Fatal intrathoracic hemorrhage in a patient with von Recklinghausen's disease.
Neurofibromatosis can involve the mediastinum. A 44-year-old woman with a dumbbell-shaped mediastinal mass developed a large pleural effusion, respiratory failure and fatal hemoptysis. Autopsy revealed systemic neurofibromatosis involving the mediastinum and pleura. Mediastinal and pleural hemorrhage probably occurred as a result of an eroded thoracic artery. Massive hemorrhage in mediastinal neurofibromatosis occurs uncommonly but with potentially fatal results.
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