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 consider resuming chemotherapy with a plan to complete the full regimen while observing the liver closely, including remaining VAC/VI and maintenance. Additionally, consideration for continuing defibrotide during the remainder of chemotherapy could be given, especially during VAC cycles given the increased hepatotoxicity risk of actinomycin.

If this patient was enrolled in a study, I also suggest a discussion with the study chair regarding this toxicity and determining if they truly need to be taken off it.