Intrathoracic tumours in von Recklinghausen's neurofibromatosis.

Abstract

We review our cases of a rare pathology, intrathoracic tumours in von Recklinghausen's neurofibromatosis, involving 6 patients operated upon in our Surgery Department for intrathoracic neurogenic tumors. All had had a positive history of von Recklinghausen's neurofibromatosis prior to thoracic surgery. The management of this rare condition is discussed. We describe the presentation of the symptoms, the operative technique and the long-term outcome, when obtainable. Our results are compared with those of other authors, and the management of these cases is reconsidered. In conclusion, we recommend that thoracic surgery should only be considered when the patient becomes symptomatic, since malignant changes in the tumour are possible due to operative trauma.