Pulmonary Epithelioid Hemangioendothelioma: A Case Report and the Literatures Review_West China Medical Journal

Authors：

 HUANGYan-hua ,  YANGXiao-dong , ZHANGJuan , HUQiu-fang , LIUZheng-man

Department of Respiratory Medicine, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding?author：

YANGXiao-dong, Email: yang_xiaodong@163.com

Keywords：

Lung neoplasm; Epithelioid hemangioendothelioma

DOI：

10.7507/1002-0179.20140328

Video：

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Objective To enhance the understanding of pulmonary epithelioid hemangioendothelioma (PEH), and improve the diagnostic rate of the disease. Methods The experience of diagnosis and treatment of a case of PEH in August 2013 was reported in the present study and the related literature was reviewed. The etiology, clinical manifestations, pathological diagnosis, differential diagnosis, therapy and prognosis of this disease were described. Results The etiology of PEH remained unknown. Symptoms of PEH were usually nonspecific and mild. Chest radiograph or computed tomography usually revealed calcification in multiple nodules of half or both lungs. Primitive lumen formed by a single cell was the pathologic feature. Immunohistochemical stains showed that the malignant cells were of endothelial type. Genetic analysis was relatively limited currently. There was no effective treatment for this disease and the prognosis was poor. Conclusion PEH is a low potential malignancy occurring exclusively in young women. Symptoms of PEH are usually nonspecific and variable which can easily lead to misdiagnosis. So, pathological diagnosis should be emphasized.

Citation： HUANGYan-hua, YANGXiao-dong, ZHANGJuan, HUQiu-fang, LIUZheng-man. Pulmonary Epithelioid Hemangioendothelioma: A Case Report and the Literatures Review. West China Medical Journal, 2014, 29(6): 1074-1077. doi: 10.7507/1002-0179.20140328 Copy

