Related Subjects:
|Brain tumour s
|Astrocytomas
|Brain Metastases
|Tuberous sclerosis
|Turcot's syndrome
|Lhermitte Duclos Disease
|Oligodendroglioma
|Acute Hydrocephalus
|Intracranial Hypertension
|Primary CNS Lymphoma (PCNSL)
About
- Can be primary brain tumour s arising from glial cells/neurons or other tissues.
- Also can be secondary from other sites. Neuroimaging and possible biopsy indicated.
Different types
- Primary brain: Glial cells - Gliomas: Milder Astrocytomas to aggressive Glioblastoma multiforme, Meningioma. Oligodendroglioma
- Secondary: Breast, Lung, Colorectal, Thyroid, Testicular, Renal cell and malignant melanoma
- HIV related: non-Hodgkin's lymphomas of B-cell type
Clinical
- Symptoms due to local effects and a general rise in ICP
- Childhood tumour s are mainly posterior fossa which presents with hydrocephalus
- Headache - worse in the morning and on stopping/straining
- Changed personality, Seizures
- Stroke like episodes when tumour bleeds, Coma
Tumours and localising signs
- Olfactory groove - leads to anosmia, disinhibition and personality changes seen with frontal lobe tumour s
- Cavernous sinus tumour s - Ophthalmoplegia with involvement of III/IV and VI as well as V1 and V2
- Foster Kennedy syndrome - Usually an olfactory groove meningioma or metastases at the medial third sphenoidal wing. Compresses optic nerve causing atrophy and increased SOL causes contralateral papilloedema.
- Pituitary adenomas - Clinical hypopituitary +/- signs of excess GH/PRL/ACTH. Headache + Bitemporal hemianopia. Similar presentation with Craniopharyngioma in the suprasellar region causing pituitary dysfunction and bitemporal hemianopia in children
- Parinaud's syndrome - Tumour in pineal region impairs Up gaze or may cause hydrocephalus
- Parasagittal tumour - bilateral spastic paraparesis with lower limb weakness but of course spinal cord compression is a far more common cause
- Cerebellopontine angle tumour s - Unilateral deafness, facial weakness, ataxia and nystagmus
Investigations
- FBC, U&E, LFTs, ESR, CRP. HIV test may be indicated.
- CXR: exclude lung cancer
- Tumour markers may be useful: Alpha-fetoprotein (testicular cancer), CEA (colorectal, pancreatic, breast), CA125 (breast), S-100 (melanoma), PSA (prostate)
- CT with contrast: There is a breakdown of the blood-brain barrier such that tumour s enhance with contrast. This often shows up with a surrounding area of oedema.
- MRI with gadolinium: There is a breakdown of the blood-brain barrier such that tumour s enhance with gadolinium. This often shows up with a surrounding area of oedema.
- Look for primary: Usually a CT Chest Abdomen and pelvis. Physical exam and Mammography for breast cancer and skin examination for melanoma.
- Brain biopsy: When a tumour is identified by imaging it can then be biopsied to obtain a definite tissue diagnosis, so as to better inform the MDT as to how best to proceed with treatment.
Difficulties of Diagnosis Tumour vs Stroke
>Day 1: admitted suspected stroke but some atypical features.
Patient improved and discharged home |
> | Day 15: clinical worsening |
> | Day 18: clinical worsening |
> | Day 21 |
> | Day 21 |
> | Day 21 |
> | Day 21 with contrast |
Differential of Butterfly tumour across the Corpus: pre and post contrast
- High grade Astrocytoma, usually a Glioblastoma (WHO grade IV)
- Primary CNS lymphoma (consider HIV)
- Cerebral Toxoplasmosis (consider HIV)
> |
|
Management
- Surgery may be considered as it enables
- Surgeons to obtain a histological diagnosis
- Reduce the mass of the tumour reducing the neurological deficit
- To treat hydrocephalus if present
- Surgery can be curative for benign tumour s
- Surgery may simply debulk tumour and leave behind residual tumour and this may be palliative
- However surgery involving the brain is always high risk and any situation can be made worse through surgical and perioperative complications.
- Most assessments now are through a multidisciplinary assessment with Surgeons, Oncologists, Radiologists.
- A full discussion with the oncologists and neurosurgeons is needed before any intervention is planned with a good discussion of treatment options and their risks and benefits.
Specific Management
- Neurosurgical Debulking can improve the prognosis for malignant tumour s provided the tumour is not infiltrating essential "eloquent" areas of the brain such as language areas. If this is not possible then a simple biopsy is the second option and modern stereotactic approaches mean that this is now more possible. Excision of at least 98% of a GBM improves life expectancy by a median of 4 months compared with patients who have 2% or more of residual tumour remaining postoperatively.
- Chemotherapy is increasingly being used in the treatment of primary brain malignancies. Temozolomide, an alkylating agent which has good penetration of the blood-brain barrier thereby allowing access to brain tissue. Certain regimens in combination with radiotherapy have shown a survival benefit in gliomas compared to controls. Chemotherapy can be used also in the treatment of oligodendrogliomas
- External beam radiotherapy is useful and may be used in addition to surgery or instead of surgery. Some tumour s are radiosensitive and it can be curative or prolong survival. Whole-brain radiotherapy is used in certain tumour s like medulloblastoma and primary CNS lymphomas. “Involved field” radiotherapy is used in other tumour s such as gliomas.
- Stereotactic radiosurgery is another method of radiotherapy, which delivers a large dose of radiation to the tumour based on imaging of the lesion. This is useful for meningiomas.
- Referral to local neuro-oncology services. Oncology can discuss with Neurosurgeons whether to biopsy, remove or resect lesion.
- Much of this focuses on performance status, co-morbidities and physiological age and whether an eloquent area of the brain is involved and the scope for resection and patient choice.
- Start anticonvulsants e.g. Keppra 250-500 mg BD with one seizure as high risk of recurrence with a focal lesion.
- Hydrocephalus is an emergency that requires transfer to the Neurosurgeons for shunting
- Start Dexamethasone 10 mg IV and Dexamethasone 4 mg PO/IV QDS which can be increased to reduce oedema
- For many palliation is the correct course when there is a poor outcome.
- It is generally agreed that a high rate of recurrence in brain tumour is due to the resistance of a sub-population of cancer stem cells