|Lhermitte Duclos Disease
| Primary CNS Lymphoma (PCNSL)
- Can be primary brain tumours arising from glial cells/neurons or other tissues.
- Also can be secondary from other sites. Neuroimaging and possible biopsy indicated.
- 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
- Symptoms due to local effects and a general rise in ICP
- Childhood tumours 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 tumours
- Cavernous sinus tumours - 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 tumours - Unilateral deafness, facial weakness, ataxia and nystagmus
- 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 tumours 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 tumours 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 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)
- 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 tumours
- 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.
- Neurosurgical Debulking can improve the prognosis for malignant tumours 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 tumours are radiosensitive and it can be curative or prolong survival. Whole-brain radiotherapy is used in certain tumours like medulloblastoma and primary CNS lymphomas. “Involved field” radiotherapy is used in other tumours 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