Alveolar rhabdomyosarcoma with massive disseminated intravascular coagulopathy treated with systemic chemotherapy

Abstract

It is uncommon for pediatric patients with rhabdomyosarcoma to present with clinical and/or laboratory features of disseminated intravascular coagulation (DIC). We report a case of metastatic alveolar rhabdomyosarcoma with severe bleeding because of DIC in a 13-year-old boy. He experienced persistent oozing at the site of a previous operation, gross hematuria, and massive epistaxis. Two weeks after initiating combination chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide, the patients' laboratory indications of DIC began to resolve. During this period, the patient received massive blood transfusion of a total of 311 units (26 units of red blood cells, 26 units of fresh frozen plasma, 74 units of platelet concentrates, 17 units of single donor platelets, and 168 units of cryoprecipitate), antithrombin-III and a synthetic protease inhibitor. Despite chemotherapy and radiation therapy, he died 1 year later because of disease progression. In children with metastatic rhabdomyosarcoma and massive DIC, prompt chemotherapy and aggressive supportive care is important to decrease malignancy-triggered procoagulant activities.

Alveolar rhabdomyosarcoma with bone marrow metastasis causes disseminated intravascular coagulation

“…The current case presented with persistent oozing at the site of puncture after BM biopsy. Aida et al and Yoon et al reported that DIC rapidly worsened after surgery or biopsy in patients with ARMS metastatic to BM. Therefore, invasive procedures and surgery require caution in these patients.…”

“…Severe DIC can occur in ARMS with BM metastasis and may be triggered by biopsy or invasive surgery . Exposure of the subendothelial tissue factor after trauma to the tumor blood vessels causes the release of cytokines with procoagulant activity.…”