# A Case Report of a Patient Presenting With Extra-skeletal Myxoid Chondrosarcoma

**Authors:** Kabhisha Gunasekaran, Vindya Johnston, Daniel Wong, Rupert Hodder, Andrew Coveney

PMC · DOI: 10.7759/cureus.100687 · Cureus · 2026-01-03

## TL;DR

This case report describes a patient with a rare type of sarcoma called extra-skeletal myxoid chondrosarcoma, highlighting its characteristics, diagnosis, and treatment.

## Contribution

The paper presents a detailed clinical case of EMC, emphasizing its unique molecular marker and management challenges.

## Key findings

- EMC is characterized by the fusion gene EWSR1::NR4A3, a key diagnostic marker.
- Surgical removal is the primary treatment, while non-surgical options are used for recurrence or metastasis.
- EMC has a high risk of local recurrence and distant metastasis despite treatment.

## Abstract

Extra-skeletal myxoid chondrosarcoma (EMC) is a type of rare sarcoma of uncertain differentiation. This malignancy is marked by the growth of primitive chondroid cells forming multiple nodules within a rich myxoid matrix. It is distinguished by a specific translocation resulting in the fusion gene EWSR1::NR4A3, which is a distinctive molecular hallmark of EMC. EMC is categorised as an intermediate-grade tumour and is distinguished by a prolonged clinical course with a high likelihood of local recurrence and distant metastasis. Surgical treatment is the only option for a cure for EMC, while non-surgical treatments are typically considered for recurrent or distant disease. This case report discusses an interesting case of a patient with EMC and further elaborates on the history, examination, imaging, pathological findings, and management of EMC.

## Linked entities

- **Genes:** EWSR1 (EWS RNA binding protein 1) [NCBI Gene 2130], NR4A3 (nuclear receptor subfamily 4 group A member 3) [NCBI Gene 8013]
- **Diseases:** extra-skeletal myxoid chondrosarcoma (MONDO:0012825), sarcoma (MONDO:0005089)

## Full-text entities

- **Diseases:** sarcoma (MESH:D012509), malignancy (MESH:D009369), metastasis (MESH:D009362), EMC (MESH:D002813)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

5 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12863220/full.md

## References

13 references — full list in the complete paper: https://tomesphere.com/paper/PMC12863220/full.md

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Source: https://tomesphere.com/paper/PMC12863220