteria that could morphologically distinguish an atypical lipomatous tumor from a well-differentiated liposarcoma. Both types are characterized by 12q13–15 amplification; this genomic segment includes genes such as murine double minute (MDM2).

Dedifferentiated liposarcoma is a transformed variant of well-differentiated liposarcoma, representing its histologic and biologic disease progression. This type is most common in the retroperitoneum.

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Modern Soft Tissue Pathology
Tumors and Non-Neoplastic Conditions
 , pp. 432 - 459
Publisher: Cambridge University Press
Print publication year: 2010

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