Fujiwara Maki, Hoshiyama Akihiro, Takahashi Takehiro, Takashima Yasushi, Kohno Yuka, Ueda Masakatsu, Kawanishi Hiroaki, Okumura Kazuhiro
The Department of Urology, Tenri Hospital.
Hinyokika Kiyo. 2020 Jul;66(7):229-234. doi: 10.14989/ActaUrolJap_66_7_229.
A 37-year-old man with no symptoms or family history of tuberous sclerosis complex presented to our hospital with abdominal pain in 2013. Abdominal computed tomography (CT) revealed a rupture in the right renal angiomyolipoma (AML) having a maximum diameter of 7 cm. He had undergone a transarterial embolization. Follow-up CT showed an increase in the size of the right tumor to 11 cm, and therefore, right nephrectomy was performed in 2016. The diagnosis of epithelioid AML (EAML) was confirmed. In 2019, he was diagnosed with a solitary tumor near right-sided transverse colon, which was resected and showed recurrence of EAML. He was disease-free 6 months after surgery. EAML has malignant potential, with 30-50% of reported EAML cases resulting in local recurrence or distant metastasis. Previous recurrence or metastasis may occur 0.25-12 years postoperatively. Furthermore, multiple and unresectable recurrences or metastases, arising early in the postoperative period may lead to a poor outcome. Therefore, close and long-term follow-up is required.
一名37岁男性,无结节性硬化症复合体的症状或家族史,2013年因腹痛就诊于我院。腹部计算机断层扫描(CT)显示右肾血管平滑肌脂肪瘤(AML)破裂,最大直径为7cm。他接受了经动脉栓塞术。随访CT显示右肿瘤大小增加至11cm,因此,2016年进行了右肾切除术。上皮样AML(EAML)的诊断得到证实。2019年,他被诊断出右侧横结肠附近有一个孤立肿瘤,该肿瘤被切除,显示为EAML复发。术后6个月他无疾病。EAML具有恶性潜能,报告的EAML病例中有30-50%会导致局部复发或远处转移。先前的复发或转移可能在术后0.25-12年发生。此外,术后早期出现的多次且无法切除的复发或转移可能导致不良预后。因此,需要密切和长期的随访。