1.	Weiss SW, Shak KG, Dail DH, et al. Epithelioid hemangioendothelioma and related lesions[J]. Semin Diagn Pathol, 1986, 3(4): 259-287.
2.	張秀茹, 盧一艷, 郝彥勇, 等. 脾上皮樣血管內皮細胞瘤3例臨床病理分析[J]. 診斷病理學雜志, 2012, 19(2): 115-117.
3.	郭潔, 楊軍, 王曉冰, 等. 心包內上皮樣血管內皮細胞瘤超聲心動圖表現1例[J]. 中國醫學影像技術, 2012, 28(2): 351.
4.	丁勇, 錢忠心, 葉樹銘, 等. 顱內上皮樣血管內皮細胞瘤1例報告[J]. 中華神經外科疾病研究雜志, 2012, 11(1): 78-79.
5.	陳易華, 簡燚, 蔣銳, 等. 骨上皮樣血管內皮細胞瘤3例臨床病理分析[J]. 診斷病理學雜志, 2010, 17(2): 104-106.
6.	貢其星, 范欽和, 謝軍, 等. 上皮樣血管瘤七例臨床病理觀察[J]. 中華病理學雜志, 2013, 42(9): 593-598.
7.	Dail DH, Liebow AA, Gmelich JT, et al. Intravascular, bronchiolar, and alveolar tumor of the lung (IVBAT). An analysis of twenty cases of a peculiar sclerosing endothelial tumor[J]. Cancer, 1983, 51(3): 452-464.
8.	Weiss SW, Ehzinger FM. Epithelioid hemangioendothelioma: a distinctive vascular tumor often mistaken for a carcinoma[J]. Cancer, 1982, 50(5): 970-981.
9.	Ohori NP, Yousem SA, Sonmez-Alpan E, et al. Estrogen and progesterone receptors in lymphangioleiomyomatosis, epithelioid hemangioendothelioma, and sclerosing hemangioma of the lung[J]. Am J Clin Pathol, 1991, 96(4): 529-535.
10.	Kitaichi M, Nagai S, Nishimura K, et al. Pulmonary epithelioid haemangioendothelioma in 21 patients, including three with partial spontaneous regression[J]. Eur Respir J, 1998, 12(1): 89-96.
11.	尉連成, 牛子民, 楊小潔. 肺內巨大上皮樣血管內皮瘤合并非胰島細胞性低血糖1例[J]. 中國臨床醫生, 2005, 33(3): 40.
12.	Vandenheede M, Salem T, Sayago I, et al. Clinical case of the month. Multiple pulmonary nodules. Epithelioid hemangioendothelioma[J]. Rev Med Liege, 1998, 53(8): 454-458.
13.	王兆平, 高志斌, 柳君, 等. 纖維支氣管鏡標本診斷肺上皮樣血管內皮瘤2例[J]. 實用醫學雜志, 2009, 25(13): 2205-2206.
14.	管星慶, 宿杰·阿克蘇, 許建芳, 等. 肺上皮樣血管內皮瘤活體組織病理學檢查的診斷意義[J]. 中國臨床醫學, 2013, 20(3): 309-311.
15.	Ryu HS, Lee SS, Choi HS, et al. A case of pulmonary malignant epithelioid hemangioendothelioma misdiagnosed as adenocarcinoma by fine needle aspiration cytology[J]. Diagn Cytopathol, 2011, 39(11): 801-807.
16.	劉梅, 巴恩平, 趙坡, 等. 上皮樣血管肉瘤的臨床病理分析[J]. 中華病理學雜志, 2002, 31(5): 407-410.
17.	Kumazawa Y, Maeda K, Ito M, et al. Expression of glucocorticoid receptor and 11beta hydroxysteroid dehydrogenase in a case of pulmonary epithelioid haemangioendothelioma[J]. Mol Pathol, 2002, 55(1): 61-64.
18.	Bharadwaj R, Hejmadi R, Mistry R, et al. Pericardial effusion and raised CA-125 level--unusual manifestations of primary pulmonary epithelioid hemangioendothelioma[J]. Indian J Cancer, 2000, 36(2/4): 194-197.
19.	Yi ES, Auger WR, Friedman PJ, et al. Intravascular bronchioloalveolar tumor of the lung presenting as pulmonary thromboembolic disease and pulmonary hypertension[J]. Arch Pathol Lab Med, 1995, 119(3): 255-260.
20.	Boudousquie AC, Lawce HJ, Sherman R, et al. Complex translocation[7;22] identified in an epithelioid hemangioendothelioma[J]. Cancer Genet Cytogenet, 1996, 92(2): 116-121.
21.	Mendlick MR, Nelson M, Pickering D, et al. Translocation t(1;3)(p36.3;q25) is a nonrandom aberration in epithelioid hemangioendothelioma[J]. Am J Surg Pathol, 2001, 25(5): 684-687.
22.	曹焱, 鄒霜梅, 馮林, 等. 肺上皮樣血管內皮瘤遺傳學分析[J]. 癌變·畸變·突變, 2009, 21(3): 185-188.

