Hepatic epithelioid angiomyolipoma: report of 5 cases_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

 ZHU Yaning ¹ , WU Jian ¹ , ZHOU Wubi ¹ , ZHANG Yuanyuan ²

1. Department of Pathology, Huai’an First People’s Hospital, Nanjing Medical University, Huai’an, Jiangsu 223300, P. R. China;
2. Department of Pediatrics, Huai’an First People’s Hospital, Nanjing Medical University, Huai’an, Jiangsu 223300, P. R. China;

Corresponding author：

ZHU Yaning, Email: 03nan.cy@163.com

Keywords：

hepatic neoplasm; epithelioid angiomyolipoma; pathology; immunohistochemistry

DOI：

10.7507/1007-9424.201812082

Video：

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Objective To summarize clinicopathologic and immunophenotypic features of hepatic epithelioid angiomyolipoma (HEAML) and to explore its diagnostic and differential diagnostic methods.Method The clinical and imaging manifestations, pathological morphology and immunohistochemical features of 5 patients with HEAML from August 2011 to December 2017 in this hospital were retrospectively analyzed.Results There were 2 males and 3 females in the 5 patients with HEAML, aged 38–64 years with an average age of 50 years. There were 2 cases of the left lobe tumors and 3 cases of the right lobe tumors. Three cases were diagnosed as the hepatocellular carcinoma and the other two cases were diagnosed as the hepatic hamartoma and (or) hemangioma by the preoperative imaging examination. The diameter of tumors ranged from 1.5 cm to 7.0 cm, with an average of 3.6 cm. Microscopically, the tumors were composed of more epithelioid smooth muscle cells, parenchyma vessels and a small amount of fat. The immunohistochemical results showed that the melan-A, HMB45, and SMA were positive, while the HepPar-1, AE1/AE3, EMA, CD117, Dog-1, CD10, CgA, Syn, and Desmin were negative. The Ki-67 proliferation index was 2%–10%. The patients were all alive without the tumor recurrence after following up for 2–76 months with an average of 31.4 months.Conclusions HEAML is a rare primary mesenchymal tumor of liver, which should be misdiagnosed for other benign or malignant tumors for influencing clinical treatment. Diagnosis and differential diagnosis can be made by histopathology and immunohistochemical staining.

Citation： ZHU Yaning, WU Jian, ZHOU Wubi, ZHANG Yuanyuan. Hepatic epithelioid angiomyolipoma: report of 5 cases. CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY, 2019, 26(6): 702-705. doi: 10.7507/1007-9424.201812082 Copy

1.	Ishak KG. Mesenchymal tumors of the liver//Okuda K, Peters RL. Hepatocellular Carcinoma. New York: John Wiley, 1976.
2.	Chen W, Liu Y, Zhuang Y, et al. Hepatic perivascular epithelioid cell neoplasm: A clinical and pathological experience in diagnosis and treatment. Mol Clin Oncol, 2017, 6(4): 487-493.
3.	Hornick JL, Fletcher CD. PEComa: what do we know so far? Histopathology, 2006, 48(1): 75-82.
4.	Im S, Yoo C, Jung JH, et al. Primary perivascular epithelioid cell tumor in the rectum: a case report and review of the literature. Pathol Res Pract, 2013, 209(4): 244-248.
5.	Yu D, Tang S. Hepatic perivascular epithelioid cell tumor: a case report and review of the literature. Intern Med, 2013, 52(12): 1333-1336.
6.	Kamimura K, Nomoto M, Aoyagi Y. Hepatic angiomyolipoma: diagnostic findings and management. Int J Hepatol, 2012, 2012: 410781.
7.	Lu HC, Chau GY, Su CW. Clinical challenges and images in GI. Diagnosis: Hepatic angiomyolipoma mimicking hepatocellular carcinoma. Gastroenterology, 2009, 136(4): 1169, 1464.
8.	刘露, 鲁亚, 刘曦娇, 等. 肝上皮样血管内皮细胞瘤的影像学表现. 中国普外基础与临床杂志, 2018, 25(2): 229-234.
9.	Liu J, Zhang CW, Hong DF, et al. Primary hepatic epithelioid angiomyolipoma: A malignant potential tumor which should be recognized. World J Gastroenterol, 2016, 22(20): 4908-4917.
10.	Flemming P, Lehmann U, Becker T, et al. Common and epithelioid variants of hepatic angiomyolipoma exhibit clonal growth and share a distinctive immunophenotype. Hepatology, 2000, 32(2): 213-217.
11.	Yang X, Li A, Wu M. Hepatic angiomyolipoma: clinical, imaging and pathological features in 178 cases. Med Oncol, 2013, 30(1): 416.
12.	路涛, 周翔平, 姜兴莲, 等. 肝血管平滑肌脂肪瘤的影像学表现及其诊断(附3例报道). 中国普外基础与临床杂志, 2010, 17(8): 860-864.
13.	Xu AM, Zhang SH, Zheng JM, et al. Pathological and molecular analysis of sporadic hepatic angiomyolipoma. Hum Pathol, 2006, 37(6): 735-741.
14.	Argani P, Aulmann S, Illei PB, et al. A distinctive subset of PEComas harbors TFE3 gene fusions. Am J Surg Pathol, 2010, 34(10): 1395-1406.
15.	Jimbo N, Nishigami T, Noguchi M, et al. Hepatic angiomyolipomas may overexpress TFE3, but have no relevant genetic alterations. Hum Pathol, 2017, 61: 41-48.
16.	Nguyen TT, Gorman B, Shields D, et al. Malignant hepatic angiomyolipoma: report of a case and review of literature. Am J Surg Pathol, 2008, 32(5): 793-798.
17.	Klompenhouwer AJ, Verver D, Janki S, et al. Management of hepatic angiomyolipoma: A systematic review. Liver Int, 2017, 37(9): 1272-1280.
18.	Brimo F, Robinson B, Guo C, et al. Renal epithelioid angiomyolipoma with atypia: a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy. Am J Surg Pathol, 2010, 34(5): 715-722.
19.	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.
20.	Huang SC, Chuang HC, Chen TD, et al. Alterations of the mTOR pathway in hepatic angiomyolipoma with emphasis on the epithelioid variant and loss of heterogeneity of TSC1/TSC2. Histopathology, 2015, 66(5): 695-705.
21.	Agaram NP, Sung YS, Zhang L, et al. Dichotomy of genetic abnormalities in PEComas with therapeutic implications. Am J Surg Pathol, 2015, 39(6): 813-825.
22.	Italiano A, Delcambre C, Hostein I, et al. Treatment with the mTOR inhibitor temsirolimus in patients with malignant PEComa. Ann Oncol, 2010, 21(5): 1135-1137.

