Clear cell sarcoma of soft tissue - neck

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Painless swelling of the right lower aspect of the neck.

Patient Data

Age: 25 years
Gender: Male

Well-circumscribed ovoid right lower cervical mass, posterolateral to the SCM muscle and anterior to the anterior scalenus muscle. It elicits an iso-to high signal to muscles on T1 and an inhomogeneous high signal on T2 with areas of restricted diffusion on DWI/ADC. The postcontrast sequences show a heterogeneous enhancement with central areas of necrosis and enhanced peripheral capsule.

No cervical lymphadenopathy was seen.

Case Discussion

The patient went on to have a complete surgical resection of the lesion with a histopathological exam that confirmed the diagnosis of clear cell sarcoma.

Clear cell sarcomas (CCS) of soft tissue are rare malignant mesenchymal tumors with melanocytic differentiation, accounting for <1% of all soft tissue neoplasms. Most frequently found in the younger adult population with women slightly more frequently affected than men.

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