原発性肺癌，肺動静脈瘻，肝動脈瘤および肝血管腫を合併したOsler-Rendu-Weber病の1剖検例

Abstract

A 63-year-old male was hospitalized because of nasal and gingival bleeding. Owing to characteristic small and red-to-violet telangiectatic lesions on his face, lips, oral, nasal mucosa, and finger tips, he was diagnosed as having Osler-Rendu-weber disease. Biopsy of the mucosa of his lower lip showed marked telangiectasia. After several months, we found association of primary pulmonary cancer, pulmonary arteriovenous fistulae, hepatic aneurysms and hepatic hemangiomas. After a few months, the patient died of disseminated intravascular coagulation. Autopsy and pathological examination, revealed primary large cell carcinoma of the lung, metastatic lesions in the brain and the liver, hepatic aneurysms and hepatic hemangiomas. Pulmonary arteriovenous fistulae are frequently complicated with this disease. To our knowledge, this is the first report on Osler-Rendu-Weber disease with primary lung cancer. Histopathologically, pulmonary arteriovenous fistulae were seen in the right lung and primary lung cancer was found in the left lung. The etiological relationship between the fistulae and the cancer was not recogized. It was suggested that the rapid metastasis to several organs could have been due to pulmonary arteriovenous fistulae.