Benign nonglial epithelial central nervous system tumor, constituting 1% to 3% of intracranial tumors 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, radiation therapy may be required.
The majority of patients require adjunctive endocrine replacement therapy.
Craniopharyngiomas are benign, extra-axial, nonglial epithelial tumors of the central nervous system 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
Other diagnostic factors
- symptoms of hypogonadotropic hypogonadism (amenorrhea, erectile dysfunction)
- symptoms of intracranial hypertension (nausea, vomiting, decreased sensorium, diplopia)
- optic atrophy
- age 5 to 14 years
- age 50 to 70 years
1st investigations to order
- ophthalmologic evaluation; computerized visual-field examination
- 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 growth hormone (GH) tests
- serum luteinizing hormone
- serum follicle-stimulating hormone
- morning serum testosterone
- serum thyroid-stimulating hormone and T3/T4
- morning serum cortisol and adrenocorticotropic hormone (ACTH)
- serum electrolytes
- urine and serum osmolality
- urine specific gravity
- plain x-rays for bone age
Investigations to consider
- tissue histology
at initial treatment
post initial treatment
Marc C. Chamberlain, MD
Division of Neuro-Oncology
Department of Neurology and Neurological Surgery
University of Washington
Seattle Cancer Care Alliance
MCC declares that he has no competing interests.
Dr Marc C. Chamberlain would like to gratefully acknowledge Dr Daniel L. Silbergeld, a previous contributor to this topic.
DLS declares that he has no competing interests.
Larry Junck, MD
Professor of Neurology
Department of Neurology
University of Michigan
LJ declares that he has no competing interests.
Jeff Raizer, MD
Associate Professor of Neurology
Director of Medical Neuro-Oncology
JR declares that he has no competing interests.
Ramez Kirollos, MBChB, FRCS(Ed), FRCS(Eng), MD, FRCS(SN)
Cambridge University Hospitals NHS Foundation Trust
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
KA declares that he has no competing interests.
Edwin S. Kulubya Jr., MD, MBA
Neurosurgical Research Fellow
UC Davis Medical Center
EK declares that he has no competing interests.
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