Craniopharyngioma

Disease Overview

Summary

A craniopharyngioma is a benign (non-cancerous) brain tumor that forms from remaining fetal tissue. It grows in or around the sellar and parasellar regions of the brain, areas near the center of the skull, where the pituitary gland is located. The pituitary gland is sometimes called the “master gland” because it controls many of the hormones the body needs for growth, metabolism, reproduction and stress response. It is located inside a small hollow in the skull bone known as the sella turcica, which is part of a skull bone called the sphenoid.

The tumor itself is usually not dangerous, as it is benign and only very rarely becomes malignant. However, the tumor’s location is such that it can compress the pituitary gland and lead to hormonal dysfunction.

The optic chiasm, where the two optic nerves partially cross, is located above the pituitary gland and can therefore be compressed by a craniopharyngioma, leading to vision abnormalities.

The hypothalamus, to which the pituitary gland is connected, can also be damaged, especially after surgery. As the hypothalamus is a regulator of many biological functions, its damage is associated with symptoms such as obesity (hypothalamic obesity) and disruption of sleep cycles.

Another structure that can be compressed by a craniopharyngioma is the foramen of Monro within the ventricles. If the foramen is occluded, the cerebrospinal fluid that normally circulates in the brain and spinal cord will accumulate (hydrocephalus), potentially leading to numerous symptoms including an enlarged head in babies as well as headaches and nausea due to an elevation in intracranial pressure.

Craniopharyngiomas are rare, affecting about 0.5 to 2 people per million each year. They are most often diagnosed in children aged 5 to 14 or adults aged 50 to 74.

Craniopharyngiomas do not always cause obvious symptoms at first. Because of this, diagnosis may take a long time, sometimes years after symptoms begin. However, an early diagnosis can help avoid long-term complications and improve quality of life.

Craniopharyngiomas are often considered a chronic disease because they tend to come back, even after surgery. Treatment typically involves surgery to remove the tumor and may include radiation therapy if the tumor cannot be fully removed. Many patients also need hormone replacement therapy, depending on how the tumor has affected their endocrine system.

 Introduction

There are two types of craniopharyngioma based on how the tumor cells look and where they come from:

• Adamantinomatous craniopharyngioma: This type is more common in children. It develops from the remaining cells of the craniopharyngeal duct, an embryonic structure. These tumors can be solid (like a lump) or cystic (fluid-filled). The cysts often contain a thick, dark brown fluid that looks like used motor oil. They often contain areas of hardened tissue with calcium deposits (calcifications) which can show up on X-rays or CT scans.

• Papillary craniopharyngioma: This type is more common in adults. It comes from cells in the front part of the pituitary gland. These tumors are usually well-defined and can also be solid or cystic, but the fluid inside is typically yellow and thick. They rarely have calcifications.

Both types usually cause the same symptoms.

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Synonyms

• Rathke’s pouch tumor
• craniopharyngeal duct tumor

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Subdivisions

Summary

A craniopharyngioma is a benign (non-cancerous) brain tumor that forms from remaining fetal tissue. It grows in or around the sellar and parasellar regions of the brain, areas near the center of the skull, where the pituitary gland is located. The pituitary gland is sometimes called the “master gland” because it controls many of the hormones the body needs for growth, metabolism, reproduction and stress response. It is located inside a small hollow in the skull bone known as the sella turcica, which is part of a skull bone called the sphenoid.

The tumor itself is usually not dangerous, as it is benign and only very rarely becomes malignant. However, the tumor’s location is such that it can compress the pituitary gland and lead to hormonal dysfunction.

The optic chiasm, where the two optic nerves partially cross, is located above the pituitary gland and can therefore be compressed by a craniopharyngioma, leading to vision abnormalities.

The hypothalamus, to which the pituitary gland is connected, can also be damaged, especially after surgery. As the hypothalamus is a regulator of many biological functions, its damage is associated with symptoms such as obesity (hypothalamic obesity) and disruption of sleep cycles.

Another structure that can be compressed by a craniopharyngioma is the foramen of Monro within the ventricles. If the foramen is occluded, the cerebrospinal fluid that normally circulates in the brain and spinal cord will accumulate (hydrocephalus), potentially leading to numerous symptoms including an enlarged head in babies as well as headaches and nausea due to an elevation in intracranial pressure.

Craniopharyngiomas are rare, affecting about 0.5 to 2 people per million each year. They are most often diagnosed in children aged 5 to 14 or adults aged 50 to 74.

Craniopharyngiomas do not always cause obvious symptoms at first. Because of this, diagnosis may take a long time, sometimes years after symptoms begin. However, an early diagnosis can help avoid long-term complications and improve quality of life.

