Karavitakis Emmanouil M, Moschovi Maria, Stefanaki Kalliopi, Karamolegou Kalliopi, Dimitriadis Euthimios, Pandis Nikos, Karakousis Constantine P, Tzortzatou-Stathopoulou Fotini
Hematology-Oncology Unit, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Athens, Greece.
Pediatr Blood Cancer. 2007 Sep;49(3):335-8. doi: 10.1002/pbc.20762.
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.
促结缔组织增生性小圆细胞肿瘤（DSRCT）是一种具有侵袭性的罕见肿瘤。通常表现为腹膜肿块，不过也有其他部位发病的病例报道。由于胸膜原发性DSRCT的病例报道少于10例，因此报告1例起源于胸膜的儿科病例很有意义。通过对EWS/WT-1融合基因产物进行分子检测确诊。化疗、放疗和手术切除的多学科治疗使无进展生存期超过了中位生存期，这表明这种传统方法可能对这种罕见且侵袭性很强的恶性肿瘤有效。
