Extraskeletal myxoid chondrosarcoma

Definition:

Extraskeletal myxoid chondrosarcoma (EMC) is a unique, rare soft-tissue tumor with prominent myxoid morphology. The tumor most commonly develops in deep parts of the proximal extremities and limb girdles in middle-aged adults, and there is a predilection for male patients. Currently, a chromosomal translocation t(9;22)(q22;q12) and the resultant fusion gene, EWSR1-NR4A3, has been defined in approximately 75% of EMC. More recently, another fusion gene, TAF15-NR4A3, resulting from t(9;17)(q22;q11.2), has been identified. The abnormal proteins resulting from these fusion genes aberrantly affect gene transcription and cellular signaling pathways thought to be responsible for initiating sarcoma formation.

References:

[1]. A M Oliveira, et al. Extraskeletal myxoid chondrosarcoma: a clinicopathologic, immunohistochemical, and ploidy analysis of 23 cases. Mod Pathol. 2000 Aug;13(8):900-8. [Content Brief]

[2]. G D Letson, et al. Genetic and molecular abnormalities in tumors of the bone and soft tissues. Cancer Control. 2001 May-Jun;8(3):239-51. [Content Brief]

[3]. Lee J Helman, et al. Mechanisms of sarcoma development. Nat Rev Cancer. 2003 Sep;3(9):685-94. [Content Brief]

[4]. S Okamoto, et al. Extraskeletal myxoid chondrosarcoma: a clinicopathologic, immunohistochemical, and molecular analysis of 18 cases. Hum Pathol. 2001 Oct;32(10):1116-24. [Content Brief]

[5]. Satoshi Kawaguchi, et al. Extraskeletal myxoid chondrosarcoma: a Multi-Institutional Study of 42 Cases in Japan. Cancer. 2003 Mar 1;97(5):1285-92. [Content Brief]

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