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Craniopharyngioma

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Other Names:
Rathke's pouch tumor; Craniopharyngeal duct tumor; Adamantinoma; Rathke's pouch tumor; Craniopharyngeal duct tumor; Adamantinoma; Adamantinomatous tumor; Dysodontogenic epithelial tumor See More
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Craniopharyngioma is a slow-growing, non-cancerous brain tumor that develops near the pituitary gland (a small endocrine gland at the base of the brain which produces several important hormones) and the hypothalamus (an endocrine organ which controls the release of hormones by the pituitary gland).[1][2][3] This tumor most commonly affects children between 5 and 10 years of age; however, adults can sometimes be affected.[1] Although craniopharyngiomas are not cancerous, they may grow and press on nearby parts of the brain, causing symptoms including hormonal changes, vision changes, slow growth, headaches, nausea and vomiting, loss of balance, hearing loss, and changes in mood or behavior.[2] The cause of these tumors is not well understood; however, researchers suspect that they begin to form during the early stages of embryo development in pregnancy (embryogenesis) and may result from metaplasia (abnormal transformation of cells).[2][4] Treatment for craniopharyngioma varies and may involve surgery to remove the tumor, radiation therapy, chemotherapy, biologic therapy, and/or hormone therapy to replace various hormones no longer produced or secreted due to the tumor or its treatment.[2][5]

 

Last updated: 7/31/2018
Craniopharyngioma may cause symptoms by increasing the pressure on the brain (intracranial pressure), disrupting the function of the pituitary gland, and/or damaging the optic nerve. Symptoms vary from person to person and depend on the specific location of the tumor and its relationship to other adjacent structures.[1][4][6][2]
  • Increased pressure on the brain may cause hydrocephalus, headache, nausea, vomiting (especially in the morning), and difficulty with balance.
  • Damage to the pituitary gland may cause hormone imbalances that can lead to many signs and symptoms such as:
  • deficiency of growth hormone, gonadotropin, thyroid stimulating hormone, and/or adrenocorticotropic hormone
  • excessive thirst and urination (diabetes insipidus)
  • sleep disturbances
  • delayed puberty and stunted growth
  • weight gain or obesity, fatigue, cold intolerance, constipation, decreased mental function, behavioral symptoms (hypothyroidism symptoms)
  • adrenal failure symptoms (heart arrhythmias, confusion, lethargy, orthostatic hypotension, low blood sugar)
  • changes in personality or mood (such as feeling depressed or having anxiety)
  • Damage to the optic nerve may result in vision problems.[1][4][6][7]
Symptoms are often permanent, and may be worse after surgery to remove the tumor.[1] People with craniopharyngioma often have impaired psychosocial health in addition to physical health, both of which contribute to reduced quality of life.[7]

About 80% of adults with this tumor complain of decreased sexual drive, and almost 90% of men complain of impotence, while most women have absent menses (amenorrhea).[4]

Because craniopharyngioma typically is a slow-growing tumor, symptoms frequently develop very slowly. The time interval between the onset of symptoms and diagnosis usually ranges from 1-2 years.[4]
Last updated: 7/5/2018

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 41 |
Medical Terms Other Names
Learn More:
HPO ID
100% of people have these symptoms
Abnormal hypothalamus morphology
Abnormal shape of hypothalamus
0012286
80%-99% of people have these symptoms
Cerebral calcification
Abnormal deposits of calcium in the brain
0002514
Enlarged pituitary gland 0012505
Intracranial cystic lesion 0010576
Neoplasm of the anterior pituitary 0011750
30%-79% of people have these symptoms
Abnormal visual field test 0030588
Bitemporal hemianopia 0030521
Central adrenal insufficiency 0011734
Central diabetes insipidus 0000863
Excessive daytime somnolence
More than typical sleepiness during day
0001262
Headache
Headaches
0002315
Hypogonadotropic hypogonadism 0000044
Increased circulating prolactin concentration 0000870
Nausea and vomiting 0002017
Obesity
Having too much body fat
0001513
Papilledema 0001085
Pituitary hypothyroidism
Low thyroid gland function due to abnormal pituitary gland
0008245
Progressive visual field defects 0007987
Slow decrease in visual acuity
Slow decrease in sharpness of vision
0007924
5%-29% of people have these symptoms
Cerebral ischemia
Disruption of blood oxygen supply to brain
0002637
Delayed puberty
Delayed pubertal development
Delayed pubertal growth
Pubertal delay
[ more ] 		0000823
Hearing impairment
Deafness
Hearing defect
[ more ] 		0000365
Hydrocephalus
Too much cerebrospinal fluid in the brain
0000238
Increased intracranial pressure
Rise in pressure inside skull
0002516
Increased susceptibility to fractures
Abnormal susceptibility to fractures
Bone fragility
Frequent broken bones
Increased bone fragility
Increased tendency to fractures
[ more ] 		0002659
Optic atrophy 0000648
Polyphagia
Voracious appetite
0002591
Proportionate short stature 0003508
Sleep apnea
Pauses in breathing while sleeping
0010535
Type II diabetes mellitus
Noninsulin-dependent diabetes
Type 2 diabetes
Type II diabetes
[ more ] 		0005978
1%-4% of people have these symptoms
Abnormality of the frontal bone
Abnormality of the bone of the forehead
0430000
Abnormality of the nasal bone 0010939
Coma 0001259
Global developmental delay 0001263
Intellectual disability
Mental deficiency
Mental retardation
Mental retardation, nonspecific
Mental-retardation
[ more ] 		0001249
Myocardial infarction
Heart attack
0001658
Postnatal growth retardation
Growth delay as children
0008897
Recurrent infections
Frequent infections
Frequent, severe infections
Increased frequency of infection
infections, recurrent
Predisposition to infections
Susceptibility to infection
[ more ] 		0002719
Seizure 0001250
Sudden loss of visual acuity 0001117
Vertigo
Dizzy spell
0002321
Showing of 41 |
Last updated: 7/1/2020
The cause of these tumors is not well understood; however, researchers suspect that they begin during the early stages of development in pregnancy (embryogenesis) and may result from metaplasia (abnormal transformation of cells). Craniopharyngiomas are thought to arise from epithelial remnants of the craniopharyngeal duct or Rathke's pouch, (an outpouch of the primitive oral cavity that forms the anterior part of the pituitary gland). Embryonic cells (early fetal cells) from abnormal development of the craniopharyngeal duct or anterior pituitary gland may give rise to a craniopharyngioma. These tumors are closely related to another cystic mass occasionally seen in the pituitary called Rathkes cleft cyst.[3][4][8]
Last updated: 3/31/2017
Treatment for craniopharyngiomas has been controversial. Two main management options have primarily been used: attempt at complete removal (gross total resection), or partial removal (partial resection) followed by radiation therapy to treat residual disease.[6] Advances in techniques used in neurosurgery have made total resection possible in more cases, but improvements in radiation therapy techniques have also made this option more effective and safe. There currently is no firm consensus of opinion regarding the best treatment option.[6] Unfortunately, even following treatment, many people experience endocrine, vascular, neurologic, and/or visual complications. Psychological, social, and emotional problems are also common.[6][9] Panhypopituitarism (when the pituitary gland does not make most or all hormones) and hypothalamic dysfunction are prevalent and are treated with hormone therapy to replace various pituitary and hypothalamic hormone deficiencies.[5] Early studies suggest that oxytocin (a less understood hormone often not replaced) may benefit some people with certain symptoms of hypothalamic dysfunction such as obesity and behavioral issues; however, additional research on larger groups of people is needed to determine its role in therapy.[9][5][10]

