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A Case of Highly Aggressive Extraskeletal Myxoid Chondrosarcoma

Extraskeletal myxoid chondrosarcoma (EMC) is a soft tissue malignancy characterized by specific chromosomal abnormalities involving the TEC gene. This disease has historically been considered largely indolent both histologically and clinically. Rarer subsets of EMC exist that demonstrate aggressive...

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Detalles Bibliográficos
Autores principales: Mitchell, Aaron P., Poiesz, Michael, Leung, Allen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3177793/
https://www.ncbi.nlm.nih.gov/pubmed/21941486
http://dx.doi.org/10.1159/000331237
Descripción
Sumario:Extraskeletal myxoid chondrosarcoma (EMC) is a soft tissue malignancy characterized by specific chromosomal abnormalities involving the TEC gene. This disease has historically been considered largely indolent both histologically and clinically. Rarer subsets of EMC exist that demonstrate aggressive histopathologic features and clinical behavior, though it remains unclear whether or not aggressive histopathology is predictive of outcome. Herein we present a case of EMC with aggressive histopathologic features that underwent rapid clinical progression despite initial treatment with curative intent. This case provides the context for a discussion of the existing literature regarding treatment, prognosis, pathology, and genetic/molecular features of EMC in general and aggressive EMC specifically.