Mediastinal tumors associated with von Recklinghausen's disease

Abstract

Four cases of mediastinal tumors associated with von Recklinghausen's disease are reported. Two neurofibromas arose from the vagus nerve, one of those is revealed histologically malignant lesion inside a benign tumor. One was a very rare invasive thymoma in a patient with myasthenia gravis, associated with an anterior chest wall malignant schwannoma which developed in the field of irradiation of the thymoma. The fourth patient was suspected to have neurofibroma with destruction of ribs, and also had a giant mesenteric neurofibroma. Although von Recklinghausen's disease is generally thought not to be associated with an abnormality of chromosomes, one of the three patients had abnormal chromosomes. We conclude that surgical treatment and careful follow-up are required in patients with von Recklinghausen's disease because of the possibility of malignant changes.