Multiple pulmonary neurofibromas with hypoxemia. Occurrence due to pulmonary arteriovenous shunts within the tumors.
A 62-year-old woman with multiple neurofibromas of the lung was found to have severe hypoxemia due to right-to-left shunting within the tumors. Pulmonary angiograms demonstrated that the major area of shunting was in a large tumor mass in the right lower lobe. Pathologically the neurofibromas were vascular with hyperplastic small arteries and arterioles and large dilated veins. Multiple pulmonary neurofibromas are rare; and, to our knowledge, never previously reported in association with pulmonary arteriovenous shunting.
D005260:Female; D006801:Humans; D000860:Hypoxia / Q000209:etiology*; D008175:Lung Neoplasms / Q000098:blood supply* / Q000150:complications / Q000473:pathology; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary; D009455:Neurofibroma / Q000098:blood supply* / Q000150:complications / Q000473:pathology; D011652:Pulmonary Circulation