Mediastinal Extraskeletal Myxoid Chondrosarcoma
Chondrosarcoma of the extraskeletal soft tissues; Chordoid sarcoma; Chordoid tumor; EMC; Extraskeletal chondrosarcoma; Tenosynovial sarcoma
Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue neoplasm characterized by distinct histopathological and molecular features. Although its name implies cartilaginous differentiation, several studies have demonstrated neural or neuroendocrine differentiation in these tumors raising the possibility of polyphenotypic origin. In the latest World Health Organization (WHO) classification of soft tissue and bone tumors, the entity is therefore grouped among the lesions of “uncertain differentiation.” Extraskeletal myxoid chondrosarcoma is primarily a tumor of the deep soft tissues of the lower extremities and limb girdles. Rare tumors have been reported to arise in unusual anatomic locations such as the retroperitoneum, digits, or intracranial cavity. Only a single case has been reported to occur in the posterior...
References and Further Reading
- Ogura, K., Fujiwara, T., Beppu, Y., Chuman, H., Yoshida, A., Kawano, H., & Kawai, A. (2012). Extraskeletal myxoid chondrosarcoma: A review of 23 patients treated at a single referral center with long-term follow-up. Archives of Orthopaedic and Traumatic Surgery, 132, 1379–1386.CrossRefGoogle Scholar