Research status of perivascular epithelioid cell tumors of the liver_West China Medical Journal

Authors：

FAN Wanli , ZHANG Shuze , CHEN Gang , HE Dong , ZHANG Zuyu ,  CHENG Zhibin

Department of General Surgery, Lanzhou University Second Hospital, Lanzhou, Gansu 730030, P. R. China;

Corresponding author：

CHENG Zhibin, Email: zhibin_cheng@hotmail.com

Keywords：

Perivascular epithelioid cell tumor; clinicopathological features; diagnosis; treatment

DOI：

10.7507/1002-0179.202210094

Video：

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Abstract Full text Figures/Tables Video References Cited by

Perivascular epithelioid cell tumor (PEComa) is a multi-potential tumor based on mesenchymal cells distributed around capillaries. The main affected population is female, and the clinical manifestations are not specific. It can affect all parts of the body. There are more PEComa in the uterus and very few PEComa in the liver. Due to its low incidence, clinicians lack awareness of it. Based on the relevant literature, this article reviews the clinicopathological features, imaging features, molecular phenotypes, diagnosis, differential diagnosis, and treatment of liver PEComa, so as to strengthen the understanding of the disease, prevent missed diagnosis and misdiagnosis, and guide clinical work.

Citation： FAN Wanli, ZHANG Shuze, CHEN Gang, HE Dong, ZHANG Zuyu, CHENG Zhibin. Research status of perivascular epithelioid cell tumors of the liver. West China Medical Journal, 2023, 38(3): 476-480. doi: 10.7507/1002-0179.202210094 Copy

