Craniopharyngioma

From Wikipedia, the free encyclopedia - View original article

Craniopharyngioma
Classification and external resources
Adamantinomatous craniopharyngioma - very low mag.jpg
Micrograph of a craniopharyngioma (adamantinomatous). HPS stain.
ICD-10D44.4
ICD-9237.0
ICD-O:9350/1
DiseasesDB3153
MedlinePlus000345
eMedicineradio/196
MeSHD003397
 
Jump to: navigation, search
Craniopharyngioma
Classification and external resources
Adamantinomatous craniopharyngioma - very low mag.jpg
Micrograph of a craniopharyngioma (adamantinomatous). HPS stain.
ICD-10D44.4
ICD-9237.0
ICD-O:9350/1
DiseasesDB3153
MedlinePlus000345
eMedicineradio/196
MeSHD003397

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] Patients 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.

Presentation[edit]

CT scan showing a craniopharyngioma.

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.

Histology[edit]

Micrograph showing the characteristic features of an adamantinomatous craniopharyngioma - cystic spaces, calcifications, and "wet" keratin. HPS stain.
Micrograph showing a papillary craniopharyngioma. HPS stain.

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]

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.

Prognosis[edit]

Craniopharyngiomas are generally benign but are known to recur after resection.

Treatment[edit]

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.[11] Adjuvant radiotherapy is also used if total removal is not possible. Due to the morbidities 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 frequently 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.[12]

Possible symptoms[edit]

Additional images[edit]

See also[edit]

References[edit]

  1. ^ "craniopharyngioma" at Dorland's Medical Dictionary[dead link]
  2. ^ Hamid R, Sarkar S, Hossain MA, Mazumder U, Akanda NI, Parvin R (2007). "Clinical picture of craniopharyngioma in childhood". Mymensingh medical journal : MMJ 16 (2): 123–6. PMID 17703145. 
  3. ^ Garnett MR, Puget S, Grill J, Sainte-Rose C (2007). "Craniopharyngioma". Orphanet journal of rare diseases 2: 18. doi:10.1186/1750-1172-2-18. PMC 1855047. PMID 17425791. 
  4. ^ Rodriguez FJ, Scheithauer BW, Tsunoda S, Kovacs K, Vidal S, Piepgras DG (2007). "The spectrum of malignancy in craniopharyngioma". Am. J. Surg. Pathol. 31 (7): 1020–8. doi:10.1097/PAS.0b013e31802d8a96. PMID 17592268. 
  5. ^ Moore, Kraig D.; Couldwell, William T. (January 15, 2000). "41. Craniopharyngioma". In Bernstein, Mark; Berger, Mitchel S. Neuro-oncology: the essentials. Endorsed by the Joint Tumor Section of the American Association of Neurological Surgeons & the Congress of Neurological Surgeons. Thieme. pp. 409–418. ISBN 978-0-86577-880-1. Retrieved August 8, 2011. 
  6. ^ "Endocrine Pathology". Retrieved 2009-05-08. 
  7. ^ Garrè ML, Cama A (2007). "Craniopharyngioma: modern concepts in pathogenesis and treatment". Curr. Opin. Pediatr. 19 (4): 471–9. doi:10.1097/MOP.0b013e3282495a22. PMID 17630614. 
  8. ^ Sekine S, Shibata T, Kokubu A, et al (December 2002). "Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations". Am. J. Pathol. 161 (6): 1997–2001. doi:10.1016/s0002-9440(10)64477-x. PMC 1850925. PMID 12466115. 
  9. ^ Sekine S, Takata T, Shibata T, et al (December 2004). "Expression of enamel proteins and LEF1 in adamantinomatous craniopharyngioma: evidence for its odontogenic epithelial differentiation". Histopathology 45 (6): 573–9. doi:10.1111/j.1365-2559.2004.02029.x. PMID 15569047. 
  10. ^ http://www.nature.com/ng/journal/v46/n2/full/ng.2868.html.  Missing or empty |title= (help)
  11. ^ Use of surgical navigation for craniopharyngioma removal[dead link]
  12. ^ Wisoff JH (February 2008). "Craniopharyngioma". J Neurosurg Pediatr 1 (2): 124–5; discussion 125. doi:10.3171/PED/2008/1/2/124. PMID 18352780. 

External links[edit]