30例肝脏血管周上皮样细胞瘤患者的临床表现、组织病理学特征

肝脏 ›› 2024, Vol. 29 ›› Issue (12): 1508-1511.

30例肝脏血管周上皮样细胞瘤患者的临床表现、组织病理学特征

温路生, 耿月华, 刘金发, 周小芹

363000 福建联勤保障部队第九〇九医院(厦门大学附属东南医院)病理科(温路生,耿月华,刘金发),输血医学科(周小芹)

收稿日期:2023-09-29 出版日期:2024-12-31 发布日期:2025-02-19
通讯作者: 周小芹,Email:353291715@qq.com
基金资助:
第九〇九医院青年苗圃基金(18Y017)

Clinical manifestations and histopathological features of 30 patients with perivascular epithelioid cell tumors of the liver

WEN Lu-sheng¹, GENG Yue-hua¹, LIU Jin-fa¹, ZHOU Xiao-qin²

1. Department of Pathology, the 909 Hospital of the Joint Support Force (Southeast Hospital Affiliated to Xiamen University), Zhangzhou 363000, China;
2. Department of Blood Transfusion Medicine (Fujian), the 909 Hospital of the Joint Support Force (Southeast Hospital Affiliated to Xiamen University), Zhangzhou 363000, China

Received:2023-09-29 Online:2024-12-31 Published:2025-02-19
Contact: ZHOU Xiao-qin,Email:353291715@qq.com

摘要/Abstract

摘要： 目的分析30例肝脏血管周上皮样细胞瘤患者的临床表现、组织病理学特征。方法 2019年2月—2020年2月联勤保障部队第九〇九医院收治的手术切除并病理确诊的30例肝脏血管周围上皮样细胞瘤患者,收集患者临床资料,分析临床表现、组织病理学特征。结果 30例病例中,男性10例,女性20例,病发年龄以25~54岁居多,多数有上腹部疼痛症状,肿瘤多为单发,病灶多位于肝左外叶,肿瘤大小在60~100 mm之间,肝功能检查血清丙氨酸氨基转移酶、碱性磷酸酶、胆红素水平升高,免疫组织化学以抗黑素瘤特异性单抗、黑色素细胞抗原和抗平滑肌抗体阳性为主。30例病例中,27例在术后2年内均可接受随访,3例只接受1年随访,随访率为90.0%。27例随访期间未发现肿瘤转移、复发、术后未行其他治疗,随访至2022年2月患者情况良好,肝脏CT、血常规、肝功能检查多数无异常。结论肝脏血管周围上皮细胞瘤是一种非常罕见的间质性肿瘤,易与其他肝脏肿瘤混淆,结合临床与病理特征,应用CT成像、腹部超声、MRI影像学检查,可以为肝脏血管周围上皮细胞肿瘤的诊断和鉴别诊断提供可靠依据。

关键词: 肝脏血管周上皮样细胞瘤, 临床表现, 组织病理学

Abstract: Objective To analyze the clinical manifestations and histopathological features of 30 patients with perivascular epithelioid cell tumors of the liver.Methods Thirty patients with surgically resected and pathologically confirmed hepatic perivascular epithelioid cell tumors admitted to the 909th Hospital of the Joint Support Force between February 2019 and February 2020 were collected for clinical data and analyzed with clinical manifestations and histopathological features.Results Among the 30 cases, there were 10 males and 20 females. The age of onset was mostly 25~54 years old, and most of them had epigastric pain symptoms. The tumors were mostly solitary, the lesions were mostly located in the left outer lobe of the liver, and the tumors were between 60~100 mm. The liver function tests showed elevated levels of serum glutamate transaminase, alkaline phosphatase and bilirubin, and immunohistochemistry was mainly positive for anti-melanoma-specific monoclonal antibody, melanocyte antigen and anti-smooth muscle antibody. Among the 30 cases, 27 cases were available for follow-up within 2 years after surgery, and 3 cases received only 1 year of follow-up, with a follow-up rate of 90.0%. 27 cases were not found to have tumor metastasis or recurrence during the follow-up period, and no other treatment was performed after surgery, and the patients were in good condition until February 2022, and most of the liver CT, blood routine, and liver function tests were not abnormal.Conclusion Perivascular epithelial cell tumor of the liver is a very rare interstitial tumor that is easily confused with other liver tumors. Combining clinical and pathological features, the application of CT imaging, abdominal ultrasound, and MRI imaging can provide reliable basis for the diagnosis and differential diagnosis of perivascular epithelial cell tumors of the liver.

Key words: Perivascular epithelioid cell tumor of the liver, Clinical manifestations, Histopathology

温路生, 耿月华, 刘金发, 周小芹. 30例肝脏血管周上皮样细胞瘤患者的临床表现、组织病理学特征[J]. 肝脏, 2024, 29(12): 1508-1511.

WEN Lu-sheng, GENG Yue-hua, LIU Jin-fa, ZHOU Xiao-qin. Clinical manifestations and histopathological features of 30 patients with perivascular epithelioid cell tumors of the liver[J]. Chinese Hepatolgy, 2024, 29(12): 1508-1511.

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