Neuroblastoma: an analysis of 160 cases

Neuroblastoma was observed in 160 patients from 1948–1978. Ninety-seven patients were boys and 63 were girls. At diagnosis, 74 patients were less than 2 years of age, 28 between 2–3 years, and 58 over 3 years. Sixty-two (38%) patients had localized disease, while 98 (62%) had metastases. Patients were grouped by extent of disease according to the staging criteria of Evans et al.: stage I (5), stage II (31), stage III (26), stage IV (82), stage IV-S (16). Tumors occurred in the neck (3), mediastinum (16), abdomen (136), and pelvis (3). Clinical findings often included abdominal mass, weight loss, anemia, bone pain, and proptosis. Six patients had diarrhea and 3 had cerebellar ataxia and nystagmus. Lesions were often calcified (>50%), and bone marrow aspirate frequently demonstrated tumor clumps (rosettes). Urinary VMA was elevated in 85% of cases. Therapy varied according to stage. Stage I patients received operative excision alone and stage II patients operative resection with radiation for residual tumor and/or positive lymph nodes. Stage III patients were managed aggressively with operative resection (when possible), irradiation, and combination chemotherapy (cyclophosphamide, vincristine, DTIC, Adriamycin®, VM-26). Patients with metastases (stage IV) were initially treated with multiagent chemotherapy with late “second-look” or delayed primary laparotomy for tumor resection done in clinical responders. Two-year disease-free survival occurred in 57 of 160 patients or 35.6%. Survival rates were best for infants under age 1 year (74%) and for patients with stage I (100%), stage II (74%) and stage IV-S (75%) tumors. There was improved survival in patients with tumors that occurred in the neck (100%), pelvis (100%), and mediastinum (75%). Survival rates were poor in patients over 2 years of age (13–17%), with abdominal tumors (28%), and with stage III (34%) and stage IV (10%) tumors. While chemotherapy and irradiation have improved tumor response, survival rate has not been improved. Immunotherapy has been disappointing. Unfortunately, at the present time, there is no specific chemotherapeutic agent that has a curative effect on this tumor.