BACKGROUND: The presence of the EWS-WT1 gene fusion transcript (GFT) is characteristic of desmoplastic small round cell tumor (DSRCT), a rare and very aggressive disease for which the treatment has not yet been clearly standardized. METHODS: This was a retrospective national multicenter analysis of young patients <30 years with tumors expressing the EWS-WT1-GFT, designed to determine whether extensive surgery had an impact on survival. RESULTS: Between 1995 and 2006, a EWS-WT1-GFT was detected in the tumors of 38 patients, 17 (44.7%) of whom had had a different initial pathologic diagnosis prior to molecular testing. Mean age was 13.2 years (range: 4-29.7 years). Only 9 patients (24%) had localized disease. Treatment was heterogeneous. Nine patients had "limited" surgical resections and 22 underwent "extensive" surgery. Two-year event-free survival and overall survival were 14.4% and 50%, respectively. Among the five patients who were alive in complete remission, four had undergone extensive and complete surgery. CONCLUSIONS: Detection of the EWS-WT1-GFT plays a major role in the diagnosis of DSRCT. No survival difference was observed according to extent of surgery, but complete surgery seemed to offer the best chance of long-term survival. High-dose chemotherapy or local radiotherapy did not appear to improve survival in this retrospective analysis, but larger prospective studies are needed to provide definitive conclusions on the role of these treatments.
BACKGROUND: The presence of the EWS-WT1 gene fusion transcript (GFT) is characteristic of desmoplastic small round cell tumor (DSRCT), a rare and very aggressive disease for which the treatment has not yet been clearly standardized. METHODS: This was a retrospective national multicenter analysis of young patients <30 years with tumors expressing the EWS-WT1-GFT, designed to determine whether extensive surgery had an impact on survival. RESULTS: Between 1995 and 2006, a EWS-WT1-GFT was detected in the tumors of 38 patients, 17 (44.7%) of whom had had a different initial pathologic diagnosis prior to molecular testing. Mean age was 13.2 years (range: 4-29.7 years). Only 9 patients (24%) had localized disease. Treatment was heterogeneous. Nine patients had "limited" surgical resections and 22 underwent "extensive" surgery. Two-year event-free survival and overall survival were 14.4% and 50%, respectively. Among the five patients who were alive in complete remission, four had undergone extensive and complete surgery. CONCLUSIONS: Detection of the EWS-WT1-GFT plays a major role in the diagnosis of DSRCT. No survival difference was observed according to extent of surgery, but complete surgery seemed to offer the best chance of long-term survival. High-dose chemotherapy or local radiotherapy did not appear to improve survival in this retrospective analysis, but larger prospective studies are needed to provide definitive conclusions on the role of these treatments.
Authors: Vivek Subbiah; Salah-Eddine Lamhamedi-Cherradi; Branko Cuglievan; Brian A Menegaz; Pamela Camacho; Winston Huh; Vandhana Ramamoorthy; Pete M Anderson; Raphael E Pollock; Dina C Lev; Wei Qiao; Mary Frances McAleer; Robert S Benjamin; Shreyaskumar Patel; Cynthia E Herzog; Najat C Daw; Barry W Feig; Alexander J Lazar; Andrea Hayes-Jordan; Joseph A Ludwig Journal: Clin Cancer Res Date: 2018-06-05 Impact factor: 12.531
Authors: Christopher J Forlenza; Brian H Kushner; Nancy Kernan; Farid Boulad; Heather Magnan; Leonard Wexler; Suzanne L Wolden; Michael P LaQuaglia; Shakeel Modak Journal: Sarcoma Date: 2015-04-07
Authors: R J Cook; Z Wang; M Arora; H M Lazarus; K A Kasow; M A Champagne; W Saber; K M van Besien; G A Hale; E A Copelan; M Elmongy; N T Ueno; B N Horn; S Slavin; M R Bishop; E A Stadtmauer Journal: Bone Marrow Transplant Date: 2012-04-02 Impact factor: 5.483
Authors: Yunyun Jiang; Vivek Subbiah; Filip Janku; Joseph A Ludwig; Aung Naing; Robert S Benjamin; Robert E Brown; Pete Anderson; Razelle Kurzrock Journal: PLoS One Date: 2014-08-13 Impact factor: 3.240