Abstract

Background

Extraskeletal myxoid chondrosarcoma, a rare soft tissue sarcoma primarily found in the extremities, generally presents as a low-grade malignant tumor with favorable prognosis. There is very limited reporting of this tumor in the head and neck, especially of this size.

Methods and results

A 65-year-old male patient presented with a large neck mass (18 × 6 × 2 cm) occluding his airway and causing dyspnea. The patient had previously undergone a fine needle aspiration of his left neck that indicated a suspected mesenchymal tumor; however, an additional biopsy after tumor growth to 17.4 × 7.1 × 5.4 cm histopathologically confirmed extraskeletal myxoid chondrosarcoma.

Conclusions

Extraskeletal myxoid chondrosarcoma is a rare tumor of the head and neck. For this unique tumor, diagnosis depends on accurate histology, immunohistochemistry, tumor location, and growth pattern. It should remain in the differential diagnosis for patients presenting with soft tissue neck mass.