EVALUATION OF NEUROBLASTOMA PATIENTS: ANALYSIS OF 42 CASES OF ISTANBUL PEDIATRIC ONCOLOGY GROUP

Neuroblastoma, the most common extracranial solid tumour of childhood, has poor prognosis in advanced stages. In this study demographic data and clinical outcome of 43 neuroblastoma cases (24 female, 18 male) followed up between October 1989 and December 1998 have been assessed retrospectively. Median age was 39 months (1 months-15 years). Three patients were diagnosed by elevated urine VMA levels and bone marrow infiltration; the rest were diagnosed histopathologically. Three patients were diagnosed as ganglioneuromas. Primary localization was abdominal in 34 (79%) patients, mediastinal in 6 (14%), thoracoabdominal in 2 (4.5%) and renal in 1 (2.5%). According to the CCG staging system 3 (7%) patients were stage II, 14 (33%) stage III and 26 (60%) stage IV. Most frequent sites of metastasis were bone, bone marrow, liver, lymph nodes and lungs. Seven of 43 cases were consulted only; 36, all bf whom were in advanced stage, received chemotherapy (8 patients OPEC and 28 modified 6-in-one chemotherapy)±radiotherapy (n=11). Objective response after four courses of chemotherapy was 93%. Patients who attained partial remission underwent second-look or delayed primary look surgery. Therapy was completed to eight courses. Retinoic acid ±interferon were used in three patients for microscopic residual disease. Twenty one patients died of disease, 2 were lost to follow-up, 7 are alive with no evidence of disease, 6 are alive with disease. Two and 5 year survival are 45% and 13% respectively. Modified 6-in-1 chemotherapy could not change the dismal outcome in this series with advanced stage neuroblastomas, as in other series receiving conventional chemotherapy. Efforts in improving outcome by the use of myeloablative chemotherapy + transplantation and/or addition of differentiation agents for microscopic residual disease are continuing.