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How do you approach a patient with metastatic rhabdomyosarcoma complicated by sinusoidal obstruction syndrome (SOS) after neoadjuvant chemotherapy and radiation therapy?

Stage 4/group 4 due to dissemination in peritoneal fluid. Translocation negative; treated per intermediate risk protocol on study arm ARST1431 VAC/VI + temsirolimus. After 9 weeks, proceeded with R0 resection (intra-abdominal disease), followed by 1 month of whole abdomen radiation. Developed SOS with 3 fractions left, taken off study, and recovered on defibrotide.
1 Answer
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Pediatric Hematology/Oncology · Connecticut Children's Medcl Center
Answered on

This is a challenging and unfortunate complication of therapy. Based solely on the information provided and with only 3 fractions left, it sounds like the patient received the majority of radiation therapy, and I would forgo the remainder despite the recovery on defibrotide. However, I would conside...

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