Craniopharyngiomas are often considered a chronic disease because they tend to come back, even after surgery. Treatment typically involves surgery to remove the tumor and may include radiation therapy if the tumor cannot be fully removed. Many patients also need hormone replacement therapy, depending on how the tumor has affected their endocrine system.

 Introduction

There are two types of craniopharyngioma based on how the tumor cells look and where they come from:

• Adamantinomatous craniopharyngioma: This type is more common in children. It develops from the remaining cells of the craniopharyngeal duct, an embryonic structure. These tumors can be solid (like a lump) or cystic (fluid-filled). The cysts often contain a thick, dark brown fluid that looks like used motor oil. They often contain areas of hardened tissue with calcium deposits (calcifications) which can show up on X-rays or CT scans.

• Papillary craniopharyngioma: This type is more common in adults. It comes from cells in the front part of the pituitary gland. These tumors are usually well-defined and can also be solid or cystic, but the fluid inside is typically yellow and thick. They rarely have calcifications.

Both types usually cause the same symptoms.

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Signs & Symptoms

Craniopharyngiomas are almost always benign, but because of their location in the brain, they can cause many serious symptoms. These symptoms happen when the tumor presses on nearby brain structures.

Pituitary gland compression: The pituitary gland helps regulate growth, metabolism, reproductive hormones and stress response. If it’s compressed, people may develop:

• Hormone deficiencies (such as low growth hormone, thyroid hormone, or sex hormones) which can result in:
  • Growth problems and short stature caused by growth hormone deficiency
  • Delayed puberty and absence of menses in females (amenorrhea) due to FSH and LH (gonadotropins) deficiency
  • Early (precocious) puberty-less commonly
• Weakness and tiredness due to ACTH (adrenocorticotropic hormone) deficiency
• Fatigue, generalized weakness, menstrual irregularity and forgetfulness caused by TSH (thyroid stimulating hormone) deficiency
• Central diabetes insipidus characterized by excessive thirst (polydipsia) and excessive urination (polyuria) due to deficits of the antidiuretic hormone (ADH)

Hypothalamus compression: The hypothalamus is connected to the pituitary gland and helps regulate appetite, sleep-wake cycles, emotions and behavior, body temperature and thirst. When affected, symptoms may include:

• Excessive weight gain (hypothalamic obesity) even with diet and exercise
• Disrupted sleep, like staying up at night and sleeping during the day
• Changes in mood, blood pressure and heart rate
• In some people, a rare condition called Froelich’s syndrome (combination of obesity, delayed puberty and small testes)

Optic chiasm compression: This structure is where the left and right optic nerves partially cross and it sits right above the pituitary gland. Pressure here can cause:

• Blurry vision
• Loss of part of the visual field (such as missing vision on the sides)

These symptoms are common and can be among the first signs of the tumor.

Foramen of Monro obstruction and hydrocephalus: The brain has hollow spaces called ventricles where cerebrospinal fluid (CSF) is made and flows. The foramen of Monro connects two of the ventricles. If the tumor blocks this channel, fluid can build up in the brain, a condition called hydrocephalus. Symptoms of hydrocephalus depend on age:

• Infants: Irritability, trouble feeding, and a large head
• Older children/adults: Headaches, neck pain, vomiting and blurred vision

Increased intracranial pressure: As the tumor grows it can raise pressure inside the skull causing:

• Headaches
• Nausea or vomiting
• Seizures
• Difficulty walking
• Muscle weakness
• Behavioral or psychiatric symptoms (like confusion or paranoia)

Because the tumor is located near delicate areas, surgery can be difficult. Even when it’s removed, craniopharyngiomas often come back, so many patients need multiple surgeries or radiation. Possible side effects related to the treatment may include:

• Damage to nearby brain areas (especially the pituitary or hypothalamus)
• Problems with memory, attention, or learning
• Hormone issues
• Blood vessel damage from radiation, leading to:
  • Aneurysms (weakened blood vessels that can rupture)
  • Moyamoya disease (narrowing of blood vessels, increasing stroke risk)
  • In rare cases, new brain tumors (like gliomas) from radiation treatment

Long term outcomes

When properly managed, people with a craniopharyngioma have an over 90% survival rate over 20 years. Many experts consider craniopharyngiomas to be a chronic disease, as tumor recurrence rates are high, even with apparent complete resection of the tumor. Significant complications that can lead to mortality include strokes, cardiac complications, respiratory complications, chronic hypothalamic insufficiency, hormonal deficiencies, and seizures. In very rare cases, craniopharyngiomas can become malignant (malignant transformation).

