Last reviewed: November 2017
Last updated: November  2017

Introduction

Conditions relevant to Brain tumour, overview

Condition
Description

Primary tumour of the cranial and spinal compartments. Estimates indicate that meningioma represent over 36% of primary brain tumours and 53.5% of all non-malignant tumours. [2] They are more common in women, and are usually benign. Neurological deficit and progressive, focal, or general headaches (in large tumours) are common. Tumours may also produce visible bony growth. Diagnosis is confirmed by the characteristic appearance on magnetic resonance imaging (MRI), with and without contrast enhancement. Asymptomatic lesions may be followed up with serial observation. [3] Treatment of symptomatic meningioma is usually surgical resection, [4] although, in some cases, local radiation therapy is used as primary treatment. [5]

Benign, slow-growing cerebellopontine angle tumour that grows from the superior vestibular component of the vestibulocochlear nerve. Usually presents with unilateral sensorineural hearing loss, [6] [7] but may be found on routine hearing examinations. Progressive dizziness and unilateral facial numbness are also common. Neurofibromatosis type 2, a rare autosomal dominant disorder, is a strong risk factor. Diagnosis is confirmed by gadolinium-enhanced MRI. Observation may be appropriate management, [8] with stereotactic radiation or radiosurgery or formal surgical resection as definitive treatment options. [9] [10]

Malignant, invasive brain tumour arising from the cerebellar vermis. The vast majority of medulloblastomas arise sporadically in the first 2 decades of life, and they are one of the most common brain tumours of childhood. [11] Symptoms, as a result of a mass effect from the tumour or due to obstructive hydrocephalus, include morning headaches, nausea, vomiting, diplopia (a manifestation of sixth nerve palsy), and ataxia. Vomiting often relieves the headaches. Cranial CT and MRI are essential diagnostic and postoperative investigations. Primary treatment is surgical resection with postoperative chemotherapy and irradiation, depending on risk stratification. [11]

Primary tumour of the brain arising from astrocytes, which are an important part of the blood-brain barrier. A neuroepithelial type tumour categorised by histological type and grade with each subtype having a different age-adjusted incidence rate, behaviour, and clinical course. [2] Patients may present with focal neurological deficits according to location (e.g., frontal, temporal, cerebellum, brainstem) or with signs of elevated intracranial pressure. It is more common in industrial countries and in white males. [12] [13] Diagnosis is by cranial imaging with surgical biopsy. Treatment depends on tumour grade and may include surgery, radiotherapy, and chemotherapy. Prognosis can range from good for low-grade benign to poor for high-grade astrocytomas.

Benign extra-axial nonglial epithelial tumour of the CNS, seen in both children and adults. Craniopharyngiomas most commonly arise within the sellar/suprasellar space. Although it may present at any age, a bimodal age distribution is reported with a peak between 5 and 14 years in children and between 50 and 70 years in adults. [14] [15] [16] Craniopharyngiomas will cause mass effect symptoms, including visual loss or symptoms of intracranial hypertension. [17] Pituitary dysfunction is also common; children may present with growth failure and adults with diabetes insipidus and sexual dysfunction. [18] Diagnostic evaluation includes cranial CT/MRI and full endocrine evaluation. Primary treatment is surgical resection, with postoperative radiotherapy in select cases. [16] [18]

An uncommon tumour, accounting for <1% of all non-Hodgkin's lymphoma. Risk factors include immunosuppression, especially HIV infection, where an association with EBV infection is recognised. [19] Diagnosis is based on cranial CT and clinical history, with LP and CSF analysis. The most effective treatment is methotrexate-based chemotherapy with adjunctive whole-brain radiation therapy. [20] Adding cytarabine to methotrexate is a standard approach in some centres. [21]

Clinically non-functional adenomas (CNFA) are associated with multiple endocrine neoplasia type 1 (MEN-1), [22] familial isolated pituitary adenomas, [23] and Carney complex. [24] May present with features of hormonal insufficiency. Most symptoms are longstanding and progress slowly. Diagnosis is via endocrine evaluation and cranial imaging. Treatment strategies include observation with serial imaging, surgical resection, and radiotherapy. [25]

Acromegaly is due to a pituitary somatotroph adenoma in about 99% of cases. Pituitary somatotroph adenomas chronically secrete excessive GH, which stimulates insulin-like growth factor-1 (IGF-1) production, leading to the majority of the clinical manifestations of the acromegaly. [26] Diagnosis is based on biochemical confirmation of IGF-1 hypersecretion. Treatment includes surgical resection and/or somatostatin analogues. [27] [28]

Cushing's disease, which is hypercortisolism caused by an ACTH-secreting pituitary adenoma, is the most common cause of Cushing's syndrome and is responsible for the majority of cases. Diagnosis is by demonstration of unsuppressed ACTH and subsequent cranial MRI. First-line therapy is generally transsphenoidal surgical resection.

Benign prolactin-expressing and secreting pituitary adenoma. It is more frequent in women, mainly during the childbearing years. [29] Prolactinoma leads to hyperprolactinaemia, which results in hypogonadism, sexual dysfunction, and galactorrhoea, and it may also cause hypopituitarism and osteoporosis. There may be symptoms of mass effect. Assessment of serum prolactin levels with cranial imaging are standard diagnostic tests. [30] Medical treatment with dopamine agonists is the primary treatment for both sexes, with surgery reserved for refractory cases. [29] [30]

Pituitary adenomas may be divided into 2 types: clinically non-functional adenoma (CNFA), where there is no hypersecretion of hormone, and functional adenoma, where there is hypersecretion of hormone. Functional adenomas are found in acromegaly, Cushing's disease, and prolactinoma. Compression of adjacent structures may cause mass effect symptoms (visual disturbances, ophthalmoplegia, headaches).

The majority of patients presenting with acute headache have a benign diagnosis, but a high index of suspicion should be maintained for life-threatening causes of headache. [31] Most brain tumours causing headaches can be seen on non-enhancing contrast CT.

Headaches are common in children, increasing in incidence from early childhood to adolescence. They account for 0.7% to 1.3% of all paediatric emergency visits. [32] [33] Of children with brain tumours, 62% have headache prior to diagnosis, and 98% have at least one neurological symptom or abnormality on examination. [34]

Ataxia can be hereditary or acquired. It can be cerebellar, sensory, or vestibular in origin. The list of acquired causes is extensive and includes vascular, demyelinating, neoplastic, autoimmune, toxic, degenerative, compressive, and infectious aetiologies.

Hyperprolactinaemia may be caused by prolactinomas (micro- or macroadenomas), acromegaly, or interruption of the hypothalamic-pituitary axis.

Short stature can be caused by craniopharyngioma or Cushing's syndrome during childhood, as well as other aetiologies. Craniopharyngioma typically presents with diplopia, vision loss, headache, and short stature. [16]

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This overview has been compiled using the information in existing sub-topics.

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