Estela-Zape Jose Luis, Sanclemente-Cardoza Valeria, Arzayus-Patiño Leonardo, Noreña-Buitrón Lizeth Dayana, Espinosa María Alejandra
Faculty of Health, Universidad Santiago de Cali, Cali, Colombia.
Faculty of Health Sciences, Fundación Universitaria Maria Cano, Cali, Colombia.
Respir Med Case Rep. 2025 Apr 17;55:102211. doi: 10.1016/j.rmcr.2025.102211. eCollection 2025.
Alveolar rhabdomyosarcoma (ARMS) in adults is a rare condition with a poor prognosis compared to other subtypes. The management of this pathology is complex due to the lack of standardized guidelines and the limited response to multimodal treatments, including chemotherapy, radiotherapy, and surgery. We report the case of a 40-year-old male with stage IV ARMS and pulmonary metastasis, who underwent resection of right supraclavicular sarcoma and cervical lymph node dissection. He was later referred for palliative chemotherapy following an acute clinical presentation lasting two days, characterized by right facial edema, inflammation, ulceration, and severe pain. Initial treatment included ampicillin/sulbactam, trimethoprim/sulfamethoxazole, and dexamethasone. Chemotherapy with doxorubicin and ifosfamide was delayed due to thrombocytopenia, elevated D-dimer and fibrinogen levels, and an echocardiographic finding that required differentiation between thrombus and metastatic lesion. Anticoagulation with fondaparinux and transfusions were initiated, and the patient received 10 sessions of radiotherapy. During hospitalization, the patient developed orthopnea, pleural effusion, superior vena cava syndrome, and hemodynamic deterioration, necessitating vasopressor support and mechanical ventilation. Despite these interventions, the patient progressed to refractory shock, severe hypoxemia, and died from cardiac arrest. This case highlights the challenges in managing ARMS in adults with severe thrombocytopenia.
成人肺泡横纹肌肉瘤(ARMS)是一种罕见疾病,与其他亚型相比预后较差。由于缺乏标准化指南以及对包括化疗、放疗和手术在内的多模式治疗反应有限,这种病理类型的管理较为复杂。我们报告了一例40岁男性IV期ARMS伴肺转移患者,该患者接受了右锁骨上肉瘤切除术和颈部淋巴结清扫术。在出现持续两天的急性临床表现后,其特征为右面部水肿、炎症、溃疡和剧痛,随后他被转诊接受姑息化疗。初始治疗包括氨苄西林/舒巴坦、甲氧苄啶/磺胺甲恶唑和地塞米松。由于血小板减少、D-二聚体和纤维蛋白原水平升高以及超声心动图检查发现需要鉴别血栓和转移灶,多柔比星和异环磷酰胺化疗被推迟。开始使用磺达肝癸钠抗凝和输血,患者接受了10次放疗。住院期间,患者出现端坐呼吸、胸腔积液、上腔静脉综合征和血流动力学恶化,需要血管活性药物支持和机械通气。尽管采取了这些干预措施,患者仍进展为难治性休克、严重低氧血症,最终死于心脏骤停。该病例突出了成人严重血小板减少的ARMS管理中的挑战。