Background: Desmoplastic small round cell tumor (DSRCT) is a recently described neoplasm characterized by aggressive biology, occurrence in body cavities, expression of antigens from multiple cell lineages and a specific translocation between chromosomes 11 and 22. Most of the published information on this neoplasm is histologic. The case presented here enabled presentation of the cytomorphologic and immunocytochemical features of aspirated cytologic material obtained from this unique tumor.
Case: The cytologic, histologic, radiologic and clinical features of a DSRCT from a 17-year-old patient are presented. Although the initial diagnosis in this case was made on histology, recurrence was proven by fine needle aspiration biopsy (FNAB).
Conclusion: The presence of sheets or clusters of small round malignant cells, associated with shards of dense fibroconnective tissue, in FNAB should lead the cytologist to consider the diagnosis of DSRCT.