Oncology Reviews

, Volume 4, Issue 4, pp 219–222 | Cite as

Clinicopathologic characteristics of primary pleural epithelioid hemangioendothelioma

  • Chad A. Reade
  • Apar Kishor Ganti
  • Anne Kessinger
Review

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.

Keywords

Pleural epithelioid hemangioendothelioma Survival Clinical features Management 

References

  1. 1.
    Cronin P, Arenberg D (2004) Pulmonary epithelioid hemangioendothelioma: an unusual case and a review of the literature. Chest 125:789–793CrossRefPubMedGoogle Scholar
  2. 2.
    Lisy M, Beierlein W, Muller H, Bultmann B, Ziemer G (2007) Left atrial epithelioid hemangioendothelioma. J Thorac Cardiovasc Surg 133:803–804CrossRefPubMedGoogle Scholar
  3. 3.
    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 55:860–863CrossRefPubMedGoogle Scholar
  4. 4.
    Stout AP (1943) Hemangio-endothelioma: a tumor of blood vessels featuring vascular endothelial cells. Ann Surg 118:445–464CrossRefPubMedGoogle Scholar
  5. 5.
    Yousem SA, Hochholzer L (1987) Unusual thoracic manifestations of epithelioid hemangioendothelioma. Arch Pathol Lab Med 111:459–463PubMedGoogle Scholar
  6. 6.
    Bevelaqua FA, Valensi Q, Hulnick D (1988) Epithelioid hemangioendothelioma. A rare tumor with variable prognosis presenting as a pleural effusion. Chest 93:665–666CrossRefPubMedGoogle Scholar
  7. 7.
    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 20:1431–1439CrossRefPubMedGoogle Scholar
  8. 8.
    Pinet C, Magnan A, Garbe L, Payan MJ, Vervloet D (1999) Aggressive form of pleural epithelioid haemangioendothelioma: complete response after chemotherapy. Eur Respir J 14:237–238CrossRefPubMedGoogle Scholar
  9. 9.
    Zhang PJ, Livolsi VA, Brooks JJ (2000) Malignant epithelioid vascular tumors of the pleura: report of a series and literature review. Hum Pathol 31:29–34CrossRefPubMedGoogle Scholar
  10. 10.
    Crotty EJ, McAdams HP, Erasmus JJ, Sporn TA, Roggli VL (2000) Epithelioid hemangioendothelioma of the pleura: clinical and radiologic features. Am J Roentgenol 175:1545–1549Google Scholar
  11. 11.
    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 58:107–109CrossRefPubMedGoogle Scholar
  12. 12.
    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 448:135–141CrossRefPubMedGoogle Scholar
  13. 13.
    Saqi A, Nisbet L, Gagneja P, Leslie KO (2007) Primary pleural epithelioid hemangioendothelioma with rhabdoid phenotype: report and review of the literature. Diagn Cytopathol 35:203–208CrossRefPubMedGoogle Scholar
  14. 14.
    Lee YJ, Chung MJ, Jeong KC, Hahn CH, Hong KP, Kim YJ, Kim YT (2008) Pleural epithelioid hemangioendothelioma. Yonsei Med J 49:1036–1040CrossRefPubMedGoogle Scholar
  15. 15.
    Kim EA, Lele SM, Lackner RP (2010) Primary pleural epithelioid hemangioendothelioma. Ann Thorac Surg (in pressGoogle Scholar
  16. 16.
    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 133:1965–1968PubMedGoogle Scholar
  17. 17.
    Weiss SW, Enzinger FM (1982) Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma. Cancer 50:970–981CrossRefPubMedGoogle Scholar
  18. 18.
    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 65:49–55CrossRefPubMedGoogle Scholar
  19. 19.
    Radzikowska E, Szczepulska-Wojcik E, Chabowski M et al (2008) Pulmonary epithelioid haemangioendothelioma: interferon 2-alpha treatment: case report. Pneumonol Alergol Pol 76:281–285PubMedGoogle Scholar
  20. 20.
    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 26:145–150PubMedGoogle Scholar
  21. 21.
    Kassam A, Mandel K (2008) Metastatic hepatic epithelioid hemangioendothelioma in a teenage girl. J Pediatr Hematol Oncol 30:550–552CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Chad A. Reade
    • 1
  • Apar Kishor Ganti
    • 1
    • 2
  • Anne Kessinger
    • 1
  1. 1.Department of Internal MedicineUniversity of Nebraska Medical CenterOmahaUSA
  2. 2.Department of Internal MedicineVA-NWIHCSOmahaUSA

Personalised recommendations