1. Weiss SW, Shak KG, Dail DH, et al. Epithelioid hemangioendothelioma and related lesions[J]. Semin Diagn Pathol, 1986, 3(4): 259-287.
2. 張秀茹, 盧一艷, 郝彥勇, 等. 脾上皮樣血管內皮細胞瘤3例臨床病理分析[J]. 診斷病理學雜志, 2012, 19(2): 115-117.
3. 郭潔, 楊軍, 王曉冰, 等. 心包內上皮樣血管內皮細胞瘤超聲心動圖表現1例[J]. 中國醫學影像技術, 2012, 28(2): 351.
4. 丁勇, 錢忠心, 葉樹銘, 等. 顱內上皮樣血管內皮細胞瘤1例報告[J]. 中華神經外科疾病研究雜志, 2012, 11(1): 78-79.
5. 陳易華, 簡燚, 蔣銳, 等. 骨上皮樣血管內皮細胞瘤3例臨床病理分析[J]. 診斷病理學雜志, 2010, 17(2): 104-106.
6. 貢其星, 范欽和, 謝軍, 等. 上皮樣血管瘤七例臨床病理觀察[J]. 中華病理學雜志, 2013, 42(9): 593-598.
7. Dail DH, Liebow AA, Gmelich JT, et al. Intravascular, bronchiolar, and alveolar tumor of the lung (IVBAT). An analysis of twenty cases of a peculiar sclerosing endothelial tumor[J]. Cancer, 1983, 51(3): 452-464.
8. Weiss SW, Ehzinger FM. Epithelioid hemangioendothelioma: a distinctive vascular tumor often mistaken for a carcinoma[J]. Cancer, 1982, 50(5): 970-981.
9. Ohori NP, Yousem SA, Sonmez-Alpan E, et al. Estrogen and progesterone receptors in lymphangioleiomyomatosis, epithelioid hemangioendothelioma, and sclerosing hemangioma of the lung[J]. Am J Clin Pathol, 1991, 96(4): 529-535.
10. Kitaichi M, Nagai S, Nishimura K, et al. Pulmonary epithelioid haemangioendothelioma in 21 patients, including three with partial spontaneous regression[J]. Eur Respir J, 1998, 12(1): 89-96.
11. 尉連成, 牛子民, 楊小潔. 肺內巨大上皮樣血管內皮瘤合并非胰島細胞性低血糖1例[J]. 中國臨床醫生, 2005, 33(3): 40.
12. Vandenheede M, Salem T, Sayago I, et al. Clinical case of the month. Multiple pulmonary nodules. Epithelioid hemangioendothelioma[J]. Rev Med Liege, 1998, 53(8): 454-458.
13. 王兆平, 高志斌, 柳君, 等. 纖維支氣管鏡標本診斷肺上皮樣血管內皮瘤2例[J]. 實用醫學雜志, 2009, 25(13): 2205-2206.
14. 管星慶, 宿杰·阿克蘇, 許建芳, 等. 肺上皮樣血管內皮瘤活體組織病理學檢查的診斷意義[J]. 中國臨床醫學, 2013, 20(3): 309-311.
15. Ryu HS, Lee SS, Choi HS, et al. A case of pulmonary malignant epithelioid hemangioendothelioma misdiagnosed as adenocarcinoma by fine needle aspiration cytology[J]. Diagn Cytopathol, 2011, 39(11): 801-807.
16. 劉梅, 巴恩平, 趙坡, 等. 上皮樣血管肉瘤的臨床病理分析[J]. 中華病理學雜志, 2002, 31(5): 407-410.
17. Kumazawa Y, Maeda K, Ito M, et al. Expression of glucocorticoid receptor and 11beta hydroxysteroid dehydrogenase in a case of pulmonary epithelioid haemangioendothelioma[J]. Mol Pathol, 2002, 55(1): 61-64.
18. Bharadwaj R, Hejmadi R, Mistry R, et al. Pericardial effusion and raised CA-125 level--unusual manifestations of primary pulmonary epithelioid hemangioendothelioma[J]. Indian J Cancer, 2000, 36(2/4): 194-197.
19. Yi ES, Auger WR, Friedman PJ, et al. Intravascular bronchioloalveolar tumor of the lung presenting as pulmonary thromboembolic disease and pulmonary hypertension[J]. Arch Pathol Lab Med, 1995, 119(3): 255-260.
20. Boudousquie AC, Lawce HJ, Sherman R, et al. Complex translocation[7;22] identified in an epithelioid hemangioendothelioma[J]. Cancer Genet Cytogenet, 1996, 92(2): 116-121.
21. Mendlick MR, Nelson M, Pickering D, et al. Translocation t(1;3)(p36.3;q25) is a nonrandom aberration in epithelioid hemangioendothelioma[J]. Am J Surg Pathol, 2001, 25(5): 684-687.
22. 曹焱, 鄒霜梅, 馮林, 等. 肺上皮樣血管內皮瘤遺傳學分析[J]. 癌變·畸變·突變, 2009, 21(3): 185-188.

West China Medical Journal

Pulmonary Epithelioid Hemangioendothelioma: A Case Report and the Literatures Review

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