Neurogenic tumors of the thorax

Abstract

We have conducted a retrospective review of 134 cases of neurogenic tumors of the thorax, including 66 in infants and children and 68 in adults. Nerve cell tumors were the majority in infants and children (84.8%) and were mostly malignant (67.2%). Nerve sheath tumors were more frequent in adults (73.5%). Nerve cell tumors were the most frequent tumors in males and nerve sheath tumors in females. Nineteen tumors were associated with von Recklinghausen's disease. The tumors were symptomatic in 76.4% of children and 36.7% of adults. Spinal cord compression was observed in 8 infants and children and in 2 adults. Neo-adjuvant treatment was administrated to 3 patients. Resection was complete for 80 benign tumors out of 86 (93%) and for 26 malignant tumors out of 48 (54%). Postoperative chemo- or radiotherapy (or both) was administrated to 17 children and 8 adults. The mean followup periods were 11 years for the infants and children and 8 years, 6 months for the adults. There was one postoperative death. There were no late deaths related to benign tumor. The prognosis of spinal cord compression depended on the malignancy and staging of the tumors. At 5 years postoperatively, 21 children out of 28 with neuroblastomas and 8 out of 9 with ganglioneuroblastomas were alive. The possibility of maturation of neuroblastomas and survival with hepatic metastases was confirmed. The prognosis in cases of chemodectoma depended on the extension. Patients with malignant schwannomas had very poor prognoses, especially when associated with von Recklinghausen's disease.