Miura Shimpei, Kijima Noriyuki, Fujimori Nagisa, Nakagawa Tomoyoshi, Nakagawa Ryota, Tachi Tetsuro, Okita Yoshiko, Kanemura Yonehiro, Nakajima Shin, Mano Masayuki, Kishima Haruhiko, Ozawa Kentaro, Fujinaka Toshiyuki
Department of Neurosurgery, Osaka National Hospital, National Hospital Organization, Osaka, Osaka, Japan.
Department of Neurosurgery, Osaka University, Graduate School of Medicine, Suita, Osaka, Japan.
NMC Case Rep J. 2020 Sep 17;7(4):189-193. doi: 10.2176/nmccrj.cr.2019-0292. eCollection 2020 Sep.
Extramammary Paget's disease (EMPD) is a rare form of neoplasm. Metastasis of EMPD to locations other than lymph nodes and intra-epithelial regions is rare; there are a limited number of case reports of metastases to the liver, lung, bone, and brain. We present a rare case of EMPD that metastasized to the brain and was treated with surgical resection. A 66-year-old man presented with a small palpable mass in the scrotum. After 5 years of observation, he was diagnosed with EMPD that metastasized to the lymph nodes and lung. Tumor resection and postoperative chemotherapy were performed. Six months after the last chemotherapy treatment, he presented with a right temporal lobe tumor and underwent surgical resection. Histopathological analysis revealed brain metastasis of EMPD. Three months after surgery, magnetic resonance imaging (MRI) showed local tumor recurrence, and intensity modulated radiation therapy (IMRT) (45 Gy/15 Fr) was performed. Although the metastatic brain tumor was well controlled, the primary tumor progressed. He was provided best supportive care and died 5 months after brain tumor resection. In this report, we present a rare case of brain metastasis of EMPD, treated with surgical resection, and histopathologically confirmed to be metastatic EMPD.
乳腺外佩吉特病（EMPD）是一种罕见的肿瘤形式。EMPD转移至淋巴结和上皮内区域以外部位的情况罕见；仅有少数关于转移至肝脏、肺、骨和脑的病例报告。我们报告一例罕见的EMPD脑转移病例，该病例接受了手术切除治疗。一名66岁男性阴囊出现一个可触及的小肿块。经过5年观察，他被诊断为EMPD伴淋巴结和肺转移。进行了肿瘤切除及术后化疗。最后一次化疗6个月后，他出现右侧颞叶肿瘤并接受了手术切除。组织病理学分析显示为EMPD脑转移。术后3个月，磁共振成像（MRI）显示局部肿瘤复发，遂进行调强放射治疗（IMRT）（45 Gy/15次分割）。尽管转移性脑肿瘤得到良好控制，但原发肿瘤进展。给予其最佳支持治疗，患者在脑肿瘤切除术后5个月死亡。在本报告中，我们呈现一例罕见的EMPD脑转移病例，经手术切除治疗，并经组织病理学证实为转移性EMPD。
