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

¹ Division of Pathology, Princess Margaret Hospital, Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, M5G2M9, Canada
² Division of Respirology, Department of Medicine, University of Toronto
³ Division of Plastic Surgery, Department of Surgery, University of Toronto
⁴ Division of Pathology, Toronto General Hospital, Department of Laboratory Medicine and Pathobiology, University of Toronto

Correspondence to:
Dr D Ghazarian
University Health Network, Princess Margaret Hospital, Suite 4-302, 610 University Avenue, Toronto, Ontario, M5G2M9, Canada; danny.ghazarian{at}uhn.on.ca
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.

Abbreviations: CEA, carcinoembryonic antigen; CT, computed tomography; EHE, epithelioid haemangioendothelioma

Keywords: epithelioid haemangioendothelioma; skin; pleura; asbestos