Kambe Takanari, Yamasaki Toshinari, Nagoshi Akihiko, Fujiwara Tasuku, Mine Yuta, Hagimoto Hiroki, Hattori Yuto, Abe Yohei, Yamashita Daisuke, Tsutsumi Naofumi, Kawakita Mutsushi
The Department of Urology, Kobe City Medical Center General Hospital.
The Department of Pathology, Kobe City Medical Center General Hospital.
Hinyokika Kiyo. 2023 Jun;69(6):151-155. doi: 10.14989/ActaUrolJap_69_6_151.
Contrast-enhanced computed tomography (CT) revealed a multilocular cystic mass extending from the level of the renal artery origin to the internal and external iliac artery regions in a woman in her 40s who presented with vomiting and diarrhea. A percutaneous biopsy was performed, and histopathological examination revealed bundle-like proliferations of spindle-shaped cells with oval nuclei in acidophilic cytoplasm. Immunohistochemical staining was positive for HMB-45, alpha-smooth muscle actin, E-cadherin, and estrogen and progesterone receptors; the provisional diagnosis was perivascular epithelioid cell tumor. Considering the patient's age and sex, the final diagnosis was primary retroperitoneal lymphangioleiomyomatosis (LAM). She did not meet the diagnostic criteria for tuberous sclerosis complex and was considered to have sporadic LAM. As complete surgical resection was considered to be impossible and no lung lesions, which indicate poor prognosis, were observed, we decided to keep her under surveillance. The patient was asymptomatic, with no significant changes on imaging for 6 months.
增强计算机断层扫描（CT）显示，一名40多岁出现呕吐和腹泻症状的女性患者，有一个多房囊性肿块，从肾动脉起始水平延伸至髂内、外动脉区域。进行了经皮活检，组织病理学检查显示梭形细胞呈束状增生，细胞核呈椭圆形，胞质嗜酸性。免疫组化染色HMB-45、α-平滑肌肌动蛋白、E-钙黏蛋白以及雌激素和孕激素受体均为阳性；初步诊断为血管周上皮样细胞瘤。考虑到患者的年龄和性别，最终诊断为原发性腹膜后淋巴管平滑肌瘤病（LAM）。她不符合结节性硬化症复合体的诊断标准，被认为患有散发性LAM。由于认为完全手术切除不可能，且未观察到提示预后不良的肺部病变，我们决定对她进行监测。患者无症状，6个月影像学检查无明显变化。