1. Ishak KG. Mesenchymal tumors of the liver//Okuda K, Peters RL. Hepatocellular Carcinoma. New York: John Wiley, 1976.
2. Chen W, Liu Y, Zhuang Y, et al. Hepatic perivascular epithelioid cell neoplasm: A clinical and pathological experience in diagnosis and treatment. Mol Clin Oncol, 2017, 6(4): 487-493.
3. Hornick JL, Fletcher CD. PEComa: what do we know so far? Histopathology, 2006, 48(1): 75-82.
4. Im S, Yoo C, Jung JH, et al. Primary perivascular epithelioid cell tumor in the rectum: a case report and review of the literature. Pathol Res Pract, 2013, 209(4): 244-248.
5. Yu D, Tang S. Hepatic perivascular epithelioid cell tumor: a case report and review of the literature. Intern Med, 2013, 52(12): 1333-1336.
6. Kamimura K, Nomoto M, Aoyagi Y. Hepatic angiomyolipoma: diagnostic findings and management. Int J Hepatol, 2012, 2012: 410781.
7. Lu HC, Chau GY, Su CW. Clinical challenges and images in GI. Diagnosis: Hepatic angiomyolipoma mimicking hepatocellular carcinoma. Gastroenterology, 2009, 136(4): 1169, 1464.
8. 刘露, 鲁亚, 刘曦娇, 等. 肝上皮样血管内皮细胞瘤的影像学表现. 中国普外基础与临床杂志, 2018, 25(2): 229-234.
9. Liu J, Zhang CW, Hong DF, et al. Primary hepatic epithelioid angiomyolipoma: A malignant potential tumor which should be recognized. World J Gastroenterol, 2016, 22(20): 4908-4917.
10. Flemming P, Lehmann U, Becker T, et al. Common and epithelioid variants of hepatic angiomyolipoma exhibit clonal growth and share a distinctive immunophenotype. Hepatology, 2000, 32(2): 213-217.
11. Yang X, Li A, Wu M. Hepatic angiomyolipoma: clinical, imaging and pathological features in 178 cases. Med Oncol, 2013, 30(1): 416.
12. 路涛, 周翔平, 姜兴莲, 等. 肝血管平滑肌脂肪瘤的影像学表现及其诊断(附3例报道). 中国普外基础与临床杂志, 2010, 17(8): 860-864.
13. Xu AM, Zhang SH, Zheng JM, et al. Pathological and molecular analysis of sporadic hepatic angiomyolipoma. Hum Pathol, 2006, 37(6): 735-741.
14. Argani P, Aulmann S, Illei PB, et al. A distinctive subset of PEComas harbors TFE3 gene fusions. Am J Surg Pathol, 2010, 34(10): 1395-1406.
15. Jimbo N, Nishigami T, Noguchi M, et al. Hepatic angiomyolipomas may overexpress TFE3, but have no relevant genetic alterations. Hum Pathol, 2017, 61: 41-48.
16. Nguyen TT, Gorman B, Shields D, et al. Malignant hepatic angiomyolipoma: report of a case and review of literature. Am J Surg Pathol, 2008, 32(5): 793-798.
17. Klompenhouwer AJ, Verver D, Janki S, et al. Management of hepatic angiomyolipoma: A systematic review. Liver Int, 2017, 37(9): 1272-1280.
18. Brimo F, Robinson B, Guo C, et al. Renal epithelioid angiomyolipoma with atypia: a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy. Am J Surg Pathol, 2010, 34(5): 715-722.
19. 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.
20. Huang SC, Chuang HC, Chen TD, et al. Alterations of the mTOR pathway in hepatic angiomyolipoma with emphasis on the epithelioid variant and loss of heterogeneity of TSC1/TSC2. Histopathology, 2015, 66(5): 695-705.
21. Agaram NP, Sung YS, Zhang L, et al. Dichotomy of genetic abnormalities in PEComas with therapeutic implications. Am J Surg Pathol, 2015, 39(6): 813-825.
22. Italiano A, Delcambre C, Hostein I, et al. Treatment with the mTOR inhibitor temsirolimus in patients with malignant PEComa. Ann Oncol, 2010, 21(5): 1135-1137.

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Hepatic epithelioid angiomyolipoma: report of 5 cases

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