Neuroblastoma : The most common extracranial solid tumour of childhood. 80% are diagnosed <4 years old. The tumour is of neural crest origin; 50% occur in the adrenal gland and most of the remainder arise along the sympathetic trunks.
Presentation Systemic symptoms and signs are common: fever, abdominal pain/distension, mass, weight loss, anaemia, and bone pain. Retro-orbital metastases may cause proptosis.
Imaging and staging Ultrasound initially; CT of chest and abdomen. Calcification in tumour helps distinguish neuroblastoma from Wilms tumour. MIBG scans are very sensitive for detection of neuroblastomas. - Stage 1 Tumour confined to organ of origin and grossly complete excision - Stage 2 Unilateral tumour with residual disease post resection or lymphadenopathy - Stage 3 Tumour crossing midline or contralateral nodes - Stage 4 Metastatic disease beyond regional nodes; survival 6% - Stage 4S Unilateral tumour with metastasis limited to liver, skin, or bone marrow; survival 77%
Treatment and prognosis Surgical excision; radiotherapy; combination chemotherapy, possibly with autologous bone marrow transplantation. Stage 4S tumours may resolve with little or no treatment. Prognosis is poor except for Stages 1 and 4S disease.
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