[Multifocal epithelioid haemangioendothelioma: a difficult diagnosis]

Epithelioid haemangioendothelioma is a rare vascular tumour of slow growth and unfavourable outlook, with occasional spontaneous and complete remissions.

We report the case of a 69 year old woman admitted to hospital on account of haemoptysis. The clinical and radiological findings were compatible with diffuse pulmonary haemorrhage of unknown aetiology. Lung biopsy revealed a picture of pulmonary haemorrhage without vascular changes or tumour cells. Further progress was characterised by recurrent haemoptysis and the development of a haemothorax, liver nodules and skin lesions, biopsy of which confirmed the diagnosis of epithelioid haemangioendothelioma. The patient died several weeks later.

The pulmonary localisation of epithelioid haemangioendothelioma is non specific, represented mainly by parenchymatous nodules with or without accompanying haemoptysis. Haemothorax is a more uncommon presentation of this disorder and a pleural localisation is often associated with a multifocal and aggressive form. The pathological diagnosis is most often made by surgical lung biopsy. Nevertheless in certain cases it can be difficult to make and this case report shows that, in the presence of a haemothorax, the search for extrapulmonary deposits accessible for biopsy may prove useful.