Clinicopathological characteristics of pulmonary epithelioid hemangioendothelioma: A report of four cases and review of the literature

Shao,Jinchen; Zhang,Jie

doi:10.3892/ol.2014.2566

Clinicopathological characteristics of pulmonary epithelioid hemangioendothelioma: A report of four cases and review of the literature

Authors:
- Jinchen Shao
- Jie Zhang
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Affiliations: Department of Pathology, Shanghai Chest Hospital, Shanghai Jiao Tong University, Shanghai 200030, P.R. China
Published online on: September 26, 2014 https://doi.org/10.3892/ol.2014.2566
Pages: 2517-2522

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Abstract

This study aimed to investigate the clinicopathological characteristics, differential diagnosis and prognosis of pulmonary epithelioid hemangioendothelioma (PEH). PEH is a rare low-grade malignant vascular tumor. The cause of PEH remains unclear. Patient prognosis is unpredictable, with life expectancy ranging from 1 to 15 years due to the fact that estrogenic receptors behave inconsistently within the tumor and the occurence of the disease in male patients does not support the usual hormonal hypothesis. The clinical manifestations, imaging findings, histopathological characteristics and immunohistochemical phenotypes of four cases of epithelioid hemangioendothelioma occurring in the lung were retrospectively analyzed, and a review of the associated literature was conducted. The age of onset for the four PEH cases was 25‑54 years, and the disease manifested as multiple nodules in the lungs or pleura. All of the patients underwent lobectomy or pulmonary wedge resection. The morphology of the tumor cells was epithelioid or spindle‑shaped with abundant eosinophilic cytoplasm in which lumina or vacuoles containing erythrocytes were observed. The cells were arranged in nests and cords with degenerated interstitial mucoid. The morphology of the majority of the tumor cells was moderate, including mild atypia and little mitosis or necrosis. Immunohistochemical staining showed positive results for CD31, CD34 and F8. PEH is a rare low‑ to moderate‑level tumor occurring in the lungs with differentiation toward vascular endothelial cells. Clinically, it is difficult to distinguish from a variety of other benign and malignant lung diseases. For diagnosis, a distinction must be made from other diseases such as chronic granulomatous disease, amyloid nodules, hamartoma, primary and metastatic lung cancers, malignant mesothelioma and vascular sarcoma. In the present study, the clinicopathological features of four cases of PEH were investigated and the associated literature was reviewed. The results of this study may improve understanding with regards to the diagnosis and therapeutic options for patients with PEH.

