Desmoplastic small round cell tumor of the pleura
| Autor: | Constantine P. Karakousis, Maria Moschovi, Nikos Pandis, Euthimios Dimitriadis, Kalliopi Karamolegou, F. Tzortzatou-Stathopoulou, Kalliopi Stefanaki, Emmanouil M. Karavitakis |
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| Rok vydání: | 2006 |
| Předmět: |
Surgical resection
Male Pathology medicine.medical_specialty Desmoplastic small-round-cell tumor Oncogene Proteins Fusion medicine.medical_treatment Pleural Neoplasms Malignancy Blood cancer medicine Biomarkers Tumor Neoplasm Humans Child Chemotherapy business.industry Hematology medicine.disease Combined Modality Therapy Magnetic Resonance Imaging Neoplasms Complex and Mixed Radiation therapy Oncology Pediatrics Perinatology and Child Health business Median survival |
| Zdroj: | Pediatric bloodcancer. 49(3) |
| ISSN: | 1545-5009 |
| Popis: | 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. Pediatr Blood Cancer 2007;49:335–338. © 2006 Wiley-Liss, Inc. |
| Databáze: | OpenAIRE |
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