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ALL / Endocrinology
Neuroblastoma
Neuroblastoma
Neuroblastoma is one of the top five causes of cancer in children, accounting for around 7-8% of childhood malignancies. The tumour arises from neural crest tissue of the adrenal medulla (the most common site) and sympathetic nervous system.Median age of onset is around 20 months
Features
- abdominal mass
- pallor, weight loss
- bone pain, limp
- hepatomegaly
- paraplegia
- proptosis
Investigation
- urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels
- neuroblastoma arises from sympathetic neural crest cells that produce catecholamines (dopamine, noradrenaline)
- these catecholamines are excessively released into circulation by tumour cells
- dopamine → homovanillic acid (HVA) and noradrenaline → vanillylmandelic acid (VMA), causing raised urinary HVA and VMA levels
- raised in > 90%
- imaging:
- ultrasound (abdomen/adrenal) - first-line imaging if mass suspected.
- MRI or CT scan - defines the primary tumour and local spread (e.g. spinal canal extension). MRI preferred for paraspinal lesions.
- metaiodobenzylguanidine (MIBG) scan - most specific for neuroblastoma – detects primary and metastatic disease (MIBG taken up by adrenergic tissue).
+PassMedicine Notes
Neuroblastoma is one of the top five causes of cancer in children, accounting for around 7-8% of childhood malignancies. The tumour arises from neural crest tissue of the adrenal medulla (the most common site) and sympathetic nervous system.
Median age of onset is around 20 months
Features- abdominal mass
- pallor, weight loss
- bone pain, limp
- hepatomegaly
- paraplegia
- proptosis
- urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels
- neuroblastoma arises from sympathetic neural crest cells that produce catecholamines (dopamine, noradrenaline)
- these catecholamines are excessively released into circulation by tumour cells
- dopamine → homovanillic acid (HVA) and noradrenaline → vanillylmandelic acid (VMA), causing raised urinary HVA and VMA levels
- raised in > 90%
- imaging:
- ultrasound (abdomen/adrenal) - first-line imaging if mass suspected.
- MRI or CT scan - defines the primary tumour and local spread (e.g. spinal canal extension). MRI preferred for paraspinal lesions.
- metaiodobenzylguanidine (MIBG) scan - most specific for neuroblastoma – detects primary and metastatic disease (MIBG taken up by adrenergic tissue).
