Desmoplastic small round cell tumor of the pleura.
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.
Karavitakis
Emmanouil M
EM
Hematology-Oncology Unit, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Athens, Greece. ekaravitakis@yahoo.com
Moschovi
Maria
M
Stefanaki
Kalliopi
K
Karamolegou
Kalliopi
K
Dimitriadis
Euthimios
E
Pandis
Nikos
N
Karakousis
Constantine P
CP
Tzortzatou-Stathopoulou
Fotini
F
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
D014408:Biomarkers, Tumor; C106032:EWS1-WT1 fusion protein, human; D015514:Oncogene Proteins, Fusion