Department of Pediatrics, Nemours Children's Hospital Delaware, Wilmington, Delaware, USA.
Department of Hematology/Oncology and Department of Palliative Medicine, Nemours Children's Hospital Delaware, Wilmington, Delaware, USA.
BMJ Case Rep. 2022 Aug 29;15(8):e250236. doi: 10.1136/bcr-2022-250236.
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.
在本报告中，我们描述了一例融合阴性、副睾区肺泡横纹肌肉瘤的青少年男性病例，该患者表现为自发性肿瘤溶解综合征和弥漫性骨转移，累及轴骨和附肢骨，骨髓广泛受累。自发性肿瘤溶解综合征和弥漫性骨转移对横纹肌肉瘤来说极其罕见。在标准长春新碱、放线菌素 D 和环磷酰胺（VAC）化疗的基础上，唯一的局部控制手段是 15 周时进行的睾丸切除术，由于疾病最初呈弥漫性且对化疗反应迅速，因此未进行放疗。在接受 VAC 治疗 43 周后，给予帕唑帕尼进行为期一年的维持治疗，患者耐受性良好。在完成治疗后 4 年，患者仍处于缓解期。