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6.	Yamasaki S, Tanaka S, Fujii H, et al. Monotypic epithelioid angiomyolipoma of the liver. Histopathology, 2000, 36(5): 451-456.
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21.	Folpe AL, Mentzel T, Lehr HA, et al. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol, 2005, 29(12): 1558-1575.
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1. Martignoni G, Pea M, Reghellin D, et al. PEComas: the past, the present and the future. Virchows Arch, 2008, 452(2): 119-132.
2. 贺亚琼, 姚景江, 刘建滨, 等. 肝脏血管周上皮样细胞肿瘤的病理与 MSCT 表现. 临床放射学杂志, 2019, 38(1): 105-109.
3. Stone CH, Lee MW, Amin MB, et al. Renal angiomyolipoma: further immunophenotypic characterization of an expanding morphologic spectrum. Arch Pathol Lab Med, 2001, 125(6): 751-758.
4. 方三高, 魏建国, 陈真伟. WHO(2020)软组织肿瘤分类. 临床与实验病理学杂志, 2020, 36(9): 1132-1134.
5. 宣兰兰, 魏建国, 刘红刚. 血管周上皮样细胞肿瘤的病理诊断及新进展. 中华病理学杂志, 2021, 50(3): 282-287.
6. Yamasaki S, Tanaka S, Fujii H, et al. Monotypic epithelioid angiomyolipoma of the liver. Histopathology, 2000, 36(5): 451-456.
7. 刘霞, 卿松, 曹峻, 等. 原发肝脏血管周上皮细胞肿瘤的病理学观察. 中华病理学杂志, 2014, 43(8): 558-559.
8. 陈平平, 张震生, 郑进方, 等. 肝脏血管周上皮样细胞瘤误诊 1 例报告并文献复习. 中国现代手术学杂志, 2020, 24(2): 90-93.
9. Nie P, Wu J, Wang H, et al. Primary hepatic perivascular epithelioid cell tumors: imaging findings with histopathological correlation. Cancer Imaging, 2019, 19(1): 32.
10. Pan CC, Chung MY, Ng KF, et al. Constant allelic alteration on chromosome 16p (TSC2 gene) in perivascular epithelioid cell tumour (PEComa): genetic evidence for the relationship of PEComa with angiomyolipoma. J Pathol, 2008, 214(3): 387-393.
11. Fricke BL, Donnelly LF, Casper KA, et al. Frequency and imaging appearance of hepatic angiomyolipomas in pediatric and adult patients with tuberous sclerosis. AJR Am J Roentgenol, 2004, 182(4): 1027-1030.
12. Zeng JP, Dong JH, Zhang WZ, et al. Hepatic angiomyolipoma: a clinical experience in diagnosis and treatment. Dig Dis Sci, 2010, 55(11): 3235-3240.
13. 陈孟达, 汪玲, 李强, 等. 肝脏上皮样血管周细胞瘤的 CT 和 MRI 特征分析. 医学影像学杂志, 2018, 28(6): 967-970.
14. 骆必伟, 张育森, 杜志勇, 等. 17 例肝脏血管周上皮样细胞肿瘤临床分析. 中华肝胆外科杂志, 2021, 27(11): 819-822.
15. 王国禹, 赵闯, 侯登峰, 等. 肝脏血管周上皮样细胞瘤 11 例临床诊治分析. 中华医学杂志, 2018, 98(34): 2715-2717.
16. 马英腾, 沈丹华. 肝脏血管周上皮样细胞肿瘤临床与病理学特征分析. 中华普通外科杂志, 2017, 32(12): 1007-1009.
17. 黄晓华, 张智弘, 范钦和, 等. 肝脏伴上皮样分化的血管周细胞肿瘤临床病理分析. 中华病理学杂志, 2015(8): 605-607.
18. 吴云飞, 刘健红, 葛振华, 等. 肝脏血管周上皮样细胞肿瘤(Pecoma)临床病理分析并文献复习. 数理医药学杂志, 2015(3): 360-361.
19. Wang XT, Xia QY, Ni H, et al. SFPQ/PSF-TFE3 renal cell carcinoma: a clinicopathologic study emphasizing extended morphology and reviewing the differences between SFPQ-TFE3 RCC and the corresponding mesenchymal neoplasm despite an identical gene fusion. Hum Pathol, 2017, 63: 190-200.
20. Gulavita P, Fletcher CDM, Hirsch MS. PNL2: an adjunctive biomarker for renal angiomyolipomas and perivascular epithelioid cell tumours. Histopathology, 2018, 72(3): 441-448.
21. Folpe AL, Mentzel T, Lehr HA, et al. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol, 2005, 29(12): 1558-1575.
22. Yang X, Wang Q, Zhou X, et al. Retrospective analysis of hepatic perivascular epithelioid cell tumour (PEComa) in a single centre for clinical diagnosis and treatment clinical diagnosis and treatment of hepatic PEComa. Medicine (Baltimore), 2022, 101(25): e29506.
23. 张淑红, 黄受方, 陆鸣, 等. 血管周上皮样细胞肿瘤的临床病理学观察. 临床和实验医学杂志, 2011, 10(15): 1149-1152.
24. 张彩, 潘飞豹, 赵纯全. 血管周上皮样细胞肿瘤的临床病理研究进展. 重庆医学, 2014(16): 2081-2083.
25. Purwar R, Soni K, Shukla M, et al. TFE3-associated perivascular epithelioid cell tumor with complete response to mTOR inhibitor therapy: report of first case and literature review. World J Surg Oncol, 2022, 20(1): 62.
26. Yang X, Li A, Wu M. Hepatic angiomyolipoma: clinical, imaging and pathological features in 178 cases. Med Oncol, 2013, 30(1): 416.
27. Yang CY, Ho MC, Jeng YM, et al. Management of hepatic angiomyolipoma. J Gastrointest Surg, 2007, 11(4): 452-457.
28. Nonomura A, Mizukami Y, Kadoya M. Angiomyolipoma of the liver: a collective review. J Gastroenterol, 1994, 29(1): 95-105.
29. Mai KT, Belanger EC. Perivascular epithelioid cell tumour (PEComa) of the soft tissue. Pathology, 2006, 38(5): 415-420.
30. Song Z, Xu F, Dai C. Chills and fever as the first presentation of hepatic perivascular epithelioid cell tumor. Hepatobiliary Surg Nutr, 2019, 8(4): 436-438.
31. 陈翼, 董丽华, 李江城, 等. 肝血管周上皮细胞瘤的多种影像表现. 医学影像学杂志, 2017, 27(7): 1405-1409.
32. Kwon BS, Suh DS, Lee NK, et al. Two cases of perivascular epithelioid cell tumor of the uterus: clinical, radiological and pathological diagnostic challenge. Eur J Med Res, 2017, 22(1): 7.
33. Tan Y, Xiao EH. Hepatic perivascular epithelioid cell tumor (PEComa): dynamic CT, MRI, ultrasonography, and pathologic features-analysis of 7 cases and review of the literature. Abdom Imaging, 2012, 37(5): 781-787.
34. Wildgruber M, Becker K, Feith M, et al. Perivascular epitheloid cell tumor (PEComa) mimicking retroperitoneal liposarcoma. World J Surg Oncol, 2014, 12: 3.
35. Cong X, Zhang J, Xu X, et al. Renal epithelioid angiomyolipoma: magnetic resonance imaging characteristics. Abdom Radiol (NY), 2018, 43(10): 2756-2763.
36. Wang SY, Kuai XP, Meng XX, et al. Comparison of MRI features for the differentiation of hepatic angiomyolipoma from fat-containing hepatocellular carcinoma. Abdom Imaging, 2014, 39(2): 323-333.
37. 何璐, 覃玲艳, 周梦琦, 等. 肝脏血管周上皮样细胞肿瘤的多模态影像学表现与病理对照分析. 临床放射学杂志, 2021, 40(5): 913-917.
38. 韩伟, 候雪琴, 宋涛, 等. 肝脏血管周上皮细胞肿瘤与肝细胞肝癌及肝血管瘤的超声特征分析. 中国临床医学影像杂志, 2021, 32(5): 342-346,355.
39. Phillips CH, Keraliya AR, Shinagare AB, et al. Update on the imaging of malignant perivascular epithelioid cell tumors (PEComas). Abdom Radiol (NY), 2016, 41(2): 368-376.
40. 杜锐, 赵年, 陈晓荣, 等. 肝脏恶性血管周上皮样细胞肿瘤并术后复发转移 1 例. 湖北医药学院学报, 2021, 40(3): 300-302, 封 3.
41. Yang X, Lei C, Qiu Y, et al. Selecting a suitable surgical treatment for hepatic angiomyolipoma: a retrospective analysis of 92 cases. ANZ J Surg, 2018, 88(9): E664-E669.
42. 刘河, 刘浩, 石荣, 等. 肝脏血管周上皮样细胞瘤的诊治分析. 中国现代医学杂志, 2021, 31(7): 22-27.
43. Akitake R, Kimura H, Sekoguchi S, et al. Perivascular epithelioid cell tumor (PEComa) of the liver diagnosed by contrast-enhanced ultrasonography. Intern Med, 2009, 48(24): 2083-2086.
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