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.
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http://dx.doi.org/10.3345/kjp.2015.58.12.505 | DOI Listing |
Front Med (Lausanne)
December 2024
Department of Nuclear Medicine, Peking University First Hospital, Beijing, China.
Background: Rhabdomyosarcoma (RMS), a rare pediatric soft tissue neoplasm, predominantly develops in late childhood and adolescence with no discernible gender bias. Alveolar rhabdomyosarcoma (ARMS) stems from mesenchymal cells and may develop most frequently in the trunk, extremities, and head/neck areas, while occurrences in the pelvic cavity are less frequent. The manifestation is typically characterized by a high rate of aggressive metastasis and a poor overall survival prognosis.
View Article and Find Full Text PDFWorld J Clin Oncol
December 2024
Department of Ophthalmology, The Sixth Affiliated Hospital of Jinan University, Dongguan 523000, Guangdong Province, China.
Background: Both rhabdomyosarcoma (RMS) and central retinal artery occlusion (CRAO) are rare medical diseases, and their simultaneous occurrence in the same patient is extraordinarily uncommon. This study presents a comprehensive overview of the clinical manifestations, diagnostic imaging results, and therapeutic interventions of a patient with both conditions.
Case Summary: In this report, we present a 30-year-old male who presented with significant protrusion, pain and vision loss and was diagnosed with RMS in the orbit and sinus with CRAO.
Virchows Arch
December 2024
Department of Pathology and Laboratory Medicine, Indiana University, 350 W 11st St, Indianapolis, IN, 46202, USA.
Rhabdomyosarcoma (RMS) is the most common soft tissue malignancy in childhood, accounting for 3% of all pediatric malignancies and 50% of all pediatric soft tissue sarcomas. In adolescents and young adults (AYA) however, RMS comprises only 6.5% of all soft tissue sarcomas.
View Article and Find Full Text PDFEClinicalMedicine
December 2024
University Hospital Frankfurt, Department for Children and Adolescents, Goethe University, Frankfurt am Main, Germany.
Background: Rhabdomyosarcoma and other soft tissue sarcomas (STS) with high-risk features are still associated with an unsatisfactory outcome. We evaluated the efficacy of oral maintenance therapy added at the end of standard therapy in patients with high-risk rhabdomyosarcoma and STS.
Methods: CWS-2007-HR was a multicentre, open-label, randomised controlled, phase 3 trial done at 87 centers in 5 countries.
Arkh Patol
December 2024
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia.
Unlabelled: Rhabdomyosarcomas (RMS) are one of the most common types of sarcomas in children and adolescents. The alveolar RMS subgroup is of particular interest because in some cases, the translocation of the and genes is combined with an amplification of the corresponding hybrid gene. According to literature data, the frequency of the translocation is 70-90% and the translocation 10-30%.
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