Intrathoracic ganglioneuroblastoma

Thirty-one patients treated for isolated intrathoracic ganglioneuroblastoma have been evaluated. The surviving patients (27/31) have been followed for periods up to 25 years. The modes of treatment consisted of complete or partial resection of the tumor, radiation therapy, or chemotherapy. Analysis of the data revealed that four patients treated with radiation alone died within 3 months after the start of treatment. Two patients had complete removal of the tumor without adjuvant therapy. Each is free of recurrent disease at 10 and 11 years postoperatively. The other 25 survivors had complete or incomplete surgical resection followed by radiation and/or chemotherapy. All are free of recurrent disease. Of the 25 patients who received postoperative radiation, 11 developed moderate-to-severe skeletal deformity. There appeared to be a correlation between deformity and the dose of radiation. Our current treatment recommendation consists of immediate operative intervention with an attempt at complete removal of the tumor. Patients with incomplete removal of tumor should be treated with radiation (2,000 r). Chemotherapy consisting of methotrexate or a combination of cyclophosphamide (Cytoxan) and vincristine should be reserved for patients with distant metastasis.