Epithelioid Hemangioendothelioma of Liver: A Clinicopathologic Analysis of 9 Cases and Review of Literatures_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

 ZHONGLan ,  ZHANGXiu-hui , HEDu , LUZhong-cui

Department of Pathology, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China;

Corresponding author：

ZHANGXiu-hui, Email: zhangxiuhui1955@126.com

Keywords：

Epithelioid hemangioendothelioma; Liver; Histopathology; Immunohistochemistry

DOI：

10.7507/1007-9424.20150060

Video：

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Objective To summarize the clinicopathologic features of hepatic epithelioid hemangioendothelioma (EHE). Method The clinical and histopathologic features, and follow-up data of 9 patients with hepatic EHE were analyzed retrospectively. Results ①There were 4 males and 5 females in 9 patients with hepatic EHE. The age was from 25 years to 69 years.②Five patients presented with nonspecific symptoms such as right upper quadrant pain, fatigue or weight loss. Four patients were asymptomatic.③Macroscopically, the tumors were usually multiple and ranged in size from 0.5 cm to 9.0 cm.④Histologically, the tumors appeared as cords or solid nets that were characterized by epithelioid, histiocytoid, spindle or signet-ring-like cells. The cells often contained vacuoles representing intracellular lumina, in which red blood cells could be seen. The neoplastic cells invaded the surrounding liver tissue and formed papillary or glomeruloid structure in vessels. The stroma was fibrous with myxohyaline areas.⑤Immunohistochemical stain showed that all the tumors were positive for endothelial markers (factorⅧ-related antigen, CD34, CD31, and so on). Conclusions EHE of liver is a very rare clinical entity. It might be easily misdiagnosed due to EHE without specific clinical and imaging changes. Its final diagnosis depends on pathological and immunohistochemical findings.

Citation： ZHONGLan, ZHANGXiu-hui, HEDu, LUZhong-cui. Epithelioid Hemangioendothelioma of Liver: A Clinicopathologic Analysis of 9 Cases and Review of Literatures. CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY, 2015, 22(2): 224-229. doi: 10.7507/1007-9424.20150060 Copy

