Malignant disease- Neuroblastoma

Question 1

Neuroblastoma

Clinical Features

• Most children have an abdominal mass, but the primary tumour can lie anywhere along the sympathetic chain from the neck to the pelvis
• abdominal primary is of adrenal origin, but at presentation the tumour mass is often large and complex, crossing the midline and enveloping major blood vessels and lymph nodes
• Paravertebral tumours may invade through the adjacent intervertebral foramen and cause spinal cord compression
• Over the age of 2 years, clinical symptoms are mostly from metastatic disease, particularly bone pain, bone marrow suppression causing weight loss and malaise

Answer 1

• Neuroblastoma and related tumours arise from neural crest tissue in the adrenal medulla and sympathetic nervous system.
• It is a biologically unusual tumour in that spontaneous regression sometimes occurs in very young infants.
• There is a spectrum of disease from the benign (ganglioneuroma) to the highly malignant (neuroblastoma).
• Neuroblastoma is most common before the age of 5 years

Question 2

Neuroblastoma

Investigations

Answer 2

• Characteristic clinical and radiological features with raised urinary catecholamine levels suggest neuroblastoma
• Confirmatory biopsy is usually obtained and evidence of metastatic disease detected with bone marrow sampling, MIBG (metaiodobenzyl-guanidine) scan
• majority of children over 1 year present with advanced disease and have a poor prognosis.

Question 3

Neuroblastoma

Management

Answer 3

• Localised primaries without metastatic disease can often be cured with surgery alone
• Metastatic disease is treated with chemotherapy, including high-dose therapy with autologous stem cell rescue, surgery and radiotherapy
• Risk of relapse is high and the prospect of cure for children with metastatic disease is still little better than 30%
• Immunotherapy and the use of long-term ‘maintenance’ treatment with differentiating agents (retinoic acid) are now establishing a role in those with high-risk disease.