Craniopharyngioma
Summary
Craniopharyngioma is a slow-growing benign tumor that develops near the pituitary gland (a small endocrine gland at the base of the brain) and the hypothalamus (a small cone-shaped organ connected to the pituitary gland by nerves).[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 benign (not cancerous), they may grow and press on nearby parts of the brain, causing symptoms including vision changes, slow growth, headaches, nausea and vomiting, loss of balance, and hearing loss.[2] 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).[2][4] Treatment for craniopharyngioma varies and may include: surgery to remove the tumor, radiation therapy, chemotherapy, and biologic therapy.[2]
Last updated: 8/4/2016
Symptoms
Craniopharyngioma causes symptoms in three different ways:[1]
- by increasing the pressure on the brain (intracranial pressure)
- by disrupting the function of the pituitary gland
- by damaging the optic nerve
Increased pressure on the brain causes headache, nausea, vomiting (especially in the morning), and difficulty with balance. Damage to the pituitary gland causes hormone imbalances that can lead to excessive thirst and urination and stunted growth. When the optic nerve is damaged by the tumor, vision problems develop. These symptoms are often permanent, and may be worse after surgery to remove the tumor. Most patients have at least some visual defects and evidence of decreased hormone production at the time of diagnosis. Behavioral and learning problems may also be present.[1]
Last updated: 8/5/2016
Cause
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, which are developmental structures related to gut (stomach) formation. 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][5]
Last updated: 8/5/2016
Treatment
There are several different types of treatment for craniopharyngiomas which may include a combination of the following: surgery to remove the full or partial tumor (resection), radiation therapy, cyst drainage, chemotherapy, and biologic therapy. Individuals with craniopharyngioma are recommended to have their treatment planned by a team of health care providers who are experts in treating brain tumors in children. This may include the following specialists: pediatrician, neurosurgeon, radiation oncologist, neurologist, ophthalmologist, rehabilitation specialist, social worker, and nurse specialist.[2]
Last updated: 8/5/2016
Prognosis
In general, the prognosis for patients with craniopharyngioma is good, with an 80-90% chance of permanent cure if the tumor can be completely removed with surgery or treated with high doses of radiation. However, the prognosis for an individual patient depends on several factors, including the ability of the tumor to be completely removed, and the neurological deficits and hormonal imbalances caused by the tumor and the treatment. Most of the problems with hormones and vision do not improve with treatment, and sometimes the treatment may even make them worse.[1]
Last updated: 8/5/2016
Research
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 studying or have studied Craniopharyngioma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
Listen
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.
GARD Answers
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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
References
Blackman S. Craniopharyngioma. MedlinePlus. 7/30/2014; http://www.nlm.nih.gov/medlineplus/ency/article/000345.htm.
General Information About Childhood Craniopharyngioma. National Cancer Institute (NCI). June 10, 2016; http://www.cancer.gov/types/brain/patient/child-cranio-treatment-pdq.
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. Accessed 8/4/2016.
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.
Craniopharyngioma. Pituitary Network Association. 2013; https://pituitary.org/knowledge-base/disorders/craniopharyngioma. Accessed 8/5/2016.