World Library  
Flag as Inappropriate
Email this Article


Article Id: WHEBN0003085444
Reproduction Date:

Title: Craniopharyngioma  
Author: World Heritage Encyclopedia
Language: English
Subject: WHO classification of the tumors of the central nervous system, History of neuroimaging, Anaplastic astrocytoma, Ganglioglioma, Neurosurgery
Publisher: World Heritage Encyclopedia


Micrograph of a craniopharyngioma (adamantinomatous). HPS stain.
Classification and external resources
ICD-10 D44.4
ICD-9-CM 237.0
ICD-O 9350/1
DiseasesDB 3153
MedlinePlus 000345
eMedicine radio/196
MeSH D003397

Craniopharyngioma is a type of brain tumor derived from pituitary gland embryonic tissue,[1] that occurs most commonly in children but also in men and women in their 50s and 60s.[2] People may present with bitemporal inferior quadrantanopia leading to bitemporal hemianopia, as the tumor may compress the optic chiasm.

It has a point prevalence of approximately 2/100,000.[3]

Craniopharyngiomas are also known as Rathke pouch tumors, hypophyseal duct tumors, or adamantinomas.

Signs and symptoms

Craniopharyngioma is a rare, usually suprasellar[4] neoplasm, which may be cystic, that develops from nests of epithelium derived from Rathke's pouch.[5][6] Rathke's pouch is an embryonic precursor of the anterior pituitary.

Craniopharyngiomas are typically very slow growing tumors. They arise from the cells along the pituitary stalk, specifically from nests of odontogenic (tooth-forming) epithelium within the suprasellar/diencephalic region and, therefore, contain deposits of calcium, which are evident on an x-ray. They are classified by histology as benign;[7] however, as with many brain tumors, their treatment can be difficult, and significant morbidities are associated with both the tumor and treatment.


The histologic pattern consists of nesting of squamous epithelium bordered by radially arranged cells. It is frequently accompanied by calcium deposition and may have a microscopic papillary architecture.

Two distinct types are recognized:[8][9]

  • Adamantinomatous craniopharyngiomas, which resemble adamantinomas, the most common type of tooth tumor, are characterized by activating CTNNB1 mutations; and,
  • Papillary craniopharyngiomas are characterized by BRAFv600E mutations.[10]

In the adamantinomatous type, calcifications are visible on neuroimaging and are helpful in diagnosis. The papillary type rarely calcifies.

On macroscopic examination, craniopharyngiomas are cystic or partially cystic with solid areas. On light microscopy, the cysts are seen to be lined by stratified squamous epithelium. Keratin pearls may also be seen. The cysts are usually filled with a yellow, viscous fluid which is rich in cholesterol crystals. Of a long list of possible symptoms, the most common presentations include: headaches, growth failure, and bitemporal hemianopsia.


Craniopharyngiomas are generally benign but are known to recur after resection. Recent research[11] has demonstrated a malignant (but rare) tendency of craniopharyngiomas. These malignant craniopharyngiomas are very rare, but are associated with poor prognosis.

Malignant craniopharyngioma

Craniopharyngiomas are usually successfully managed with a combination of adjuvant chemotherapy and neurosurgery. Recent research describes the rare occurrence of malignant transformations of these normally benign tumors. Malignant craniopharyngiomas can occur at any age, are slightly more common in females, and are usually of the adamantinomatous type.[11]

The malignant transformations can take years to occur (although 1 in 5 of the diagnosed cases were de nove transformations), hence the need for lengthier follow up in patients diagnosed with the more common benign forms.[11]

There was no link found between malignancy and initial chemo-radiotherapy treatment,[11] and the overall survival rate was very poor with median survival being 6 months post diagnosis of malignancy.


Treatment generally consists of subfrontal or transsphenoidal excision. Surgery using the transsphenoidal route is often performed by a joint team of ENT and neurosurgeons. Because of the location of the craniopharyngioma near the brain and skullbase, a surgical navigation system might be used to verify the position of surgical tools during the operation.[12]

Additional radiotherapy is also used if total removal is not possible. Due to the poor outcomes associated with damage to the pituitary and hypothalamus from surgical removal and radiation, experimental therapies using intracavitary phosphorus-32, yttrium, or bleomycin delivered via an external reservoir are sometimes employed, especially in young patients. The tumor, being in the pituitary gland, can cause secondary health problems. The immune system, thyroid levels, growth hormone levels and testosterone levels can be compromised from craniopharygioma. All of the before mentioned health problems can be treated with modern medicine.[13] There is no high quality evidence looking at the use of bleomycin in this condition.[14]

The most effective treatment 'package' for the malignant craniopharyngiomas described in literature is a combination 'gross total resective' surgery with adjuvant chemo radiotherapy. The chemotherapy drugs Paclitaxel and Carboplatin have shown a clinical (but not statistical) significance in increasing the survival rate in patients who've had gross total resections of their malignant tumours.[11]

See also


  1. ^ "craniopharyngioma" at Dorland's Medical Dictionary
  2. ^
  3. ^
  4. ^
  5. ^
  6. ^
  7. ^
  8. ^
  9. ^
  10. ^
  11. ^ a b c d e
  12. ^ Use of surgical navigation for craniopharyngioma removal
  13. ^
  14. ^

External links

  • Cancer.Net: Craniopharyngioma - Childhood
  • Boston Neurosurgical Foundation
  • Adamantinomatous Craniopharyngioma
  • Malignant transformation in craniopharyngiomas
This article was sourced from Creative Commons Attribution-ShareAlike License; additional terms may apply. World Heritage Encyclopedia content is assembled from numerous content providers, Open Access Publishing, and in compliance with The Fair Access to Science and Technology Research Act (FASTR), Wikimedia Foundation, Inc., Public Library of Science, The Encyclopedia of Life, Open Book Publishers (OBP), PubMed, U.S. National Library of Medicine, National Center for Biotechnology Information, U.S. National Library of Medicine, National Institutes of Health (NIH), U.S. Department of Health & Human Services, and, which sources content from all federal, state, local, tribal, and territorial government publication portals (.gov, .mil, .edu). Funding for and content contributors is made possible from the U.S. Congress, E-Government Act of 2002.
Crowd sourced content that is contributed to World Heritage Encyclopedia is peer reviewed and edited by our editorial staff to ensure quality scholarly research articles.
By using this site, you agree to the Terms of Use and Privacy Policy. World Heritage Encyclopedia™ is a registered trademark of the World Public Library Association, a non-profit organization.

Copyright © World Library Foundation. All rights reserved. eBooks from Project Gutenberg are sponsored by the World Library Foundation,
a 501c(4) Member's Support Non-Profit Organization, and is NOT affiliated with any governmental agency or department.