An experienced multidisciplinary team of specialists (neurosurgeon, radiation oncologist, neuro-oncologist, endocrinologist, ophthalmologist) is essential for the best treatment of both pediatric and adult patients with craniopharyngiomas.[6]
Last updated: 7/31/2018
The prognosis for each patient depends on several factors, including the ability of the tumor to be completely removed and the presence of neurological problems or hormonal imbalances caused by the tumor prior to treatment, as well as caused by the treatment itself. Most of the problems with hormones and vision do not improve with treatment, and sometimes the surgery can make them even worse, because it may damage the brain structures neighboring the tumor.[1]

Craniopharyngiomas tend to develop again, mostly in the first 3 years after surgery. Overall recurrence rates range from 0-17% after total removal of the tumor and from 25-63% after partial removal of the tumor with radiotherapy.[4] 
Last updated: 3/31/2017

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
The differential diagnosis includes other tumours occurring in this region (pituitary adenomas), infectious or inflammatory processes (eosinophilic granulomas), vascular malformations (aneurysms) and congenital anomalies (Rathke's cleft cysts).
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Craniopharyngioma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Craniopharyngioma. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • I underwent surgery for a craniopharyngioma last year. Although my cognitive symptoms are improved, I continue to suffer from hypersomnolence. Can you provide me with information? See answer


  1. Craniopharyngioma. MedlinePlus. 7/30/2014; http://www.nlm.nih.gov/medlineplus/ency/article/000345.htm.
  2. General Information About Childhood Craniopharyngioma. National Cancer Institute (NCI). June 10, 2016; http://www.cancer.gov/types/brain/patient/child-cranio-treatment-pdq.
  3. Garnett MR, Puget S, Grill J & Sainte-Rose C. Craniopharyngioma. Orphanet. 2007; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=54595.
  4. George C Bobustuc, George I Jallo, Franco DeMonte, Gregory N Fuller, Morris D Groves, Lee S Hwang. Craniopharyngioma. Medscape. Oct 27, 2014; http://emedicine.medscape.com/article/1157758.
  5. Hsu EA, Miller JL, Perez FA, Roth CL. Oxytocin and Naltrexone Successfully Treat Hypothalamic Obesity in a Boy Post-Craniopharyngioma Resection. J Clin Endocrinol Metab. February 1, 2018; 103(2):370-375. https://www.ncbi.nlm.nih.gov/pubmed/29220529.
  6. Harsh GR, Recht LD & Marcus KJ. Craniopharyngioma. UpToDate. Waltham, MA: UpToDate; October, 2017; http://www.uptodate.com/contents/craniopharyngioma.
  7. Roemmler-Zehrer J, Geigenberger V, Störmann S. Specific behaviour, mood and personality traits may contribute to obesity in patients with craniopharyngioma. Clin Endocrinol (Oxf). January, 2015; 82(1):106-114. https://www.ncbi.nlm.nih.gov/pubmed/24923438.
  8. Craniopharyngioma. Pituitary Network Association. 2013; https://pituitary.org/knowledge-base/disorders/craniopharyngioma. Accessed 8/5/2016.
  9. Hoffmann A, Özyurt J, Lohle K, Reichel J, Thiel CM, Müller HL. First experiences with neuropsychological effects of oxytocin administration in childhood-onset craniopharyngioma.. Endocrine. April, 2017; 56(1):175-185. https://www.ncbi.nlm.nih.gov/pubmed/28213803.
  10. Daubenbüchel AM, Hoffmann A, Eveslage M, et al. Oxytocin in survivors of childhood-onset craniopharyngioma. Endocrine. November, 2016; 54(2):524-531. https://www.ncbi.nlm.nih.gov/pubmed/27585663.