How long will the needs last?
Brain tumour Duration Guidance
This guidance covers -:
- Tumours – benign – other / type not known
- Brain and spinal cord – cancer of
Primary brain tumours
There are around 100 different types of brain tumour and prognosis is highly variable between them. For this reason guidance on award duration is provided by tumour diagnosis in the table below. It is appropriate to use this guidance for children over 3, for children under 3 years treatment may be prolonged because a long course of chemotherapy is being given to delay radiotherapy treatment. In cases where needs are present related to disease or its treatment in the under 3s it may be most appropriate to make the initial award duration until the 4th birthday to give them time to recover from their prolonged treatment. Medical evidence will often include a proposed plan of treatment on which award duration can be based.
In order to use the table the correct histological diagnosis must be known. This information is most likely to be available from the Clinical Nurse Specialist, treating neurosurgeon, treating neuro-oncologist or the GP. Accurate information on impairment is most likely to be available from the Consultant or Clinical Nurse Specialist.
For many tumours, awards are recommended for two years in the presence of neurological deficits if needs are identified. This is because function may improve significantly over time especially with neurorehabilitation. For example, a child may learn how to walk again or their personality may substantially return to normal. Review at two years to assess residual impairment once neurorehabilitation is complete. Once recovery is complete needs may be absent or reduced. In cases where disease progresses despite treatment needs are likely to increase.
Recurrent brain tumours
If needs are identified indefinite awards are recommended.
Types of brain tumour & prognosis information
| Name of brain tumour | Features |
|---|---|
| Astrocytic tumours
There are many types of astrocytoma. | |
Pilocytic astrocytoma (WHO grade 1) and subtypes:
|
This is a slow growing type of tumour that has a good prognosis; it is often curable. The main treatment is surgery very occasionally followed by radiation therapy. Typically occur in the optic nerve, optic chiasm/hypothalamus, thalamus, basal ganglia, cerebral hemispheres, brain stem, spinal cord. 80% of astrocytomas occurring in the cerebellum are this type and are usually curable by surgery – may also be called Gilles Type A in the medical evidence. If needs are identified, a 2 year award is recommended. Recovery is expected in the typical case. Note that visual field defects are not likely to improve. Enduring side effects related to radiotherapy treatment in rare cases where this has been used. This is the commonest type of brain tumour in children and 10 year survival is around 90%. |
Diffuse astrocytoma/low-grade diffuse astrocytoma (WHO grade 2) including 3 different subtypes:
|
These are slow growing tumours that have a good prognosis. Survival at 4 years is 90%. Older children do better than younger children. The main treatment is surgery occasionally followed by radiation therapy. If needs are identified a 2 year award is recommended. If needs persist on renewal, an indefinite award is recommended. Diffuse tumours Gilles Type B astrocytomas have a worse prognosis. Recovery is expected in the typical case. Note that visual field defects are not likely to improve. Enduring side effects related to radiotherapy treatment in rare cases where this has been used. |
Anaplastic or malignant astrocytoma (WHO grade 3) |
These tumours have a poor prognosis with survival at best 40% and on average 19% at 5 years. The main treatment is surgery followed by radiation therapy. Younger children are likely to have chemotherapy. Children whose initial treatment successfully removes the entire tumour are most likely to do well (40% 5 year survival); although removal of the entire tumour with clear margins is unusual. If needs are identified, a 2 year award is recommended. If needs persist on renewal, an indefinite award is recommended. |
Glioblastoma/ malignant glioma/glioblastoma multiforme (WHO grade 4) |
These tumours have a poor prognosis with survival at best 40% and on average 19% at 5 years. The main treatment is surgery followed by radiation therapy. Younger children are likely to have chemotherapy. Children whose initial treatment successfully removes the entire tumour are most likely to do well (40% 5 year survival); although removal of the entire tumour with clear margins is unusual. If needs are identified, a 2 year award is recommended. If needs persist on renewal, an indefinite award is recommended. |
Pleomorphic xanthoastrocytoma (WHO grade 2) |
Is very rare it usually affects young adults. Recurrence free survival rates of 71% at 5 years and 61% at 10 years are reported adults. If needs are identified, a 2 year award is recommended. Recovery can be expected in the typical case. |
Subependymal giant cell astrocytoma (SEGA) (WHO grade 1) |
Very slow growing treatable tumour that mainly affects children with tuberous sclerosis (a rare genetic disorder). If needs are identified, a 2 year award is recommended. Recovery is expected in the typical case, however there may be ongoing needs unrelated to the brain tumour in this group. |
| Brain Stem Gliomas – the brain stem is the most primitive part of the brain it consists of the following structures: the pons, the midbrain, the tectum, the medulla. | |
Diffuse Intrinsic Pontine Glioma |
Surgical removal is not usually possible; radiotherapy to the head is the main treatment. Survival is poor at less than 10% at 18 months from diagnosis. If needs are identified, a 5 year award is recommended. |
Focal or low grade brain stem gliomas, this group includes pilocytic astrocytomas. |
Surgery is the main treatment of these tumours. Long term survival is seen even if residual tumour is not removed. A shunt may be required to treat hydrocephalus. Tumours that progress may be treated with conformal radiotherapy or chemotherapy or both. Prognosis is good. If needs are identified, a 2 year award is recommended. |
Neurofibromatosis and brain tumours associated with it |
Children with neurofibromatosis type 1 commonly develop slow growing brain tumours that may not require treatment or cause symptoms. Consider in each case whether there are disabling effects of the tumour and what treatment, if any, is being used. |