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Causes

Craniopharyngiomas are caused by malformations of embryonic tissue in the sellar and parasellar regions. Adamantinomatous craniopharyngioma, which occur mostly in children, and papillary craniopharyngioma, which occur mostly in adults, have different embryological origins.

Adamantinomatous craniopharyngioma

Adamantinomatous craniopharyngiomas arise from the transformation into tumor cells (neoplastic transformation) of cells from the craniopharyngeal duct, an embryological structure connected to Rathke’s pouch, which gives rise to part of the pituitary gland. The exact cause of development of adamantinomatous craniopharyngioma is unknown, although variants in the CTNNB1 or APC genes are present in more than 70% of tumors. These genes produce a protein known as beta-catenin, that is important in the development of the embryo (embryogenesis). Variants in these genes might therefore play a role in the development of adamantinomatous craniopharyngiomas.

Papillary craniopharyngioma

Papillary craniopharyngiomas are caused by a change in cell type (metaplasia) of cells in the anterior pituitary gland. This leads to the formation of nests made from a cell type known as squamous cells. As for adamantinomatous craniopharyngioma, the exact cause of development of papillary craniopharyngioma is unknown.

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Affected populations

Craniopharyngiomas occur in around 0.5 to 2 people per million each year and represent around 1.2 to 4% of all intracranial tumors in children. They mostly develop in two age groups: children aged 0 to 14 years and adults aged 50 to 74 years. They occur in males and females equally. Adamantinomatous craniopharyngioma are more common, representing 86 to 89% of all craniopharyngiomas.

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Disorders with Similar Symptoms

Disorders that can damage or compress structures in proximity to the sellar region, such as the pituitary gland, the hypothalamus and the optic chiasm can have clinical manifestations similar to those of a craniopharyngioma. Examples of conditions that can lead to compression or damage include tumors, cysts and blood vessel disruptions.

Pituitary adenomas are hormone-secreting tumors from the pituitary gland. They can secrete various pituitary hormones depending on the type of cell that is affected. This can lead to various clinical manifestations related to the hormone that is secreted in excess. For instance, a prolactinoma is a pituitary adenoma that secretes prolactin. Symptoms related to excessive prolactin include irregular menstrual periods, infertility, production of milk even if an affected female is not pregnant and erectile dysfunction in males. Pituitary adenomas can also cause compression of the pituitary gland, leading to pituitary hormone deficiencies and compression of the optic nerves and optic chiasm, leading to vision abnormalities. Some pituitary adenomas, known as non-functioning adenomas, do not secrete any pituitary hormone but can still be responsible for compressive symptoms.

Glioma is a tumor of the central nervous system that arises from glial cells, a type of cell widely present in the nervous system, mostly in the brain, and rarely in the spinal cord. They occur at various ages, depending on the subtype. Developing gliomas can compress areas of the brain where they occur and cause various symptoms including headaches, nausea, vomiting, cognitive impairment, seizures, gait imbalance, language impairment (aphasia), weakness of one side of the body (hemiparesis), visual field defects and personality changes. Gliomas compressing structures such as the pituitary gland, the hypothalamus, and the optic chiasm can have symptoms similar to those of a craniopharyngioma.

Intracranial germ cell tumors are derived from germ cells and are located within the skull. The exact mechanism for the development of intracranial germ cell tumor is not known, but embryological abnormalities play a role. They can clinically mimic craniopharyngiomas by compressing structures such as the pituitary gland, the hypothalamus and the optic chiasm.

Langerhans cells histiocytosis (LCH) is a rare disease involving the proliferation of Langerhans cells, which are derived from the bone marrow. Symptoms related to multiple organ systems can result, including hormonal abnormalities such as diabetes insipidus. If the front part of the pituitary gland is damaged by LCH, the patient may have low levels of pituitary hormones.

An aneurysm is a local weakening and outward bulging of a blood vessel. Cerebral aneurysm can bleed and rupture, leading to a sub-arachnoid hemorrhage (SAH) and symptoms such as headaches, seizures and loss of consciousness. The outward bulging of the vessel can also compress adjacent anatomical structures. Visual symptoms and hormonal deficiencies can occur if the aneurysm is located near the pituitary gland or an optic nerve.

Similarly, cysts, which are small, fluid-filled sacs, can form in the brain and press on nearby structures, especially the sellar region. These cysts can have different causes including:

• Rathke cleft cysts
• Arachnoid cysts
• Colloid cysts (usually found in the third ventricle, a fluid-filled space in the brain)

When these cysts press on surrounding tissue, they can cause symptoms similar to those of a craniopharyngioma.

