Benign nonglial epithelial CNS 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 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
- 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
- ophthalmologic evaluation; computerized visual-field examination
- plain x-rays for bone age
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.
Daniel L. Silbergeld, MD
Professor of Neurological Surgery and Pathology (Neuropathology)
University of Washington
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, FRSC (NeuroSurg.)
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.
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