1.	Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer, 1982, 50(5): 970-981.
2.	Ishak KG, Sesterhenn IA, Goodman ZD, et al. Epithelioid hemangioendothelioma of the liver: a clinicopathologic and follow-up study of 32 cases. Hum Pathol, 1984, 15(9): 839-852.
3.	Makhlouf HR, Ishak KG, Goodman ZD. Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases. Cancer, 1999, 85(3): 562-582.
4.	Christopher DM, Fletcher, Julia A, et al. WHO classification of tumours of soft tissue and bone. Lyon:IARC Press, 2013: 155-156.
5.	张树辉, 丛文铭, 吴孟超.肝上皮样血管内皮瘤的临床病理特点(附8例报告及文献复习).中华肝胆外科杂志, 2003, 9(6): 327-330.
6.	Mehrabi A, Kashfi A, Fonouni H, et al. Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy. Cancer, 2006, 107(9): 2108-2121.
7.	Lauffer JM, Zimmermann A, Krahenbuhl L, et al. Epithelioid hemangioendothelioma of the liver. A rare hepatictumor. Cancer, 1996, 78(11): 2318-2327.
8.	Hayashi Y, Inagaki K, Hirota S, et al. Epithelioid hemangioendothelioma with marked liver deformity and secondary Budd-Chiari syndrome: pathological and radiological correlation. Pathol Int, 1999, 49(6): 547-552.
9.	Komatsu Y, Koizumi T, Yasuo MA, et al. Malignant hepatic epithelioid hemangioendothelioma with rapid progression and fatal outcome. Intern Med, 2010, 49(12): 1149-1153.
10.	Zis P, Assi A, Kravaritis D, et al. Ischemic stroke as the first manifestation of hepatic epithelioid hemangioendothelioma. J Stroke Cerebrovasc Dis, 2014, 23(3): e237-e240.
11.	Hsieh MS, Liang PC, Kao YC, et al. Hepatic epithelioid hemangioendothelioma in Taiwan: a clinicopathologic study of six cases in a single institution over a 15-year period. J Formos Med Assoc, 2010, 109(3): 219-227.
12.	Thin LW, Wong DD, De Boer BW et al. Hepatic epithelioid haemangioendothelioma: challenges in diagnosis and management. Intern Med J, 2010, 40(10): 710-715.
13.	赵爱莲, 周立新, 李向红.肝脏上皮样血管内皮瘤穿刺标本的病理诊断与鉴别诊断.中华病理学杂志, 2011, 40(1): 23-26.
14.	Christopher DM, Fletcher K, Krishnan U.软组织与骨肿瘤病理学和遗传学.北京:人民卫生出版社, 2006: 199-202.
15.	Liu YI, Brown SS, Elihu A, et al. Hepatic epithelioid hemangioendothelioma. Dig Dis Sci, 2011, 56(2): 303-306.
16.	Emamaullee JA, Edgar R, Toso C, et al. Vascular endothelial growth factor expression in hepatic epithelioid hemangioendothelioma: Implications for treatment and surgical management. Liver Transpl, 2010, 16(2): 191-197.
17.	Fujii T, Zen Y, Sato Y, et al. Podoplanin is a useful diagnostic marker for epithelioid hemangioendothelioma of the liver. Mod Pathol, 2008, 21(2): 125-130.
18.	Errani C, Zhang L, Sung YS, et al. A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites. Genes Chromosomes Cancer, 2011, 50(8): 644-653.
19.	Antonescu CR, Le Loarer F, Mosquera JM, et al. Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma. Genes Chromosomes Cancer, 2013, 52(8): 775-784.
20.	Lerut JP, Orlando G, Adam R, et al. The place of liver transplantation in the treatment of hepatic epitheloid hemangioendothelioma: report of the European liver transplant registry. Ann Surg, 2007, 246(6): 949-957.
21.	Mascarenhas RC, Sanghvi AN, Friedlander L, et al. Thalidomide inhibits the growth and progression of hepatic epithelioid hemangioendothelioma. Oncology, 2004, 67(5-6): 471-475.
22.	Sangro B, Iñarrairaegui M, Fernández-Ros N. Malignant epithelioid hemangioendothelioma of the liver successfully treated with Sorafenib. Rare Tumors, 2012, 4(2): e34.
23.	Hertl M, Cosimi AB. Liver transplantation for malignancy. Oncologist, 2005, 10(4): 269-281.
24.	Wang LR, Zhou JM, Zhao YM, et al. Clinical experience with primary hepatic epithelioid hemangioendothelioma: retrospective study of 33 patients. World J Surg, 2012, 36(11): 2677-2683.
25.	Choi KH, Moon WS. Epithelioid hemangioendothelioma of the liver. Clin Mol Hepatol, 2013, 19(3): 315-319.

1. Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer, 1982, 50(5): 970-981.
2. Ishak KG, Sesterhenn IA, Goodman ZD, et al. Epithelioid hemangioendothelioma of the liver: a clinicopathologic and follow-up study of 32 cases. Hum Pathol, 1984, 15(9): 839-852.
3. Makhlouf HR, Ishak KG, Goodman ZD. Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases. Cancer, 1999, 85(3): 562-582.
4. Christopher DM, Fletcher, Julia A, et al. WHO classification of tumours of soft tissue and bone. Lyon:IARC Press, 2013: 155-156.
5. 张树辉, 丛文铭, 吴孟超.肝上皮样血管内皮瘤的临床病理特点(附8例报告及文献复习).中华肝胆外科杂志, 2003, 9(6): 327-330.
6. Mehrabi A, Kashfi A, Fonouni H, et al. Primary malignant hepatic epithelioid hemangioendothelioma: a comprehensive review of the literature with emphasis on the surgical therapy. Cancer, 2006, 107(9): 2108-2121.
7. Lauffer JM, Zimmermann A, Krahenbuhl L, et al. Epithelioid hemangioendothelioma of the liver. A rare hepatictumor. Cancer, 1996, 78(11): 2318-2327.
8. Hayashi Y, Inagaki K, Hirota S, et al. Epithelioid hemangioendothelioma with marked liver deformity and secondary Budd-Chiari syndrome: pathological and radiological correlation. Pathol Int, 1999, 49(6): 547-552.
9. Komatsu Y, Koizumi T, Yasuo MA, et al. Malignant hepatic epithelioid hemangioendothelioma with rapid progression and fatal outcome. Intern Med, 2010, 49(12): 1149-1153.
10. Zis P, Assi A, Kravaritis D, et al. Ischemic stroke as the first manifestation of hepatic epithelioid hemangioendothelioma. J Stroke Cerebrovasc Dis, 2014, 23(3): e237-e240.
11. Hsieh MS, Liang PC, Kao YC, et al. Hepatic epithelioid hemangioendothelioma in Taiwan: a clinicopathologic study of six cases in a single institution over a 15-year period. J Formos Med Assoc, 2010, 109(3): 219-227.
12. Thin LW, Wong DD, De Boer BW et al. Hepatic epithelioid haemangioendothelioma: challenges in diagnosis and management. Intern Med J, 2010, 40(10): 710-715.
13. 赵爱莲, 周立新, 李向红.肝脏上皮样血管内皮瘤穿刺标本的病理诊断与鉴别诊断.中华病理学杂志, 2011, 40(1): 23-26.
14. Christopher DM, Fletcher K, Krishnan U.软组织与骨肿瘤病理学和遗传学.北京:人民卫生出版社, 2006: 199-202.
15. Liu YI, Brown SS, Elihu A, et al. Hepatic epithelioid hemangioendothelioma. Dig Dis Sci, 2011, 56(2): 303-306.
16. Emamaullee JA, Edgar R, Toso C, et al. Vascular endothelial growth factor expression in hepatic epithelioid hemangioendothelioma: Implications for treatment and surgical management. Liver Transpl, 2010, 16(2): 191-197.
17. Fujii T, Zen Y, Sato Y, et al. Podoplanin is a useful diagnostic marker for epithelioid hemangioendothelioma of the liver. Mod Pathol, 2008, 21(2): 125-130.
18. Errani C, Zhang L, Sung YS, et al. A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites. Genes Chromosomes Cancer, 2011, 50(8): 644-653.
19. Antonescu CR, Le Loarer F, Mosquera JM, et al. Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma. Genes Chromosomes Cancer, 2013, 52(8): 775-784.
20. Lerut JP, Orlando G, Adam R, et al. The place of liver transplantation in the treatment of hepatic epitheloid hemangioendothelioma: report of the European liver transplant registry. Ann Surg, 2007, 246(6): 949-957.
21. Mascarenhas RC, Sanghvi AN, Friedlander L, et al. Thalidomide inhibits the growth and progression of hepatic epithelioid hemangioendothelioma. Oncology, 2004, 67(5-6): 471-475.
22. Sangro B, Iñarrairaegui M, Fernández-Ros N. Malignant epithelioid hemangioendothelioma of the liver successfully treated with Sorafenib. Rare Tumors, 2012, 4(2): e34.
23. Hertl M, Cosimi AB. Liver transplantation for malignancy. Oncologist, 2005, 10(4): 269-281.
24. Wang LR, Zhou JM, Zhao YM, et al. Clinical experience with primary hepatic epithelioid hemangioendothelioma: retrospective study of 33 patients. World J Surg, 2012, 36(11): 2677-2683.
25. Choi KH, Moon WS. Epithelioid hemangioendothelioma of the liver. Clin Mol Hepatol, 2013, 19(3): 315-319.

CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Epithelioid Hemangioendothelioma of Liver: A Clinicopathologic Analysis of 9 Cases and Review of Literatures

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