SDL 8 Intracranial Space Occupying Lesion BMS16091064 Jonathan

Introduction
An “Intra-cranial space occupying lesion” (ICSOL)is defined as a mass lesion in the
cranial cavity with a diverse aetiology like benign or malignant
neoplasm,inflammatory or parasitic lesion, haematoma, or arterio-venous
malformation.

Brain tumours
Primary brain tumours are a heterogeneous collection of neoplasms arising from the
brain tissue or meninges, and vary from benign to highly malignant. Primary
malignant brain tumours are rare, accounting for 1% of all adult tumours but a higher
proportion in children. The most common benign brain tumour is a meningioma.

Primary brain tumours do not metastasise due to the absence of lymphatic drainage in
the brain. There are rare pathological subtypes, however, such as medulloblastoma,
which do have a propensity to metastasise; the reasons for this are not clear.

Most cerebral tumours are sporadic but may be associated with genetic syndromes
such as neurofibromatosis or tuberous sclerosis. Gliomas account for 60% of brain
tumours, with the aggressive glioblastoma multiforme (WHO grade IV) the most
common glioma, followed by meningiomas (20%) and pituitary tumours (10%).
Gliomas
These malignant tumours of neuroepithelial origin are usually seen within the
hemispheres, but occasionally in the cerebellum, brainstem or cord. Their cause is
unknown. Gliomas are occasionally associated with neurofibromatosis. They tend to
spread by direct extension, virtually never metastasizing outside the CNS

. • Astrocytomas are gliomas that arise from astrocytes. They are classified
histologically into grades I–IV. Grade I astrocytomas grow slowly over many years,
while grade IV tumours (glioblastoma multiforme) cause death within several months.
Cystic astrocytomas of childhood are relatively benign and usually cerebellar.

• Oligodendrogliomas arise from oligodendrocytes. They grow slowly, usually over

several decades. Calcification is common.

Neurofibromas (schwannomas)

These solid benign tumours arise from Schwann cells and occur principally in the
cerebellopontine angle, where they arise from the VIIIth nerve sheath (acoustic
neuroma; . They may be bilateral in neurofibromatosis type 2

Other neoplasms
Other less common neoplasms include cerebellar haemangioblastomas, ependymomas
of the IVth ventricle, colloid cysts of the IIIrd ventricle, pinealomas, chordomas of the
skull base, glomus tumours of the jugular bulb, medulloblastomas (a cerebellar
childhood tumour), craniopharyngiomas and primary CNS lymphomas
Meningiomas
These benign tumours arise from the arachnoid and may grow to a large size, usually
over years. Those close to the skull erode bone or cause local hyperostosis. They often
occur along the intracranial venous sinuses, which they may invade. They are unusual
below the tentorium. Common sites are the parasagittal region, sphenoidal ridge,
subfrontal region, pituitary fossa and skull base.
Clinical features
The presentation is variable and usually influenced by the rate of growth. High-grade
disease (WHO grades III and IV) tends to present with a short (weeks) history of mass
effect (headache, nausea secondary to RICP), while more indolent tumours can
present with slowly progressive focal neurological deficits, depending on their
location; generalised or focal seizures are common in either. Headache, if present, is
usually accompanied by focal deficits or seizures, and solated stable headache is
almost never due to intracranial tumour.
The size of the primary tumour is of far less prognostic significance than its location
within the brain. Tumours within the brainstem will result in early neurological
deficits, while those in the frontal region may be quite large before symptoms occur.

Mass lesions within the brain produce symptoms and signs by three mechanisms:

• direct effect – brain is infiltrated and local function impaired • secondary effects of
raised intracranial pressure and shift of intracranial contents (e.g. papilloedema,
vomiting, headache) • provocation of generalized and/or partial seizures. Although
neoplasms, either secondary or primary, are the most common mass lesions in the UK,
cerebral abscess, tuberculoma, neurocysticercosis, and subdural and intracranial
haematomas can also produce features that are clinically similar.

Direct effects of mass lesions

The hallmark of a direct effect of a mass is local progressive deterioration of
function. Tumours can occur anywhere within the brain.

Three examples are given:

• A left frontal meningioma caused a frontal lobe syndrome over several years with
vague disturbance of personality, apathy and impaired intellect. Expressive aphasia
developed, followed by progressive right hemiparesis as the corticospinal pathways
became involved. As the mass enlarged further, pressure headaches and papilloedema
occurred.

• A right parietal lobe glioma caused a left homonymous field defect (optic
radiation). Cortical sensory loss in the left limbs and left hemiparesis followed over 3
months. Partial seizures (episodes of tingling of the left limbs) developed.

• A left VIIIth nerve sheath neurofibroma (acoustic neuroma, schwannoma) in

the cerebellopontine angle caused, over 3 years, progressive deafness (VIII), left
facial numbness (V) and weakness (VII), followed by cerebellar ataxia on the same
side.

With a hemisphere tumour, epilepsy and the direct effects commonly draw attention
to the problem. The rate of progression varies greatly, from a few days or weeks in a
highly malignant glioma, to several years with a slowly enlarging mass such as a
meningioma. Cerebral oedema surrounds mass lesions; its effect is difficult to
distinguish from that of the tumour itself.
Investigations

Imaging
Both CT and MRI are useful in detecting brain tumours; MRI is superior for posterior
fossa lesions. Benign and malignant tumours, brain abscess, tuberculosis,
neurocysticercosis and infarction have characteristic, but not entirely reliable,
appearances, and refined imaging techniques and biopsy are often necessary. MR
angiography is used occasionally to define blood supply and MR spectroscopy to
identify patterns typical of certain gliomas. PET is sometimes helpful to locate an
occult primary tumour with brain metastases.

Routine tests Since metastases are more common than primary tumours, routine tests,
such as chest X-ray, should be performed.

Lumbar puncture This is contraindicated when there is any possibility of a mass

lesion, as withdrawing CSF may provoke immediate coning. CSF examination is
rarely helpful and has been superseded by imaging.

Biopsy and tumour removal

Stereotactic biopsy via a skull burr-hole is carried out to ascertain the histology of
most suspected malignancies. With a benign tumour, such as a symptomatic,
accessible meningioma, craniotomy and removal are usual.

Management
Cerebral oedema surrounding a tumour responds rapidly to steroids: intravenous or
oral dexamethasone. Epilepsy is treated with anticonvulsants.

While complete surgical removal of a tumour is an objective, it is often not possible;

nor is surgery always necessary. Follow-up with serial imaging is sometimes
preferable in low- grade gliomas. At exploration, some benign tumours can be entirely
removed (e.g. acoustic neuromas, some parasagittal meningiomas). With a malignant
tumour, it is not possible to remove an infiltrating mass entirely. Biopsy and
debulking are performed.

Within the posterior fossa, tumour removal is often necessary because of raised
pressure and danger of coning. Overall mortality for posterior fossa exploration
remains around 10%.

For gliomas and metastases, radiotherapy is usually given and improves survival, if
only slightly. Solitary metastases can often be excised successfully. Chemotherapy
has little real value in the majority of primary or secondary brain tumours. Vincristine,
procarbazine and temozolomide (an oral alkylating agent) can be used. Bevacizumab
has been shown to have some benefit, although no effect on overall survival. Most
malignant gliomas have a poor prognosis despite advances in imaging, surgery,
chemotherapy and radiotherapy: survival for grade IV gliomas at 2 years is less than
50%. Surgical debulking and radiotherapy improve survival by 4–5 months.