Hémangioendothéliome épithélioïde pulmonaire : à propos de trois cas et revue de la littérature

Abstract

Pulmonary epithelioid haemangioendothelioma (PEH) is a rare vascular tumour of intermediate malignancy that predominantly affects women. We report three cases of PEH. Though all three diagnoses were confirmed by surgical biopsy, it is notable that, in one case, a tracheal biopsy by flexible bronchoscopy was contributory. Two patients had undergone positron emission tomography, which showed the lesions to be well established. The three cases show that the evolution of this pathology can be very varied. In the first case, the disease was multifocal and needed to be treated with several pulmonary resections. In the second case no recurrence has been observed after surgery; in the third case the patient died following a haemothorax. The discovery of a PEH is usually fortuitous. The diagnosis is confirmed by immuno-chemical analysis of a lung biopsy. No standard treatment has been validated and no treatment is necessary if the disease is asymptomatic. If there is only one nodule, surgery is the treatment of choice. The prognosis is variable. Usually the evolution is slow except when the disease is complicated by haemoptysis, pleural effusion, haemothorax, mediastinal lymphadenopathy or hepatic spread.