Taniguchi Ayumu, Kakuta Yoichi, Fujita Kazutoshi, Uemura Motohide, Kiuchi Hiroshi, Imamura Ryoichi, Miyagawa Yasushi, Nonomura Norio, Ueda Yutaka
The Department of Urology, Osaka University Graduate School of Medicine.
The Department of Obstetrics and Gynecology, Osaka University Graduate School of Medicine.
Hinyokika Kiyo. 2019 Nov;65(11):479-484. doi: 10.14989/ActaUrolJap_65_11_479.
We report a case of perivascular epithelioid cell tumor (PEComa) of the uterus associated with tuberous sclerosis complex discovered as a result of intraabdominal bleeding. A 54-year-old woman visited the department of obstetrics and gynecology with chief complaints of genital bleeding and abdominal pain lasting three weeks. Abdominal computed tomography (CT) revealed intraabdominal bleeding from the uterine corpus. She had a history of bilateral renal angiomyolipoma, which was treated with partial bilateral nephrectomy at age 25. Moreover, pulmonary lymphangioleiomyomatosis was diagnosed at age 43. Both conditions were detected by the CT scan during this hospitalization. Immediately after the CT scans, the patient entered a state of hemorrhagic shock, and we performed an emergency simple total hysterectomy. Histopathological examination on the resected specimen yielded a diagnosis of uterine perivascular epithelial cell tumor (PEComa). After making a definitive diagnosis of tuberous sclerosis complex (TSC), systemic evaluation was performed. As a result, multiple skin and intracranial lesions characteristic of TSC were identified and we stated administration of everolimus.
我们报告一例因腹腔内出血而发现的与结节性硬化症相关的子宫血管周上皮样细胞肿瘤（PEComa）。一名54岁女性因生殖器出血和腹痛持续三周为主诉就诊于妇产科。腹部计算机断层扫描（CT）显示子宫体部腹腔内出血。她有双侧肾血管平滑肌脂肪瘤病史，25岁时接受了双侧部分肾切除术。此外，43岁时被诊断为肺淋巴管平滑肌瘤病。此次住院期间通过CT扫描发现了这两种情况。CT扫描后不久，患者进入失血性休克状态，我们进行了急诊单纯全子宫切除术。对切除标本进行组织病理学检查，诊断为子宫血管周上皮样细胞肿瘤（PEComa）。在明确诊断为结节性硬化症（TSC）后，进行了全身评估。结果，发现了多个具有TSC特征的皮肤和颅内病变，我们开始给予依维莫司治疗。
