The Journal of Thoracic and Cardiovascular Surgery, Vol 80, 605-612, Copyright © 1980 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
Intrathoracic ganglioneuroblastoma
R Zajtchuk, TE Bowen, AE Seyfer and WH Brott
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.
