Makindo Medical Notes.com

A Neuroblastoma is the most common solid tumour in children that occurs outside of the brain and makes up 8% of the total number of children's cancers.

About

• Embryonal tumour of neural crest origin
• Less than 100 children develop neuroblastoma in the UK each year

Anatomy

• Tumour usually in adrenal medulla and sympathetic ganglia
• Spectrum of disease from benign ganglioneuroma to malignant
• Tumour can be anywhere from neck to pelvis

Clinical

• Most common in the first years of life usually before 5 years old
• Poor appetite, weight loss, lethargy, Constipation, difficulty passing urine
• Bruising around the eyes, breathlessness, leg weakness (this is less common

Investigations

• Urine: vanillylmandelic acid (VMA) and homovanillic acid (HVA). Levels of these are often elevated at diagnosis.
• Chest X-ray and Ultrasound scan: will show tumour
• CT scan: stage tumour size and spread. Tumour can be large and cross the midline and envelop other organs. Paravertebral tumour can cause cord compression.
• Biopsy of tumour
• MIBG (meta-iodo-benzyl guanidine) scan: identifies spread to bone, bone marrow and lymph nodes
• Bone marrow aspirate and trephine
• Bone scan: tell if bony spread.

Poorer prognosis

• N-myc oncogene expression
• Deletion of material Chromosome 1 and addition Chr 17
• Age greater than 1 year old
• Stage, size and spread of tumour

Staging

• Stage I: The tumour is localised with or without a microscopic residual tumour after surgery.
• Stage IIA: The tumour is localised but cannot be completely removed by surgery.
• Stage IIB: The tumour is localised but has spread to local lymph nodes.
• Stage III: The tumour crosses the midline, with or without local lymph nodes and cannot be removed surgically. There is localised tumour with lymph node involvement on the opposite side. There is a midline tumour with lymph nodes on both sides.
• Stage IV: The tumour has spread to distant lymph nodes, bone marrow, bone, liver and/or other organs.
• Stage IVS: The tumour is localised (stage I, IIA or IIB), with spread limited to liver, skin and/or bone marrow in an infant under one year of age.

Management

• Depends on the age, size and position of the tumour and whether it has spread.
• Surgery: Stage I/II can be completely removed will be treated with surgery alone or after chaemotherapy.
• Chemotherapy: Some will need high-dose chaemotherapy with a stem cell transplant.
• Monoclonal antibodies can destroy some types of cancer cells while causing little harm to normal cells. Some may receive a monoclonal antibody called anti-GD2 after their high dose therapy. At present, antibody treatment is given in combination with a medicine taken by mouth called retinoic acid. This medicine helps to stop cancer cells from growing and instead helps them to develop into non-cancerous cells.
• External beam radiotherapy: may be used after surgery or high dose chaemotherapy to destroy any remaining tumour cells.

References