History and Physical Examination

The onset of symptoms is generally insidious, with a delay of approximately 1-2 years between initial symptoms and diagnosis. The clinical presentation can include a wide range of symptoms, which depend on the location of the tumor and involvement of adjacent structures. Headache is the most common presenting symptom, followed by endocrine deficiencies and visual disturbances.

Headache is usually due to either the tumor’s mass effect or hydrocephalus (from obstruction of the foramen of Monro, third ventricle, or aqueduct of Sylvius), which occurs in 15-30% of patients. This obstructive hydrocephalus may, on rare occasions, necessitate emergency neurosurgical management.

Endocrine disturbances are related to direct compression from the tumor. Endocrine deficiency most commonly involves growth hormone (75%), followed by gonadotropin (40%), thyroid-stimulating hormone (25%), and corticotropin (25%).^[8] Growth failure may be noted in as many as 93% of children with craniopharyngioma and is related to growth-hormone deficiency, hypothyroidism, or both. Adults have more varied presentation and may develop sexual or menstrual dysfunction, with 88% of men experiencing decreased sex drive and 82% of women experiencing amenorrhea. Other endocrine dysfunction may lead to precocious puberty and obesity.

Large tumors in adults can cause psychiatric symptoms, memory loss, apathy, incontinence, depression, and hypersomnia. Long-standing cognitive deficits and profound memory loss have been reported and suggest a worse prognosis. Visual deficits are caused by compression of the optic chiasm from suprasellar tumor growth. Classically, the tumor presents as a bitemporal hemianopsia, but it may also manifest as homonymous hemianopsia, scotoma, or optic atrophy with papilledema.

Other presenting symptoms can include chemical meningitis from rupture of cyst contents into the subarachnoid space and resulting in headaches, stiff neck, and seizures.

Workup

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Media Gallery

Coronal MRI shows a craniopharyngioma in the suprasellar space that causes compression of the optic nerves and chiasm.
Sagittal MRI shows a cystic craniopharyngioma in the suprasellar space with extension into the third ventricle.
Axial MRI shows a craniopharyngioma cyst that contains proteinaceous fluid in the third ventricle. The cyst fluid appears hyperintense.
Dissection of craniopharyngioma cyst with aspiration.

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Author

Lawrence S Chin, MD, FACS, FAANS Robert B and Molly G King Endowed Professor and Chair, Department of Neurosurgery, State University of New York Upstate Medical University

Lawrence S Chin, MD, FACS, FAANS is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association for Cancer Research, Children's Oncology Group, Society for Neuro-Oncology, Congress of Neurological Surgeons, American Association of Neurological Surgeons, American College of Surgeons, Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Ryszard M Pluta, MD, PhD (Retired) Associate Professor, Neurosurgical Department Medical Research Center, Polish Academy of Sciences, Poland; (Retired) Clinical Staff Scientist, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health (NIH); (Retired) Fishbein Fellow, JAMA

Ryszard M Pluta, MD, PhD is a member of the following medical societies: Congress of Neurological Surgeons, Polish Society of Neurosurgeons

Disclosure: Nothing to disclose.

Chief Editor

Brian H Kopell, MD Associate Professor, Department of Neurosurgery, Icahn School of Medicine at Mount Sinai

Brian H Kopell, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, American Society for Stereotactic and Functional Neurosurgery, Congress of Neurological Surgeons, International Parkinson and Movement Disorder Society, North American Neuromodulation Society

Disclosure: Received consulting fee from Medtronic for consulting; Received consulting fee from Abbott Neuromodulation for consulting; Received Consulting Fee from ClearPoint Neuro.

Additional Contributors

Paul L Penar, MD, FACS Professor, Department of Surgery, Division of Neurosurgery, Director, Functional Neurosurgery and Radiosurgery Programs, University of Vermont College of Medicine

Paul L Penar, MD, FACS is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, World Society for Stereotactic and Functional Neurosurgery, Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

Mayur Jayarao, MD, MSc

Disclosure: Nothing to disclose.

Gentian Toshkezi, MD Assistant Clinical Professor, Department of Neurological Surgery, Jefferson Torresdale Hospital and Thomas Jefferson University Hospital

Gentian Toshkezi, MD is a member of the following medical societies: American Academy of Neurological Surgery, American Society for Neural Therapy and Repair, Congress of Neurological Surgeons, North American Skull Base Society

Disclosure: Nothing to disclose.

Surgery for Craniopharyngiomas Clinical Presentation

History and Physical Examination

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