Bárdos Dávid, Molnár Mária Judit, Dudás Ibolyka, Tuza Sebestyén, Szijártó Attila, Hahn Oszkár
1st Department of Surgery and Interventional Gastroenterology, Semmelweis University, Budapest, 1085, Budapest, Üllői út 78, Hungary.
Institute of Genomic Medicine and Rare Disorders, Semmelweis University, 1085, Budapest, Üllői út 78, Hungary.
Ann Med Surg (Lond). 2021 Apr 4;65:102269. doi: 10.1016/j.amsu.2021.102269. eCollection 2021 May.
Rhabdomyolysis is a syndrome characterized by a rapid necrosis of muscle fibers and the release of muscle-derived metabolic products into the circulatory system. A rare cause of rhabdomyolysis is paraneoplastic polymyositis.
A 67-year-old man was diagnosed with paraneoplastic polymyositis and rhabdomyolysis caused by hepatocellular carcinoma (HCC). Intravenous steroid was used as a symptomatic therapy for rhabdomyolysis, and the tumour was removed by left hemihepatectomy to treat the underlying cause. After muscle strength gradually improved, steroid therapy was discontinued. The patient was reoperated multiple times due to bleeding and bile leakage. Following the operations, his overall state and muscle strength further improved. Despite that, the patient's condition worsened again, and eventually, he died of candida albicans pneumonia and sepsis.
HCC is an extremely rare cause of paraneoplastic polymyositis and rhabdomyolysis. Treatment is challenging, as none of the few available case reports record long term survival and less than half of the reports record muscle strength improvement. In our case, the patient was treated with systemic steroid therapy and resection of the tumour. The patient's muscle strength temporarily improved, but subsequently, the patient died.
Our case confirms the importance of a definitive treatment of HCC, as we achieved a significant improvement in muscle strength by removing the tumour. On the other hand, our paper highlights the dangers of double-sided steroid therapy, which, combined with the essential, effective treatment of rhabdomyolysis, may have contributed to the development of postoperative complications and candida sepsis leading to death.
横纹肌溶解症是一种以肌纤维快速坏死以及肌肉衍生的代谢产物释放进入循环系统为特征的综合征。副肿瘤性多发性肌炎是横纹肌溶解症的一种罕见病因。
一名67岁男性被诊断为副肿瘤性多发性肌炎及由肝细胞癌（HCC）引起的横纹肌溶解症。静脉注射类固醇被用作横纹肌溶解症的对症治疗，并且通过左半肝切除术切除肿瘤以治疗潜在病因。在肌肉力量逐渐改善后，停用了类固醇治疗。患者因出血和胆漏多次接受再次手术。手术后，他的整体状况和肌肉力量进一步改善。尽管如此，患者的病情再次恶化，最终死于白色念珠菌肺炎和脓毒症。
HCC是副肿瘤性多发性肌炎和横纹肌溶解症极其罕见的病因。治疗具有挑战性，因为现有的少数病例报告均未记录长期生存情况，且不到一半的报告记录了肌肉力量的改善。在我们的病例中，患者接受了全身类固醇治疗和肿瘤切除。患者的肌肉力量暂时改善，但随后患者死亡。
我们的病例证实了对HCC进行确定性治疗的重要性，因为通过切除肿瘤我们使肌肉力量有了显著改善。另一方面，我们的论文强调了双侧类固醇治疗的危险性，这种治疗与横纹肌溶解症的必要有效治疗相结合，可能导致了术后并发症和念珠菌败血症的发生，进而导致死亡。
