title: Desmoplastic small round cell tumors of the abdomen.
abstract: BACKGROUND
  Desmoplastic small round cell tumors (DSRCT) have been only recently identified.
  
  
  METHODS
  The authors report DSRCT in two pediatric patients (an 8-year-old boy and 12-year-old boy). In both patients, the initial diagnosis was rhabdomyosarcoma. The resistance to standard chemotherapy and radiation therapy prompted the authors to review the initial biopsy specimens and perform complementary immunophenotypic characterization.
  
  
  RESULTS
  These analyses revealed that the tumor cells were strongly positive for keratin epithelial marker antigen, desmin, vimentin, neurospecific enolase, and S100 protein, corresponding to pleomorphic differentiation, characteristic of DSRCT:
  
  
  CONCLUSIONS
  The authors suggest that extensive immunohistologic characterization be performed in all cases of small round cell tumors of the abdomen so that the diagnosis of DSRCT is not overlooked. These rare tumors are refractory to chemotherapy, and initial aggressive surgery is warranted.
authors.affiliation: Pediatric and Pathology Department, Centre Léon Bérard, France.
authors.forename: D
authors.initials: D
authors.lastname: Frappaz
authors.forename: E
authors.initials: E
authors.lastname: Bouffet
authors.forename: D
authors.initials: D
authors.lastname: Dolbeau
authors.forename: R
authors.initials: R
authors.lastname: Bouvier
authors.forename: C
authors.initials: C
authors.lastname: Carrie
authors.forename: D
authors.initials: D
authors.lastname: Louis
authors.forename: C
authors.initials: C
authors.lastname: Pondarre
authors.forename: E
authors.initials: E
authors.lastname: Tabone
authors.forename: T
authors.initials: T
authors.lastname: Philip
authors.forename: M
authors.initials: M
authors.lastname: Brunat-Mentigny
mesh_terms: D000951:Antigens, Neoplasm / Q000032:analysis; D002648:Child; D003893:Desmin / Q000032:analysis; D003937:Diagnosis, Differential; D006801:Humans; D007633:Keratins / Q000032:analysis; D008297:Male; D008562:Membrane Glycoproteins / Q000032:analysis; D018396:Mucin-1; D009361:Neoplasm Invasiveness; D010386:Pelvic Neoplasms / Q000473:pathology*; D010534:Peritoneal Neoplasms / Q000473:pathology*; D010751:Phosphopyruvate Hydratase / Q000032:analysis; D012208:Rhabdomyosarcoma / Q000473:pathology; D014746:Vimentin / Q000032:analysis
chemical_list: D000951:Antigens, Neoplasm; D003893:Desmin; D008562:Membrane Glycoproteins; D018396:Mucin-1; D014746:Vimentin; D007633:Keratins; D010751:Phosphopyruvate Hydratase