Craniopharyngioma

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Last reviewed: December 2018

Last updated: February 2018

Summary

Benign non-glial epithelial CNS tumour, constituting 1% to 3% of intracranial tumours in adults, most often located near the optic chiasm.

Bimodal age incidence with 50% in children aged between 5 and 14 years and a second peak in adults 50 to 70 years of age.

Common acute presentations are sudden visual loss and symptoms of intracranial hypertension.

Pituitary dysfunction is also common; children may present with growth failure and adults with diabetes insipidus and sexual dysfunction.

Primary treatment consists of surgery. Following surgery, radiotherapy may be required.

The majority of patients require adjunctive endocrine replacement therapy.

Definition

Craniopharyngiomas are benign, extra-axial, non-glial epithelial tumours of the CNS and are seen in both children and adults. They most commonly arise within the sellar/suprasellar space. Clinically, craniopharyngiomas cause mass effect symptoms, including visual loss or symptoms of intracranial hypertension. In addition, pituitary dysfunction is common and the majority of patients will have an associated endocrinopathy.

History and exam

Key diagnostic factors

visual loss
macrocephaly and hydrocephalus
growth failure

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Other diagnostic factors

symptoms of hypogonadotrophic hypogonadism (amenorrhoea, erectile dysfunction)
headache
symptoms of intracranial hypertension (nausea, vomiting, decreased sensorium, diplopia)
galactorrhoea
optic atrophy
polyuria/polydipsia

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Risk factors

age 5 to 14 years
age 50 to 70 years

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Diagnostic investigations

1st investigations to order

MRI brain (contrast-enhanced)
CT brain (contrast-enhanced)
serum prolactin
serum growth hormone (GH)
serum insulin-like growth factor 1 (IGF-1)
serum insulin-like growth factor binding protein-3 (IGFBP-3)
provocative GH tests
serum LH
serum FSH
morning serum testosterone
serum TSH and T3/T4
morning serum cortisol and ACTH
serum electrolytes
urine and serum osmolality
urine specific gravity
ophthalmological evaluation; computerised visual-field examination
plain x-rays for bone age

Full details

Investigations to consider

tissue histology

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Treatment algorithm

ACUTE

at initial treatment

ONGOING

post initial treatment

Contributors

Authors VIEW ALL 

Marc C. Chamberlain, MD

Marc C. Chamberlain

Chief

Division of Neuro-Oncology

Department of Neurology and Neurological Surgery

University of Washington

Seattle Cancer Care Alliance

Seattle

WA

Disclosures

MCC declares that he has no competing interests.

Daniel L. Silbergeld, MD

Daniel L. Silbergeld

Professor of Neurological Surgery and Pathology (Neuropathology)

University of Washington

Seattle

WA

Disclosures

DLS declares that he has no competing interests.

Peer reviewers VIEW ALL 

Larry Junck, MD

Professor of Neurology

Department of Neurology

University of Michigan

Ann Arbor

MI

Disclosures

LJ declares that he has no competing interests.

Jeff Raizer, MD

Associate Professor of Neurology

Director of Medical Neuro-Oncology

Northwestern University

Chicago

IL

Disclosures

JR declares that he has no competing interests.

Ramez Kirollos, MBChB, FRCS(ed.), FRCS (Eng.), MD, FRSC (NeuroSurg.)

Consultant Neurosurgeon

Addenbrooke's Hospital

Cambridge University Hospitals NHS Foundation Trust

Cambridge

UK

Disclosures

RK declares that he has no competing interests.

Keyoumars Ashkan, BA, BSc, MB BCh, MRCP, FRCS, FRCPS, FRCS(SN), MD

Consultant Neurosurgeon and Lead of Neuro-Oncology

King's College Hospital

London

UK

Disclosures

KA declares that he has no competing interests.

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