Jayapalan Ronie Romelean, Bahuri Nor Faizal Ahmad, Mun Kein Seong, Narayanan Vairavan
Division of Neurosurgery, Department of Surgery, University Malaya Medical Centre, Kuala Lumpur, Malaysia.
Department of Pathology, University Malaya Medical Centre, Kuala Lumpur, Malaysia.
SAGE Open Med Case Rep. 2019 Feb 4;7:2050313X19828539. doi: 10.1177/2050313X19828539. eCollection 2019.
Perivascular epithelioid cell tumour is a rare mesenchymal tumour with distinct immunohistochemical profile. While it is known to occur in various anatomical sites, the central nervous system had always been a protected site for primary or secondary perivascular epithelioid cell tumours. We describe a 61-year-old lady who presented with symptoms of raised intracranial pressure, 3 months after the resection of duodenal and thoracic tumours which were histologically consistent with perivascular epithelioid cell tumour. She was investigated and then subsequently subjected to resection of two metastatic intracranial lesions. The radiological, intraoperative as well as histopathological findings of the metastatic lesions are discussed. Metastatic perivascular epithelioid cell tumour of the brain is extremely rare. However, patients who are stratified as high risk for recurrence or metastases should undergo an early magnetic resonance imaging/computed tomography of the brain in addition to a whole-body positron emission tomography scan, to allow for early detection and management of these tumours.
血管周上皮样细胞瘤是一种罕见的间叶组织肿瘤,具有独特的免疫组织化学特征。虽然已知其可发生于各种解剖部位,但中枢神经系统一直是原发性或继发性血管周上皮样细胞瘤的一个“保护区”。我们描述了一位61岁的女性,她在十二指肠和胸部肿瘤切除术后3个月出现颅内压升高症状,这些肿瘤组织学上与血管周上皮样细胞瘤一致。她接受了检查,随后切除了两个颅内转移病灶。讨论了转移病灶的放射学、术中及组织病理学表现。脑转移性血管周上皮样细胞瘤极为罕见。然而,被分层为复发或转移高风险的患者,除了进行全身正电子发射断层扫描外,还应早期进行脑部磁共振成像/计算机断层扫描,以便早期发现和处理这些肿瘤。