Cord Compression in the oncology patient
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Please discuss patient urgently with the paediatric oncologist on call. Discuss the use of steroids prior to transfer. Patient transfer should be arranged urgently without an attempt to get tissue for diagnosis - this can be arranged at the oncology centre. It may be useful to keep patients nil by mouth if surgical or radiological procedures are likely to be necessary at the oncology centre.
Cord compression occurs in about 5% of all children with cancer at some stage during the disease process. In children, epidural disease is the most common cause of cord compression. This is most likely due to dumbbell extension of tumour through the intervertebral foramina from an extra-spinal site.
Other causes include:
- intradural metastases of CNS tumours
- vertebral compression secondary to bony metastases or crush fractures from prolonged steroid use
- direct invasion of intraspinal tumours.
Neuroblastoma is the most common cause of spinal cord compression at diagnosis, followed by soft tissue sarcomas and lymphoma. Overall, soft tissue sarcomas are the most common tumours in children over five years, while neuroblastoma is the most common in under five year olds.
With quick, appropriate management, 50% of children who are unable to walk at presentation regain independent ambulation.
The investigation of choice for spinal imaging is MRI. Indications for MRI include:
- any neurological deficit suggestive of cord compression
- urinary retention/leakage
- constipation, overflow, or lack of anal sensation
- weakness, numbness or parathesia of limbs
- decreased sensation with a sensory level on the torso
- decreased reflexes
- paravertebral neuroblastoma
- unexplained localized backache. Backache in children is more common than appreciated. However, backache that is persistent or worsening, wakes a child from sleep, or pain that breaks through non-steroidal therapy requires further investigation. Imaging may be appropriate after full physical and neurological exam.
- Cord compression is an oncological emergency. Contact the paediatric oncologist on call urgently.
- Keep nil by mouth.
- Send urgent spot urine for catecholamines for new patients (discuss with specialist biochemistry lab).
- start dexamethasone 1 mg/kg IV followed by 0.25 mg/kg/dose q6h. The dose is controversial but the usual dose is a maximum of 20mg as an initial bolus and a maximum of 12mg q8h. Please discuss with the paediatric oncologist or paediatric neurosurgeon on call.
- If lymphoma is part of the differential diagnosis, biopsy should be obtained within 24 hours of starting therapy.
Cord compression at diagnosis
It has been shown for neuroblastoma and Ewings/PNET that chemotherapy is as good as surgery in relieving cord compression. Rhabdomyosarcoma may also invade the intervertebral foramen - it rarely produces cord compression, but is chemosensitive. Therefore, the principles of management should apply to this tumour as well.
First line treatment for most tumour types is steroids followed by chemotherapy. Surgery, particularly multilevel laminectomy should be avoided in a child if possible because this can lead to orthopaedic instability later in life. Urgent external beam radiotherapy should also be considered, depending on the clinical scenario.
Biopsy extraspinal component only. This can occasionally cause swelling of the intraspinal component and further neurological deterioration.
Tumours with intraspinal component only
Discuss with neurosurgeons. Plan for surgical decompression.
- The important principle here is to avoid drugs that require hyperhydration which has been known to cause tumour swelling and neurological deterioration. Therefore, cisplatin and higher doses of cyclophosphamide should not be given initially.
- The chemotherapy combination may be dictated by the study on which the child is entered.
Cord compression presenting in previously diagnosed patients
Therapy depends on the child's current progress and expected
Chemotherapy is likely to be less effective initially than in newly diagnosed patients. Radiotherapy, surgery, chemotherapy or no treatment may be appropriate depending on the clinical circumstances. Follow the initial steps above and discuss with the on call oncologist urgently.
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- Date last published: 25 November 2016
- Document type: Clinical Guideline
- Services responsible: National Child Cancer Network
- Owner: Stephen Laughton
- Review frequency: 2 years
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