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Fusion-negative rhabdomyosarcoma with diffuse bony metastases and remarkable chemosensitivity
  1. Paige Kube1,
  2. Katie Parisio2,
  3. David Spencer Mangum3 and
  4. Jonathan Powell1
  1. 1Department of Pediatrics, Nemours Children's Hospital Delaware, Wilmington, Delaware, USA
  2. 2Department of Hematology/Oncology and Department of Palliative Medicine, Nemours Children's Hospital Delaware, Wilmington, Delaware, USA
  3. 3Division of Hematology/Oncology, Department of Pediatrics, University of Utah and Primary Children's Hospital, Salt Lake City, Utah, USA
  1. Correspondence to Dr Jonathan Powell; Jonathan.powell{at}


In this report, we describe the case of an adolescent male with an unusual case of fusion-negative, paratesticular alveolar rhabdomyosarcoma who presented with spontaneous tumour lysis syndrome and diffuse bony metastases throughout the axial and appendicular skeleton with additional significant bone marrow involvement. Both spontaneous tumour lysis syndrome and diffuse bony metastases are extremely unusual for rhabdomyosarcoma. On the backbone of standard vincristine, dactinomycin and cyclophosphamide (VAC) chemotherapy, the only local control was orchiectomy at 15 weeks, with no radiation administered due to the initially diffuse nature of the disease and rapid response to chemotherapy. Following 43 weeks of VAC, a year-long maintenance phase with pazopanib was given which was well tolerated. The patient remains in remission now 4 years after completion of therapy.

  • Paediatric oncology
  • Cancer intervention
  • Chemotherapy

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  • Contributors The authors confirm contribution to the paper as follows: conception and design: PK and KP; draft manuscript preparation: PK and KP; critical revision: DSM and JP. All authors reviewed and approved the final version of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.