title: Desmoplastic small round cell tumor: Diagnostic dilemma and uncertain prognosis: Report of few cases.
abstract: Desmoplastic small round cell tumor (DSRCT) is rare and highly malignant neoplasm. DSRCT affects usually young males but can occur in adults also. Intra-abdominal pelvic region is the preferred site. Though confirmed by histology, immunohistochemistry (IHC) plays a key role in diagnosis. IHC profile is characteristic, it shows simultaneous expression of epithelial (epithelial membrane antigen (EMA) and cytokeratin (CK)), muscular (desmin), and neural (neuron-specific enolase) markers. Many cases of DSRCT are diagnosed as poorly differentiated carcinoma due to lack of proper panel of IHC. It is difficult to predict if there has been a true increase in incidence. Prognosis is uncertain in such an aggressive neoplasm as chemotherapy (CT) or radiotherapy (RT) shows various outcomes. Here in; we report four cases, all of which showed diagnostic dilemma and uncertain prognosis.
authors.forename: Palash Kumar
authors.initials: PK
authors.lastname: Mandal
authors.affiliation: Department of Pathology, Bankura Sammilani Medical College, Bankura, West Bengal, India.
authors.forename: Anindya
authors.initials: A
authors.lastname: Adhikari
authors.forename: Anuradha
authors.initials: A
authors.lastname: De
authors.forename: Santosh Kumar
authors.initials: SK
authors.lastname: Mondal
mesh_terms: D000008:Abdominal Neoplasms / Q000145:classification / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism; D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D058405:Desmoplastic Small Round Cell Tumor / Q000145:classification / Q000175:diagnosis* / Q000188:drug therapy / Q000378:metabolism; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D011379:Prognosis
chemical_list: D014408:Biomarkers, Tumor