DSRCT Articles
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[edit] Sarcoma Journal 2008
Volume 2008, Article ID 261589, 9 pages doi:10.1155/2008/261589
CaseReport
DollyAguilera, 1AndreaHayes-Jordan, 2PeterAnderson,1 ShiaoWoo,3 MargaretPearson,1 andHollyGreen2
1 Department of Pediatrics, University of Texas MDAnderson Cancer Center,Houston,TX77030, USA
2 Department of Surgical Oncology, University of Texas MDAnderson Cancer Center, Houston,TX 77030, USA
3 Department of Radiation Oncology, University of Texas MDAnderson Cancer Center,Houston,TX 77030, USA
Correspondence should be addressed to Peter Anderson, pmanders@mdanderson.org
Received 14 December 2007; Accepted 29 April 2008
Desmoplastic Small Round Cell Tumor (DSRCT) has a very poor prognosis. This report illustrates novel chemotherapy and local control interventions in a 5-year old patient. The patient was treated in the outpatient setting, achieved remission, with excellent quality of life. The patient presented with massive ascites and >1000 abdominal tumors. Neoadjuvant chemotherapy included vincristine (1.5mg/m2), ifosfamide (3 g/m2 /day ×3), dexrazoxane/doxorubicin (750/75 mg/m2), and etoposide (150 mg/m2). Continuous hyperthermic peritoneal perfusion (CHPP) with cisplatin (100 mg/m2) was given after extensive cytoreductive surgery. This was followed by irinotecan (10 mg/m2/day ×5 ×2 weeks) + temozolomide monthly ×2, then abdominal radiation 30 Gy with simultaneous temozolomide (100 mg/m2/day ×5). A total of 12 cycles of irinotecan and temozolamide were given. Except for initial chemotherapy, subsequent courses were in the outpatient setting. Focal retroperitoneal relapse at 18 months was treated with IMRT with bevacizumab (5 mg/kg) and 2 perihepatic metastases with radio frequency ablation/cryoablation followed by chronic outpatient maintenance chemotherapy (valproic acid, cyclophosphamide, and rapamycin). Almost 2 years from diagnosis, the patient maintained an excellent quality of life. This is a novel approach to the treatment of children with massive abdomino-pelvic DSRCT.
Copyright © 2008 Dolly Aguilera et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
