Servicio de Cirugía General y del Aparato Digestivo, Complejo Hospitalario Universitario de Vigo, Vigo, Galicia, España.
Servicio de Cirugía General y del Aparato Digestivo, Complejo Hospitalario Universitario de Vigo, Vigo, Galicia, España.
Gastroenterol Hepatol. 2024 Jan;47(1):93-100. doi: 10.1016/j.gastrohep.2023.05.009. Epub 2023 May 24.
Pancreatic PEComas are extremely rare neoplasms with malignant potential, which mostly affect middle-aged women and are characterized by presenting melanocytic and myogenic markers in immunohistochemical analysis. There are no symptoms or pathognomonic imaging tests, so the diagnosis is established with the analysis of the surgical specimen or the FNA obtained with preoperative endoscopic ultrasound. The mean treatment consists on radical excision, adapting the intervention to the location of the tumor. To date, 34 cases have been described; however, more than 80% of them have been reported in the last decade, which suggests that it is a more frequent pathology than expected. A new case of pancreatic PEComa is reported and a systematic review of the literature is carried out according to the PRISMA guidelines with the aim of divulge this pathology, deepening its knowledge and updating its management.
胰腺上皮样血管平滑肌肿瘤是一种极其罕见的具有恶性潜能的肿瘤，主要影响中年女性，其特点是免疫组织化学分析中呈现黑色素细胞和肌源性标志物。这些肿瘤没有症状或特征性影像学检查，因此诊断是通过对手术标本或术前内镜超声获得的细针穿刺抽吸活检（FNA）进行分析来确立的。标准治疗方法是根治性切除，根据肿瘤位置调整干预措施。迄今为止，已描述了 34 例病例；然而，其中超过 80%是在过去十年中报告的，这表明它是一种比预期更常见的病理学。报告了一例新的胰腺上皮样血管平滑肌肿瘤病例，并根据 PRISMA 指南进行了文献系统性回顾，旨在传播这一病理学知识，加深对其认识并更新其管理。
