Neurogenic intrathoracic tumors. A clinicopathological review of 92 cases.

Ninety-two cases of intrathoracic neurogenic tumors operated on between 1950 and 1982 are reviewed. The benign forms (86 cases, 93.5%) included 23 ganglioneuromas, 50 neurilemmomas and 13 neurofibromas. Of the latter, 4 occurred in patients with Von Recklinghausen's disease. A double local recurrence was observed after the removal of a neurilemmoma. One of the patients with generalized neurofibromatosis died 5 months after operation from local sarcomatous degeneration and distant metastases. In this group of benign lesions, no other death was observed which could be attributed to the endothoracic neural tumor. The malignant forms (6 cases, 6.5%) included 4 ganglioneuroblastomas and 2 neurofibrosarcomas. The surgical excisions were described as radical in every case and all the patients were given radiotherapy postoperatively. One patient with ganglioneuroblastoma died from metastases 2 years later, and one with neurofibrosarcoma from local recurrences 7 months later. One patient with neurofibrosarcoma and 3 with ganglioneuroblastomas are alive and well 4, 5, 6 and 11 years, respectively, later. Surgical excision remains the best method of diagnosing and treating endothoracic tumors of neural origin. Of special interest are the 'dumbbell' or hourglass tumors and lesions which occur in Von Recklinghausen's disease.