| Embryonal and Primitive Neuroectodermal tumours (PNETs) | |
Medulloblastoma subtypes:
|
Arise in the posterior fossa. May spread via the cerebrospinal fluid into the spinal cord. Children under 3 are in the high risk group. The main treatment is surgery; enduring neurological defects after surgery are common: mutism, suprabulbar palsy (difficulty feeding and speaking) ataxia (difficulty walking). Surgery is followed by radiotherapy with or without chemotherapy. If needs are identified, a 5 year award is recommended. 5 year survival ranges from 85% in standard risk children to 30% for high risk children. High dose chemotherapy and stem cell transplant may be used in this group. |
Supratentorial primitive neuroectodermal tumours includes:
|
Arise in the cerebrum and suprasellar region. Surgery is the main treatment but because of the location of these tumours, complete removal is rarely achieved. Radiotherapy treatment with or without chemotherapy is given after surgery. 5 year survival is 40 to 50%. High dose chemotherapy and stem cell transplant may be used in this group. 2 year awards are recommended in children aged 3 and over. |
Medulloepithelioma and Ependymoblastoma |
These are rare occurring most frequently in infants and younger children. 5 year survival is 0 to 30%. If needs are identified, a 5 year award is recommended. |
Atypical Teratoid/Rhabdoid tumour (WHO grade 4) |
Most common in children under 3, presents with hydrocephalus or regression of motor skills/ataxia. Treatment includes surgery, chemotherapy and often radiotherapy. Median survival is 12 months and 2 year survival is 14% in children who receive chemotherapy alone. Older children who receive radiotherapy median survival around 4 years. If needs are identified, a 5 year award is recommended. |
CNS germ cell tumours includes:
|
Primary treatment of germinomas is radiotherapy, this is usually curative. Other types are treated with chemotherapy. No survival data. If needs are identified, a 2 year award is recommended. If needs persist on renewal, an indefinite award is recommended. |
| Oligodendroglial tumours: there are two types of this tumour see below to be treated with radiotherapy or chemotherapy and survival is good if response to treatment is complete. | |
Oligodendroglioma (low grade, WHO grade 2)) |
Very rare in children. Good prognosis if completely excised with surgery. If needs are identified, a 2 year award is recommended. If needs persist on renewal, an indefinite award is recommended. |
Anaplastic oligodendroglioma (WHO grade 3). |
Poorly differentiated tumour with a worse outcome than low grade oligodendroglioma. If needs are identified, a 2 year award is recommended. If needs persist on renewal, an indefinite award is recommended. |
| Mixed gliomas | |
Oligoastrocytoma (low grade, WHO grade 2) |
Usually occurs in the frontal or temporal lobe. If needs are identified, a 2 year award is recommended. If needs persist on renewal, an indefinite award is recommended. |
Anaplastic Oligoastrocytoma (WHO grade 3) |
These are high grade tumours with outcomes similar to other malignant astrocytomas. If needs are identified, a 2 year award is recommended. If needs persist on renewal, an indefinite award is recommended. |
| Ependymoma – subtypes account for 9% of childhood brain tumours | |
Myxopapillary ependymoma (WHO grade 1) |
This tumour commonly affects the lower end of the spinal cord – survival is excellent. Recovery is expected in the typical case. Surgery is the main treatment with local radiotherapy performed afterwards. If needs are identified, a 2 year award is recommended. If needs persist on renewal, an indefinite award is recommended. |
Subependymoma (WHO grade 1) |
Commonly arises in the tissue lining the ventricles of the brain and may present with hydrocephalus. If needs are identified, a 2 year award is recommended. |
Ependymoma (WHO grade 2) Variants of this tumour are called: Cellular Papillary Tancytic Clear cell mixed |
5 year survival is 78%. It is difficult to completely remove these tumours surgically (25%) radiotherapy is likely to be used in every case. Some children will also have chemotherapy. Can occur in the spine and cause paraplegia that is permanent. Recovery from initial treatment is expected in the typical case. If needs are identified, a 2 year award is recommended. If needs persist on renewal, an indefinite award is recommended. |
Anaplastic ependymoma also called malignant ependymoma (WHO grade 3) |
No survival data. Survival is worst with this type of ependymoma. Treatment is surgery and radiotherapy. If needs are identified, a 2 year award is recommended. If needs persist on renewal, an indefinite award is recommended. |
| Meningioma | |
Meningioma – these can be benign or highly malignant. Benign tumours will be WHO grade 1 and malignant tumours will be grade 4. |
Account for 1-2% of brain tumours in children. In all cases outcome will depend on whether complete surgical removal is achieved. Radiotherapy may be used, chemotherapy treatment may be used. If needs are identified, a 2 year award is recommended. If needs persist on renewal, an indefinite award is recommended. |
| Pituitary tumours | |
Benign adenomas of the pituitary gland |
Around 80% of this group experience resolution of symptoms with treatment. Needs unlikely, recovery expected. Good long term prognosis. Rarely visual field problems will persist after treatment. May require hormone replacement. |
Carcinomas of the pituitary gland. |
Median survival of around 2 years. If needs are identified, a 5 year award is recommended. |
Craniopharyngoma Subtypes called: Adamantinomous Squamous papillary |
Rare – account for 6% of childhood brain tumours. These arise near the pituitary gland in the optic chiasm. Hormonal problems such as restricted growth or visual field defects are common. Hydrocephalus may occur. Long term survival is good at 79%. Main treatment is surgery. Almost all children will require life long pituitary hormone replacement therapy. Enduring complications of surgery include obesity, mood change, blindness, epilepsy, difficulty moving the eyes. Radiotherapy can be used following minimal surgery to decompress optic nerves; this can also lead to blindness, loss of pituitary function and difficulty with eye movements. Although these tumours are benign, complications of treatment are life long. If needs are identified, a 5 year award is recommended. |
