Intrathoracic ganglioneuroma and ganglioneuroblastoma: report of four cases

BACKGROUND: Intrathoracic tumors arising from primitive sympathetic ganglion cells are a rare entity in childhood. Their histopathological appearance and biological characteristics range from benign (ganglioneuromas) to malignant (ganglioneuroblastomas) and aggressively malignant tumors (neuroblastomas). Apart from this fact they all grow expansively and typically spread into the neuroforamina, which can cause damage to the spinal cord. Most of these tumors are asymptomatic for a long time and detected accidentally. Treatment options include surgery, chemotherapy, and radiation therapy. Surgery alone represents the primary treatment of low-risk tumors. Chemotherapy is used for advanced stages of disease and is most effective in ganglioneuroblastomas. Radiotherapy is reserved for unresectable tumors unresponsive to chemotherapy. METHODS: Within the last 6 years, four patients were treated at the University Hospital Innsbruck for thoracic neoplasms of the sympathetic chain. 3 patients had a ganglioneuroblastoma and one patient a ganglioneuroma. All tumors extended to two or more neuroforamina. In three children, total excision of the tumors was possible. In one case, to achieve complete resection, the lower cervical ganglion had to be sacrificed, which caused a Horner syndrome. The fourth patient underwent incomplete resection with adjuvant chemotherapy. RESULTS: During an observation period ranging from 12 months to 6 years all patients are without recurrent disease. CONCLUSIONS: Intrathoracic tumors of the sympathetic chain are rare in childhood. The broad spectrum of clinical behavior can range from a benign tumor to an aggressive disease with metastatic dissemination. Generally treatment consists of surgery and chemotherapy. Prognosis of ganglioneuroma and ganglioneuroblastoma after radical resection is excellent.