Last reviewed: 10 Nov 2024
Last updated: 05 Jan 2024

This page compiles our content related to brain tumors. For further information on diagnosis and treatment, follow the links below to our full BMJ Best Practice topics on the relevant conditions and symptoms.

Introduction

ConditionDescription

Meningioma

Primary tumor of the cranial and spinal compartments. Estimates indicate that meningioma represent over 37.1% of primary brain tumors and 53.1% of all nonmalignant tumors.[3] They are more common in women, and are usually benign. The incidence of meningioma increases with age in both sexes; meningioma is the most common primary brain tumor diagnosed in adults ages over 35 years.[3]​ Neurologic deficit and progressive, focal, or general headaches (in large tumors) are common. Tumors 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.[4]

Acoustic neuroma (vestibular schwannoma)

Benign, slow-growing cerebellopontine angle tumor that grows from the superior vestibular component of the vestibulocochlear nerve. They are the third most common intracranial benign tumor in adults and represent over 80% of all cerebellopontine angle tumors.[3][5]​​ Usually presents with unilateral sensorineural hearing loss, but may be found on routine hearing examinations.[6][7]​ 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.[5] 

Medulloblastoma

Malignant, invasive brain tumor arising from the cerebellar vermis. The vast majority of medulloblastomas arise sporadically in the first 2 decades of life; medulloblastoma is the most common malignant brain tumor of childhood, representing 15% to 20% of all brain tumors. Symptoms, as a result of a mass effect from the tumor or due to obstructive hydrocephalus, include morning headaches, nausea, vomiting, diplopia (a manifestation of sixth nerve palsy), and ataxia. Vomiting often relieves the headaches. Computed tomography and MRI are essential diagnostic and postoperative investigations.

Gliomas

Primary tumor of the brain arising from glial progenitor cells. Patients may present with focal neurologic deficits according to tumor location (e.g., frontal, temporal, cerebellum, brainstem) or with signs of elevated intracranial pressure. Gliomas are more common in industrial countries and in white males.[8][9]​​​​ Median age at diagnosis is 65 years. Diagnosis is by cranial imaging with surgical biopsy. Prognosis can range from good for low-grade benign gliomas to poor for high-grade gliomas.

Craniopharyngioma

Benign extra-axial nonglial epithelial tumor of the central nervous system, seen in both children and adults. Craniopharyngiomas 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.[10][11][12][13]​ Craniopharyngiomas will cause mass effect symptoms, including visual loss or symptoms of intracranial hypertension.[14] Pituitary dysfunction is common; children may present with growth failure and adults with diabetes insipidus and sexual dysfunction.[15] Diagnostic evaluation includes cranial MRI, computed tomography, and full endocrine evaluation. 

Primary CNS lymphoma

An uncommon tumor, accounting for <1% of all non-Hodgkin lymphoma (NHL). Median age at diagnosis is 67 years. Men are more likely to be diagnosed with NHL than women.[16]​ NHL is more common in white people than in black, Hispanic, or Asian people.[16]​ Risk factors include immunosuppression, especially HIV infection, and Epstein-Barr virus infection.[17] Diagnosis is based on history, physical exam, laboratory investigations, imaging (computed tomography/positron emission tomography), tissue biopsy, and cell markers obtained from immunohistochemical, flow cytometric, and cytogenetic testing.

Nonfunctional pituitary adenoma

Clinically nonfunctional adenomas are associated with multiple endocrine neoplasia type 1, familial isolated pituitary adenomas, and Carney complex.[18][19][20]​​Pituitary adenomas account for about 15% of all intracranial tumors.[21]​ May present with features of hormonal insufficiency. Most symptoms are longstanding and progress slowly. Diagnosis is via endocrine evaluation and cranial imaging.

Acromegaly

Acromegaly is due to a pituitary somatotroph adenoma in about 95% to 99% of cases. Acromegaly is a rare condition with an insidious onset, often resulting in diagnostic delay.[22][23]​​​ Pituitary somatotroph adenomas chronically secrete excessive growth hormone, which stimulates insulin-like growth factor-1 (IGF-1) production, leading to the majority of the clinical manifestations of the disease.[24] Diagnosis is based on biochemical confirmation of IGF-1 hypersecretion.

Cushing syndrome

Cushing disease, which is hypercortisolism caused by an adrenocorticotropic hormone-secreting pituitary adenoma, is the most common cause of Cushing syndrome and is responsible for 70% to 80% of cases. Cushing syndrome occurs 3 times more commonly in women than in men, and Cushing's disease has a 3:1 to 5:1 female-to-male predominance.[25]​ Diagnosis is by demonstration of unsuppressed adrenocorticotropic hormone and subsequent cranial MRI.

Prolactinoma

Benign prolactin-expressing and secreting pituitary adenoma. They are the most common type of pituitary adenoma, constituting about 50% of these tumors.[26][27]​​​ It is more frequent in women, mainly during the childbearing years.[28] Prolactinoma leads to hyperprolactinemia, which results in hypogonadism, sexual dysfunction, and galactorrhea. There may be symptoms of mass effect. Assessment of serum prolactin levels and cranial imaging are standard diagnostic tests.[29]

Evaluation of pituitary mass

Pituitary adenomas may be divided into 2 types: functional adenoma, where there is hypersecretion of hormone, and clinically nonfunctional adenoma (nonhormone secreting). Functional adenomas are found in acromegaly, Cushing disease, and prolactinoma. Compression of adjacent structures may cause mass effect symptoms (e.g., visual disturbances, headaches).

Evaluation of acute headache in adults

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.[30] Most brain tumors causing headaches can be seen on nonenhancing contrast computed tomography.

Evaluation of acute headache in children

Headaches are common in children, increasing in incidence from early childhood to adolescence. They account for 0.7% to 1.3% of all pediatric ER visits.[31][32] Of children with brain tumors, 62% have headache prior to diagnosis, and 98% have at least one neurologic symptom or abnormality on examination.[33]

Evaluation of ataxia

Ataxia can be hereditary or acquired. The list of acquired causes is extensive and includes toxic, vascular, infectious/postinfectious, neoplastic, and autoimmune etiologies.

Evaluation of hyperprolactinemia

Hyperprolactinemia is a condition of elevated serum prolactin; it is the most common endocrine disorder of the hypothalamus-pituitary axis. Among women presenting with concomitant amenorrhea and galactorrhea, 70% are hyperprolactinemic.

Evaluation of short stature

Defined as a height that is 2 or more standard deviations below the mean for age and sex within a population (below the 2.5th percentile). Causes of short stature include those that are due to normal variation, responsible for the majority of cases, and those due to disease pathology (e.g., endocrine causes, genetic syndromes, chronic illness).

Contributors

Authors

Editorial Team

BMJ Publishing Group

Disclosures

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

Use of this content is subject to our disclaimer