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Review
. 2005 Jan;58(1):107-9.
doi: 10.1136/jcp.2004.018937.

Primary pleural epithelioid haemangioendothelioma with metastases to the skin. A case report and literature review

M Al-Shraim  1 , B Mahboub, P C Neligan, D Chamberlain, D Ghazarian
Affiliations
Review

Primary pleural epithelioid haemangioendothelioma with metastases to the skin. A case report and literature review

M Al-Shraim et al. J Clin Pathol. 2005 Jan.

Abstract

Epithelioid haemangioendothelioma (EHE) is a rare vascular tumour of intermediate behaviour. It can arise from various sites including the liver, spleen, pleura, or lung. Cutaneous EHE can be primary or secondary. This report describes the case of a 51 year old man who presented with a history of dry cough, shortness of breath, and pleural effusion, and who developed two cutaneous nodules in the anterior abdominal wall a few weeks later. He had a previous history of asbestos exposure. Computed tomography scan showed a left sided pleural effusion and nodular pleural mass. Histology of both the pleural and cutaneous lesions was compatible with EHE. Electron microscopic examination demonstrated the presence of Weibel-Palade bodies. The patient underwent elliptical excision of the metastatic cutaneous nodules after decortication of the primary pleural tumour and adjuvant treatment. A few reports have described metastasis of intrathoracic EHE to the skin. Despite treatment with interferon, the patient developed more cutaneous lesions two years after the initial diagnosis. Even though the tumour has the classic light histological and ultrastructural features of EHE, it behaved in an aggressive manner.

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Figures

Figure 1
Figure 1
Computed tomography scan showing pleural thickening, a nodular mass in the inferior left pleural surface, and a pleural effusion with multiple gas loculations.
Figure 2
Figure 2
(A) Haematoxylin and eosin (H&E) section of pleura disclosing trabeculae of epithelioid tumour cells with cytoplasmic vacuoles and lumens (original magnification, ×200); (B) H&E section of skin showing epithelioid haemangioendothelioma cells separated by collagen fibres (original magnification, ×200).
Figure 3
Figure 3
Immunoperoxidase staining of the tumour showing positive immunoreactivity for (A) CD31, (B) CD34, (C) monoclonal carcinoembryonic antigen, and (D) vimentin.
Figure 4
Figure 4
Electron micrograph showing Weibel-Palade bodies.
See this image and copyright information in PMC

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