Primary hepatic perivascular epithelioid cell tumors: imaging findings with histopathological correlation
Primary hepatic perivascular epithelioid cell tumors: imaging findings with histopathological correlation
复制标题
原发性肝血管周围上皮样细胞瘤：影像学表现与组织病理学相关性
DOI：
10.1186/s40644-019-0212-x
复制
发表时间：
2019-06-06
期刊：
影响因子：
4.9
通讯作者：
Yang, Guangjie
中科院分区：
文献类型：
--
作者：
Nie, Pei;Wu, Jie;Yang, Guangjie
背景：肝PEComas非常罕见。很少有系统的报告描述肝PEComa的影像学和病理学特征。为探讨原发性肝血管周围上皮样细胞肿瘤（PEComa）的影像学表现及其与病理组织学特征的关系，回顾性分析22例原发性肝PEComa的CT、MRI、超声影像学表现及病理组织学特征。大多数患者（17/22）无症状，常规实验室检查正常。右叶肿瘤多见（13/22），平均直径76.7mm。21例患者行手术治疗，1例患者行活检。免疫组化结果显示HMB-45和Melan A在肿瘤细胞中的表达率分别为100%（22/22）和86.4%（19/22）。病理诊断为血管平滑肌脂肪瘤（n= 18）、淋巴管平滑肌瘤（n= 2）、镰状韧带/圆韧带透明细胞肌黑素细胞瘤（n = 1），另有1例未特别说明。15例病例被分类为恶性潜能不确定（n= 13）或恶性（n = 2）。CT、MRI和超声表现为边界清楚（19/22）、内部不均匀性（20/22）、动脉强化（20/22）、血管畸形（17/22）、脂肪（9/22）、出血（3/22）、坏死（8/22）和钙化（2/22）。诊断准确率仅为27.3%（6/22）。结论CT、MRI及超声图像显示，肝PEComa多为边界清晰、不均匀、动脉强化、血管畸形的肿块，伴或不伴脂肪，尤以中年女性多见。由于其具有恶性潜能，及时手术切除和长期随访可能有助于改善预后。
BackgroundHepatic PEComas are very rare. Few systematic reports are available characterizing the imaging and pathological features of hepatic PEComa. The aim of this study was to investigate the imaging findings of primary hepatic perivascular epithelioid cell tumors (PEComa) and its correlation with histopathological features.MethodsThe CT, MRI and ultrasound images and pathological findings of 22 patients with primary hepatic PEComa were retrospectively reviewed.ResultsMore females (14/22) were affected with the mean age of 47.1 years. Most patients (17/22) were asymptomatic and the routine laboratory tests were normal. More tumors occurred in the right lobe (13/22) with a mean diameter of 76.7 mm. Surgery was performed in 21 patients, and biopsy was performed in 1 patient. Immunohistochemical studies showed the expression rate of HMB-45 and Melan A was 100% (22/22) and 86.4% (19/22) within the tumor cells. The pathology diagnoses were angiomyolipoma (n= 18), lymphangioleiomyoma (n= 2), clear-cell myomelanocytic tumor of falciform ligament/ligamentum teres (n = 1), and not otherwise specified (n = 1). Fifteen cases were classified as uncertain malignant potential (n= 13) or malignant (n = 2). CT, MRI and ultrasound features included well-defined margins (19/22), internal heterogeneity (20/22), arterial enhancement (20/22), dysmorphic vessels (17/22), fat (9/22), hemorrhage (3/22), necrosis (8/22), and calcification (2/22). The diagnostic accuracy was only 27.3% (6/22). No local recurrence or metastasis was found in the follow-up patients (12/22).ConclusionsOn CT, MRI and ultrasound images, most hepatic PEComas are well-defined, heterogeneous, arterial enhanced masses with dysmorphic vessels, with or without fat, especially in middle-aged females. With the potential to be malignant, timely surgical resection and long-term follow-up may be helpful for improving the prognosis.