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Histopathological features and genomic alterations
Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 21, 2025 · Captured 2026-09-09.
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Context: Treatment of Chondro-osseous Tumors / Extraskeletal Mesenchymal Chondrosarcoma
Mesenchymal chondrosarcoma is a rare tumor characterized by small round cells and hyaline cartilage, and it more commonly affects young adults.
Mesenchymal chondrosarcoma has been associated with a consistent chromosomal rearrangement. A retrospective analysis of cases of mesenchymal chondrosarcoma identified a HEY1::NCOA2 gene fusion in 10 of 15 tested specimens.[1] This gene fusion was not associated with chromosomal changes that could be detected by karyotyping. In one instance, translocation t(1;5)(q42;q32) was identified in a case of mesenchymal chondrosarcoma and shown to be associated with a novel IRF2BP::CDX1 gene fusion.[2]
A retrospective study analyzed 13 patients with mesenchymal chondrosarcoma, all with confirmed HEY1::NCOA2 gene fusions.[3]
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The median age of presentation was 19 years.
Five patients with mesenchymal chondrosarcomas (39%) had an intraosseous presentation (skull, maxilla, palate, and mandible), while the remaining eight cases occurred in the brain/meninges, orbit, and nasal cavity.
Microscopically, head and neck mesenchymal chondrosarcomas were characterized by primitive round cells arranged in a distinctive nested architecture and a rich staghorn vasculature.
A cartilaginous component of hyaline cartilage islands and/or single chondrocytes were present in 69% of cases.
A combined immunoprofile of CD99(+)/SATB2(+)/CD34(-)/STAT6(-) was typically noted.
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