Abstract
Desmoplastic small round cell tumour (DSRCT) is a rare disease usually affecting young males. There are no other articles with a sub-maxillary location. The tumour consists of nests and masses of undifferentiated small round cells embedded in a desmoplastic stroma. The coexpression of epithelial, muscular and neuronal antigens distinguishes this entity from other small round cell tumours. The t(11;22) (p13;q12) translocation is a recurrent characteristic of this type of tumour.We report a case of desmoplastic small round cell tumour of the sub-maxillary gland, with an evolution of 8 months, affecting a 36-year-old male. He suffered chronic lymphatic leukaemia 5 years ago and needed a bone marrow transplant. There was a 4×3cm tumour. There were no signs of malignancy on the CT scan. A right submaxillectomy was performed. The pathology analysis gave a diagnosis of DSRCT. Post-surgical radiotherapy was given.The definitive diagnosis was reached using immunohistochemical techniques, such as polyphenotypical differentiation (epithelial, mesenchymal, and neural), and by demonstration of translocation (11;22) (p13;q12). Sub-maxillary location is very rare.
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