Prabhu N, Osifodunrin N, Murphy D, Butler S, Hunter L E
Department of Paediatric Cardiology, Royal Hospital for Children, Glasgow, United Kingdom.
Department of Paediatric Oncology, Royal Hospital for Children, Glasgow, United Kingdom.
J Pediatr Intensive Care. 2018 Jun;7(2):90-93. doi: 10.1055/s-0037-1606574. Epub 2017 Sep 12.
Rhabdomyomas are histologically benign tumors known to be associated with tuberous sclerosis. The natural history predicts the majority of tumors to be asymptomatic and regress within the first year of life. We describe a neonate presenting on day 1 of life with cardiovascular collapse secondary to a massive rhabdomyoma. Surgical resection was excluded due to the extensive nature of the lesion and oral sirolimus, a mammalian target of rapamycin inhibitor, was commenced to promote tumor regression. The patient developed intractable arrhythmias requiring extracorporeal life support during therapy.
横纹肌瘤是组织学上的良性肿瘤，已知与结节性硬化症相关。其自然病程预示大多数肿瘤无症状，并在出生后第一年内消退。我们描述了一名新生儿，出生第1天因巨大横纹肌瘤继发心血管衰竭。由于病变范围广泛，排除了手术切除，开始口服西罗莫司（一种雷帕霉素哺乳动物靶点抑制剂）以促进肿瘤消退。患者在治疗期间出现顽固性心律失常，需要体外生命支持。
