Abstract
Epithelioid hemangioendothelioma (EHE) is a rare vascular-derived malignancy. Within the thorax, EHE originating in the pleura is less often reported than EHE originating in the lung. Pulmonary EHE has been referred to as a borderline or low-grade malignancy that exhibits an unpredictable course. It often is associated with a long life expectancy and occasional spontaneous remission. Because the natural history of pleural EHE is less well understood, a literature review was conducted to investigate the characteristics of the disease. Twenty-two cases of pleural EHE were identified. The disease occurred more often in men than women, appeared in the right thorax more often than the left, exhibited a virulent course, was usually refractory to systemic therapy, and was often associated with a short survival. These characteristics differ substantially from those of pulmonary EHE and suggest that the clinician should approach pleural EHE anticipating an especially aggressive malignancy which likely will not respond to current therapeutic measures.
Similar content being viewed by others
References
Cronin P, Arenberg D (2004) Pulmonary epithelioid hemangioendothelioma: an unusual case and a review of the literature. Chest 125:789–793
Lisy M, Beierlein W, Muller H, Bultmann B, Ziemer G (2007) Left atrial epithelioid hemangioendothelioma. J Thorac Cardiovasc Surg 133:803–804
Attanoos RL, Suvarna SK, Rhead E, Stephens M, Locke TJ, Sheppard MN, Pooley FD, Gibbs AR (2000) Malignant vascular tumours of the pleura in “asbestos” workers and endothelial differentiation in malignant mesothelioma. Thorax 55:860–863
Stout AP (1943) Hemangio-endothelioma: a tumor of blood vessels featuring vascular endothelial cells. Ann Surg 118:445–464
Yousem SA, Hochholzer L (1987) Unusual thoracic manifestations of epithelioid hemangioendothelioma. Arch Pathol Lab Med 111:459–463
Bevelaqua FA, Valensi Q, Hulnick D (1988) Epithelioid hemangioendothelioma. A rare tumor with variable prognosis presenting as a pleural effusion. Chest 93:665–666
Lin BT, Colby T, Gown AM, Hammar SP, Mertens RB, Churg A, Battifora H (1996) Malignant vascular tumors of the serous membranes mimicking mesothelioma. A report of 14 cases. Am J Surg Pathol 20:1431–1439
Pinet C, Magnan A, Garbe L, Payan MJ, Vervloet D (1999) Aggressive form of pleural epithelioid haemangioendothelioma: complete response after chemotherapy. Eur Respir J 14:237–238
Zhang PJ, Livolsi VA, Brooks JJ (2000) Malignant epithelioid vascular tumors of the pleura: report of a series and literature review. Hum Pathol 31:29–34
Crotty EJ, McAdams HP, Erasmus JJ, Sporn TA, Roggli VL (2000) Epithelioid hemangioendothelioma of the pleura: clinical and radiologic features. Am J Roentgenol 175:1545–1549
Al-Shraim M, Mahboub B, Neligan PC, Chamberlain D, Ghazarian D (2005) Primary pleural epithelioid hemangioendothelioma with metastases to the skin. A case report and literature review. J Clin Pathol 58:107–109
Knuuttila A, Jee KJ, Taskinen E, Wolff H, Knuutila S, Anttila S (2006) Spindle cell tumors of the pleura: a clinical, histological and comparative genomic hybridization analysis of 14 cases. Virchows Arch 448:135–141
Saqi A, Nisbet L, Gagneja P, Leslie KO (2007) Primary pleural epithelioid hemangioendothelioma with rhabdoid phenotype: report and review of the literature. Diagn Cytopathol 35:203–208
Lee YJ, Chung MJ, Jeong KC, Hahn CH, Hong KP, Kim YJ, Kim YT (2008) Pleural epithelioid hemangioendothelioma. Yonsei Med J 49:1036–1040
Kim EA, Lele SM, Lackner RP (2010) Primary pleural epithelioid hemangioendothelioma. Ann Thorac Surg (in press
Weinreb I, Cunningham KS, Perez-Ordoñez B, Hwang DM (2009) CD10 is expressed in most epithelioid hemangioendotheliomas: a potential diagnostic pitfall. Arch Pathol Lab Med 133:1965–1968
Weiss SW, Enzinger FM (1982) Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer 50:970–981
Stacher E, Gruber-Mosenbacher U, Halbwedl I et al (2009) The VEGF-system in primary pulmonary angiosarcomas and haemangioendotheliomas: new potential therapeutic targets. Lung Cancer 65:49–55
Radzikowska E, Szczepulska-Wojcik E, Chabowski M et al (2008) Pulmonary epithelioid haemangioendothelioma: interferon 2-alpha treatment: case report. Pneumonol Alergol Pol 76:281–285
Calabro L, Di Giacomo AM, Altomonte M et al (2007) Primary hepatic epithelioid hemangioendothelioma progressively responsive to interferon-alpha: is there room for novel antiangiogenetic treatments. J Exp Clin Cancer Res 26:145–150
Kassam A, Mandel K (2008) Metastatic hepatic epithelioid hemangioendothelioma in a teenage girl. J Pediatr Hematol Oncol 30:550–552
Conflict of interest
No funding was received for this work beyond salary support from the University of Nebraska Medical Center.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Reade, C.A., Ganti, A.K. & Kessinger, A. Clinicopathologic characteristics of primary pleural epithelioid hemangioendothelioma. Oncol Rev 4, 219–222 (2010). https://doi.org/10.1007/s12156-010-0053-0
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12156-010-0053-0