背景:恶性肾外横纹肌样瘤(MERT)是一种罕见且高度转移的肿瘤,超过75%的患者在初次诊断后6个月内死亡,经常导致误诊和延误治疗。
方法:本文报道了一个主诉急性腹痛的16岁女孩。她接受了腹腔镜探查和切除活检,然后病理检查和免疫组化显示“肾外恶性横纹肌瘤”。“手术后一个月,她死于腹腔内出血和多器官功能障碍。
结论:MERT常被误诊,预后不良。手术和化疗通常有利于延长MERT患者的生存时间。
BACKGROUND: Malignant extrarenal rhabdoid tumor (MERT) is a rare and highly metastatic tumor, which is more than 75% of patients dying within 6 months of initial diagnosis, and it often leads to misdiagnosis and delayed treatment.
METHODS: This paper reports a 16-year-old girl who presented with the chief complaint of acute abdominal pain. She underwent laparoscopic exploration and excisional biopsy, then pathological examination and immunohistochemistry revealed \"extrarenal malignant rhabdomyoma.\" One month after operation, she died of intra-abdominal hemorrhage and multiple organ dysfunction.
CONCLUSIONS: MERT were often misdiagnosed and had a poor prognosis. The surgery and chemotherapy are usually beneficial to prolong the survival time of patients with MERT.