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Diagnosis

A diagnosis of craniopharyngioma is based on clinical history and physical exam, laboratory testing and imaging.

Clinical history and physical exam

A diagnosis of craniopharyngioma requires an extensive clinical history and physical examination. Clinical manifestations that rise a suspicion of craniopharyngioma include a combination of headache, visual impairment, decreased growth rate, increased thirst and urination (polydipsia and polyuria) and other signs of hormonal (endocrine) deficiency.

Laboratory tests

Laboratory testing will usually be needed to confirm clinical suspicion of endocrine deficiency. Typically tests for craniopharyngioma include evaluation of serum electrolytes as well as all the hormones that can be affected by pituitary dysfunction: GH, IGF-1, TSH, free thyroxin, cortisol, FSH, LH, testosterone, estradiol and prolactin.

Imaging

Computed tomography (CT scan) and magnetic resonance imaging (MRI) are the imaging modalities of choice to diagnose craniopharyngiomas. CT scan is used to detect tumor calcification, while an MRI can be used to detect fluid accumulation inside cystic tumors. The diagnosis can be confirmed by analyzing the tumor under the microscope once it has been removed (resected).

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Standard Therapies

Treatment
Individuals with a craniopharyngioma might initially present with neurological, visual, and hormonal dysfunctions and might need to be evaluated by neurologists, ophthalmologists and endocrinologists.

Neurosurgical treatment is central to the management of craniopharyngioma. If there is no risk of damaging nearby anatomical structures, a neurosurgeon will remove (resect) the entirety of the tumor. If the tumor is in proximity to crucial structures, the surgical team might decide not to remove the entire tumor. Radiation oncologists will then be involved to administer radiotherapy to patients. There is no clear consensus on the balance that should be adopted between attempt at resecting the tumor and radiation therapy. Multiple surgical procedures might be required throughout a patient’s lifetime, as craniopharyngiomas tend to recur even when they seem to have been completely resected.

Endocrinologists will often be involved, as hormone replacement therapy is required to treat patients with hypothalamic and pituitary gland dysfunction. Hypothalamic obesity is also an issue that can significantly impact the quality of life of affected individuals. Medication, such as octreotide, that reduces insulin secretion, and bariatric surgery might be indicated for some patients suffering from morbid obesity. Due to the high rate of recurrence of craniopharyngioma and the numerous effects they can have on the body, affected individuals will usually have to be followed by numerous medical specialists throughout their life.

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Clinical Trials and Studies

Recent research has found targeted treatments, which are directed at the tumor cells without harming as much of the healthy brain.

For papillary craniopharyngioma (PCP):

• Treatments that block the BRAF gene variant (like dabrafenib or vemurafenib) and MEK pathway (like trametinib) have shown good results. In some people, tumors have shrunk over 90%, sometimes avoiding the need for surgery or radiation.

• For adamantinomatous craniopharyngioma (ACP): This type is more challenging to treat, but researchers are trying anti-inflammatory drugs (like tocilizumab and bevacizumab), interferon therapy to shrink cysts, medications that target tumor cell growth signals and immunotherapy (helping the body’s immune system recognize and fight the tumor).

These treatments are still being studied, but early results give hope, especially for people who have tumors that come back or are hard to remove with surgery.

Inflammation plays a big role in these tumors. Researchers think that calming this inflammation might help stop the tumors from growing or coming back. Some drugs being tested like anti-0IL-6R and anti-VEGF agents and immune checkpoints inhibitors, block inflammation-related proteins like IL-6, VEGF and PD-1/PD-L1, have been used in other types of cancer as immunotherapy. 

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]

Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/

For information about clinical trials sponsored by private sources, contact:
https://www.centerwatch.com/

For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

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References

JOURNAL ARTICLES
Pires de Oliveira Neto C, Nascimento GC, Damianse SDSP, Faria MDS. Recent advances in craniopharyngioma pathophysiology and emerging therapeutic approaches. Front Endocrinol (Lausanne). 2025;16:1562942. Published 2025 May 13. doi:10.3389/fendo.2025.1562942

Jacobsen MF, Thomsen ASS, Bach-Holm D, et al. Predictors of visual outcome in patients operated for craniopharyngioma – a Danish national study. Acta Ophthalmol. 2018;96:39-45.

Wijnen M, van den Heuvel-Eibrink MM, Janssen J, et al. Very long-term sequelae of craniopharyngioma. Eur J Endocrinol. 2017;176:755-67.

Chunhui L, Chuzhong L, Zhenye L, Yilin S, Yazhuo Z. Malignant Transformation of Radiotherapy-Naive Craniopharyngioma. World Neurosurg. 2016;88:690 e1-5.

