Pleuropulmonary
angiosarcomas are very rare, with less than fifty cases reported in the literature. In most cases, the etiology is unknown but the presence of a chronic tuberculous
pyothorax has been reported in several Asian case reports as a possible risk factor. We report the case of a Caucasian 68-year old man who presented with a pleuropulmonary
angiosarcoma that arose from a chronic tuberculous
pyothorax and which involved the ribs and the vertebrae, the psoas muscle, and the jejunum. The patient received adapted anti-
tuberculosis treatment, embolization of the mass in the small bowel, palliative external beam
radiotherapy on the spine and systemic
chemotherapy with liposomal non-pegylated
doxorubicin and
ifosfamide. With this multidisciplinary approach the patient's symptoms were well controlled and he achieved a complete metabolic response after six cycles of
chemotherapy. Unfortunately, the patient died after eight months from the beginning of
chemotherapy due to an
acute lung injury secondary to extensive bilateral interstitial infiltrates. Opportunistic pathogens or
drug-induced lung toxicity were the most probable causes. Treatment with liposomal non-pegylated
doxorubicin and
ifosfamide could be a reasonable option in pleuropulmonary
angiosarcoma but it should be validated in clinical trials. Chronic
pyothorax seems to be a predisposing factor for the development of pleural
angiosarcoma but further investigations are required to assess a causal association.