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1	Dail DH and Liebow AA: Intravascular bronchioalveolar tumor. Am J Pathol. 78:6–7. 1975.
2	Anagnostou V, Mossa E, Mihas S, Lepouras A and Tiniakos DG: Epithelioid haemangioendothelioma of the lung presenting with pulmonary nocardiosis. In Vivo. 21:1123–1126. 2007.
3	Robinson AA, Tolentino LF, Uyanne J, Melrose R and Calhoun CC: Malignant epithelioid hemangioendothelioma of the lip: a case report and comprehensive literature review. J Oral Maxillofac Surg. 72:695–701. 2014.
4	Leleu O, Lenglet F, Clarot C, Kleinmann P and Jounieaux V: Pulmonary epithelioid haemangioendothelioma: reports of three cases and a review of the literature. Rev Mal Respir. 27:778–783. 2010.
5	Cronin P and Arenberg D: Pulmonary epithelioid hemangioendothelioma: an unusual case and a review of the literature. Chest. 125:789–793. 2004.
6	Darbari A, Singh D, Singh PK and Bharadwaj M: Pulmonary epithelioid hemangioendothelioma: A rare pulmonary tumor in differential diagnosis of bronchogenic carcinoma. Lung India. 27:37–38. 2010.
7	Amin RM, Hiroshima K, Kokubo T, et al: Risk factors and independent predictors of survival in patients with pulmonary epithelioid haemangioendothelioma. Review of the literature and a case report. Respirology. 11:818–825. 2006.
8	Weiss SW, Ishak KG, Dail DH, Sweet DE and Enzinger FM: Epithelioid hemangioendothelioma and related lesions. Sem Diag Pathol. 3:259–287. 1986.
9	Kitaichi M, Nagai S, Nishimura K, Itoh H, Asamoto H, Izumi T and Dail DH: Pulmonary epithelioid haemangioendothelioma in 21 patients, including three with partial spontaneous regression. Eur Respir J. 12:89–96. 1998.
10	Yang S, Sun R, Zhou Z, Zhou J, Liang J and Mu H: Expression of Amyloid-β Protein and Amyloid-β Precursor Protein After Primary Brain-Stem Injury in Rats. Am J Forensic Med Pathol. Jun 19–2014.(Epub ahead of print).
11	Harb H and Habil I: Frequency and profile of induced abortions: hospital based study in tertiary hospitals in Egypt. J Prev Med Hyg. 54:159–162. 2013.
12	Erasmus JJ, McAdams HP and Carraway MS: A 63-year old woman with weight loss and multiple lung nodules. Chest. 111:236–238. 1997.
13	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. Cancer. 51:452–464. 1983.
14	Liao QL, Chen XD, Wang ZC, Wang W and Lai RQ: Pulmonary epithelioid hemangioendothelioma: a clinicopathological analysis. Zhonghua Jie He He Hu Xi Za Zhi. 34:187–191. 2011.(In Chinese).
15	Xu JH and Chen LR: Pulmonary epithelioid hemangioendothelioma accompanied by bilateral multiple calcified nodules in lung. Diagn Pathol. 6:212011.
16	Rosengarten D, Kramer MR, Amir G, Fuks L and Berkman N: Pulmonary epithelioid hemangioendothelioma. Isr Med Assoc J. 13:676–679. 2011.
17	Bahrami A, Allen TC and Cagle PT: Pulmonary epithelioid hemangioendothelioma mimicking mesothelioma. Pathol Int. 58:730–734. 2008.
18	Sardaro A, Bardoscia L, Petruzzelli MF, Nikolaou A, Detti B and Angelelli G: Pulmonary epithelioid hemangioendothelioma presenting with vertebral metastases: a case report. J Med Case Rep. 8:2012014.
19	Al-Shraim M, Mahboub B, Neligan PC, Chamberlain D and Ghazarian D: Primary pleural epithelioid haemangioendothelioma with metastases to the skin. A case report and literature review. J Clin Pathol. 58:107–109. 2005.
20	Weissferdt A and Moran CA: Primary vascular tumors of the lungs: a review. Ann Diagn Pathol. 14:296–308. 2010.
21	Díaz R, Segura A, Calderero V, Cervera I, Aparicio J, Jordá MV and Pellín L: Central nervous system metastases of a pulmonary epitheloid haemangioendothelioma. Eur Respir J. 23:483–486. 2004.
22	Białas M, Papla B and Bulanda A: Immunohistochemical investigation of selected endothelial markers in pulmonary epithelioid haemangioendothelioma. Pol J Pathol. 62:236–240. 2011.
23	Traweek ST, Kandalaft PL, Mehta P and Battifora H: The human hematopoietic progenitor cell antigen (CD34) in vascular neoplasia. Am J Clin Pathol. 96:25–31. 1991.
24	Molina Palma MI, Cervantes Góngora JA, García de la Torre E, Conde Pérez de la Blanca I and Ramírez Tortosa CL: Primary intraoral epithelioid hemangioendothelioma. Case report and review of the literature. Acta Otorrinolaringol Esp. 53:215–218. 2002.(In Spanish).
25	Otani K, Ishikawa T, Aizawa Y, Fujise K, Koyama T, Ohkusa T and Tajiri H: A long-term survival case of liver epithelioid hemangioendothelioma with multiple lung metastases that regressed by long-term administration of interleukin-2. Nihon Shokakibyo Gakkai Zasshi. 109:2097–2102. 2012.
26	Jinghong X and Lirong C: Pulmonary epithelioid hemangioendothelioma accompanied by bilateral multiple calcified nodules in lung. Diagn Pathol. 6:212011.(In Japanese).
27	Reich S, Ringe H, Uhlenberg B, Fischer B and Varnholt V: Epithelioid hemangioendothelioma of the lung presenting with pneumonia and heart rhythm disturbances in a teenage girl. J Pediatr Hematol Oncol. 32:274–276. 2010.
28	Pálföldi R, Radács M, Csada E, Molnár Z, Pintér S, Tiszlavicz L, Molnár J, Valkusz Z, Somfay A and Gálfi M: Pulmonary epithelioid haemangioendothelioma studies in vitro and in vivo: new diagnostic and treatment methods. In Vivo. 27:221–225. 2013.
29	Bagan P, Hassan M, Le Pimpec Barthes F, et al: Prognostic factors and surgical indications of pulmonary epithelioid. hemangioendothelioma: a review of the literature. Ann Thorac Surg. 82:2010–2013. 2006.