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Title: Desmoplastic small round cell tumor of major salivary glands: Report of 1 case and a review of the literature
Authors: Pang, B.
Leong, C.C.
Salto-Tellez, M. 
Petersson, F.
Keywords: Desmoplastic small round cell tumor
EWS-WT1 translocation
fine-needle aspiration cytology
salivary gland
Issue Date: Jan-2011
Citation: Pang, B., Leong, C.C., Salto-Tellez, M., Petersson, F. (2011-01). Desmoplastic small round cell tumor of major salivary glands: Report of 1 case and a review of the literature. Applied Immunohistochemistry and Molecular Morphology 19 (1) : 70-75. ScholarBank@NUS Repository.
Abstract: Desmoplastic small round cell tumor is a rare malignant neoplasm mostly occurring in the vicinity of or within the peritoneal cavity, and is uncommon in the head and neck region. Tumor location within a major salivary gland is exceptional. We report a case of a 41-year-old Chinese man with a history of diabetes mellitus and end-stage renal failure on peritoneal dialysis with a desmoplastic small round cell tumor occurring in the left submandibular gland. Fine-needle aspiration cytology showed variably cohesive clusters of small cells with hyperchromatic nuclei and fine granular chromatin. On histology the neoplasm displayed classic features of a desmoplastic small round cell tumor with angulated nests of small round blue cells in a fibromyxoid/desmoplastic stroma. Neoplastic cells were immunoreactive for cytokeratins (AE1/3), desmin (paranuclear dot-like), WT-1 (nuclear), epithelial membrane antigen, and CD56. EWS gene translocation and EWS-WT1 gene fusion were detected by fluorescence in situ hybridization and reverse transcriptase polymerase chain reaction, respectively. The case presented is the sixth case of and the oldest reported patient with a desmoplastic small round cell tumor occurring in a major salivary gland to date. Desmoplastic small round cell tumor should be considered in the differential diagnosis of a salivary gland neoplasm with a basaloid or small cell pattern on fine-needle aspiration cytology. Copyright © 2010 by Lippincott Williams & Wilkins.
Source Title: Applied Immunohistochemistry and Molecular Morphology
ISSN: 15412016
DOI: 10.1097/PAI.0b013e3181eec73c
Appears in Collections:Staff Publications

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