Higham CE, Johannsson G, Shalet SM. Hypopituitarism. Lancet 2016;388:2403-15.

Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 2016;131:803-20.

Hoffmann A, Bootsveld K, Gebhardt U, Daubenbuchel AM, Sterkenburg AS, Muller HL. Nonalcoholic fatty liver disease and fatigue in long-term survivors of childhood-onset craniopharyngioma. Eur J Endocrinol. 2015;173:389-97.

Melmed S. Pituitary tumors. Endocrinol Metab. Clin North Am 2015;44:1-9.

Muller HL. Craniopharyngioma. Handb Clin Neurol. 2014;124:235-53.

Muller HL. Craniopharyngioma. Endocr Rev. 2014;35:513-43.

Lo AC, Howard AF, Nichol A, et al. Long-term outcomes and complications in patients with craniopharyngioma: the British Columbia Cancer Agency experience. Int J Radiat Oncol Biol Phys. 2014;88:1011-8.

Hussain I, Eloy JA, Carmel PW, Liu JK. Molecular oncogenesis of craniopharyngioma: current and future strategies for the development of targeted therapies. J Neurosurg. 2013;119:106-112.

Fernandez-Miranda JC, Gardner PA, Snyderman CH, et al. Craniopharyngioma: a pathologic, clinical, and surgical review. Head Neck 2012;34:1036-44.

Lustig RH. Hypothalamic obesity after craniopharyngioma: mechanisms, diagnosis, and treatment. Front Endocrinol (Lausanne) 2011;2:60.

Stephen MD, Zage PE, Waguespack SG. Gonadotropin-dependent precocious puberty: neoplastic causes and endocrine considerations. Int J Pediatr Endocrinol. 2011;2011:184502.

Muller HL. Childhood craniopharyngioma–current concepts in diagnosis, therapy and follow-up. Nat Rev Endocrinol. 2010;6:609-18.

Liu AK, Bagrosky B, Fenton LZ, et al. Vascular abnormalities in pediatric craniopharyngioma patients treated with radiation therapy. Pediatr Blood Cancer 2009;52:227-30.

Garre ML, Cama A. Craniopharyngioma: modern concepts in pathogenesis and treatment. Curr Opin Pediatr. 2007;19:471-9.

Karavitaki N, Cudlip S, Adams CB, Wass JA. Craniopharyngiomas. Endocr Rev. 2006;27:371-97.

Halac I, Zimmerman D. Endocrine manifestations of craniopharyngioma. Childs Nerv Syst. 2005;21:640-8.

Levy A. Pituitary disease: presentation, diagnosis, and management. J Neurol Neurosurg Psychiatry 2004;75 Suppl 3:iii47-52.

Rhoton AL, Jr. The sellar region. Neurosurgery 2002;51:S335-74.

Packer RJ, Cohen BH, Cooney K. Intracranial germ cell tumors. Oncologist 2000;5:312-20.

INTERNET
Ortiz Torres M, Shafiq I, Mesfin FB. Craniopharyngioma. [Updated 2023 Apr 24]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK459371/ Accessed June 23, 2025.

Ortega-Ruiz OR, Martinez-Sosa MM and Jallo GI. Craniopharyngioma. Medscape. Updated: Mar 21, 2024.  https://emedicine.medscape.com/article/1157758-overview Accessed June 23, 2025.

Harris BS and Price TM. Luteinizing Hormone Deficiency. Medscape. Last updated: Feb 08, 2022.  https://emedicine.medscape.com/article/255046-overview. Accessed June 23, 2025.

Kirkland L. Adrenal Crisis in Emergency Medicine. Medscape. Last updated: Mar 28, 2024.
https://emedicine.medscape.com/article/765753-overview Accessed June 23, 2025.

Marquez RRC. Hypopituitarism (Panhypopituitarism). Medscape. Last updated: Sep 10, 2024.
https://emedicine.medscape.com/article/122287-overview Accessed June 23, 2025.

Schraga ED. Hypothyroidism and Myxedema Coma. Medscape. Last updated: Apr 04, 2024.
https://emedicine.medscape.com/article/768053-overview Accessed June 23, 2025.

Liebeskind DS. Cerebral Aneurysm, Medscape. Last updated: Dec 16, 2024.
https://emedicine.medscape.com/article/1161518-overview Accessed June 23, 2025.

Su JM. Intracranial germ cell tumors. UpToDate. Last updated: May 23, 2025.
https://www.uptodate.com/contents/intracranial-germ-cell-tumors Accessed June 23, 2025.

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