title: Desmoplastic small round cell tumor of the pleura.
abstract: Desmoplastic small round cell tumor (DSRCT) is a rare neoplasm with aggressive behavior. Usually it presents as a peritoneal mass, although other cases in various locations have been described. Since less than 10 cases of primary DSRCT in the pleura have been described, it is of interest to report a pediatric case arising from the pleura. The diagnosis was confirmed by molecular detection of the EWS/WT-1 fusion gene product. Multidisciplinary treatment with chemotherapy, radiotherapy, and surgical resection resulted in a progression-free survival time above the median survival, suggesting that this conventional approach could prove effective for this rare and very aggressive malignancy.
authors.affiliation: Hematology-Oncology Unit, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Athens, Greece. ekaravitakis@yahoo.com
authors.forename: Emmanouil M
authors.initials: EM
authors.lastname: Karavitakis
authors.forename: Maria
authors.initials: M
authors.lastname: Moschovi
authors.forename: Kalliopi
authors.initials: K
authors.lastname: Stefanaki
authors.forename: Kalliopi
authors.initials: K
authors.lastname: Karamolegou
authors.forename: Euthimios
authors.initials: E
authors.lastname: Dimitriadis
authors.forename: Nikos
authors.initials: N
authors.lastname: Pandis
authors.forename: Constantine P
authors.initials: CP
authors.lastname: Karakousis
authors.forename: Fotini
authors.initials: F
authors.lastname: Tzortzatou-Stathopoulou
mesh_terms: D014408:Biomarkers, Tumor; D002648:Child; D003131:Combined Modality Therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018193:Neoplasms, Complex and Mixed / Q000473:pathology / Q000628:therapy; D015514:Oncogene Proteins, Fusion; D010997:Pleural Neoplasms / Q000473:pathology / Q000628:therapy
chemical_list: D014408:Biomarkers, Tumor; C106032:EWS1-WT1 fusion protein, human; D015514:Oncogene Proteins, Fusion