ENT case 5. Parotid mass from a 40 YO male patient

Multiple neck lymph nodes are positive for tumor:

Next generation squence showed EWSR1-WT1 fusion.

Diagnosis: Desmoplastic Small Round Cell Tumor

DSRCT is extremely rare in salivary gland, with less than 7 cases reported in the literature. It is possible that majority of salivary gland DSRCT cases are not recognized as DSRCT and misdiagnosed as poorly differentiated/unclassified carcinoma.