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Veno-Occlusive Disease in Pediatric Patients Receiving Actinomycin D and Vincristine Only for the Treatment of Rhabdomyosarcoma

Objectives: Veno-occlusive disease (VOD) following standard chemotherapy has been reported in patients receiving vincristine actinomycin D, and cyclophosphamide for the treatment of Wilms tumor and more rarely rhabdomyosarcoma. The dose and schedule of administration of actinomycin D in patients with Wilms tumor and the increased dose of cyclophosphamide administered to patients with rhabdomyosarcoma have been considered the likely etiology for VOD. Methods: The authors report four cases of VOD in patients with rhabdomyosarcoma treated with vincristine and actinomycin D only. No risk factors for the development of VOD were identified. VOD was diagnosed clinically by the presence of at least two of three findings as defined by McDonald et al. VOD occurred after two to four doses of actinomycin D and approximately 7 to 14 days after the dose. All patients recovered with no evidence of permanent hepatic damage. Conclusions: VOD can occur in patients with "low-stage" rhabdomyosarcoma treated with vincristine and actinomycin D alone. Although chemotherapy-related VOD is a potentially severe disease, the outcome is good and resumption of chemotherapy is well tolerated.