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RESEARCH PRODUCT

Retroperitoneal neuroblastomas - a single institution experience in 90 patients

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Purpose Nowadays neuroblastomas are detected incidentally by ultrasound in early stages. Primary surgery, chemotherapy and/or radiotherapy are treatment options. However, the approach in babies is controversial. A surveillance strategy is propagated by some groups. In this retrospective study, we evaluated age and stage at diagnosis, therapy and outcome of patients treated at our institutions since 1970. Material and methods 90 patients with retroperitoneal neuroblastomas were treated. They were divided into 2 groups according to age at time of diagnosis:  1 year. Results 40 patients were  1 year (1- 18 years). Detection of an asymptomatic tumour:  1 year 10%. Tumor stage at diagnosis > 1 year Stage I 4 3 Stage II 4 2 Stage III 17 10 Stage IV 14 35 One patient in each group could not be classified adequately. Therapy > 1 year Resection alone 13 4 Resection + Chemo 11 14 Resection + Radio-chemo 5 12 Chemo alone 8 10 Radio-chemo alone 3 10 In 5/40 patients  1 year: 15/50 patients (30%) have no evidence of disease, 33/50 patients (66%) died, 8 of which > 2 years after diagnosis and 2 patients (4%) have residual tumour. Conclusions In patients with a neuroblastoma, early detection by ultrasound at a younger age and lower stage of disease compared to detection by clinical symptoms is associated with an improvement of prognosis. For babies the prognosis is good, even in advanced stages requiring aggressive therapy. Due to the large size of most high stage tumours, expectant management seems not to be justified. Patients older than 1 year with a stage III-IV disease have a poor prognosis even with aggressive treatment.