Craniopharyngioma

Craniopharyngioma
Craniopharyngioma
Classification and external resources

Micrograph of a craniopharyngioma (adamantinomatous). HPS stain.
ICD-10 D44.4
ICD-9 237.0
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] It arises from nests of odontogenic (tooth-forming) epithelium within the suprasellar/diencephalic region and, therefore, contains deposits of calcium, which are evident on an x-ray. Histologically, craniopharyngiomas resemble adamantinomas (the most common tumors of the tooth). 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.

Contents

Presentation

CT scan showing a craniopharyngioma.

Craniopharyngiomas are typically very slow growing tumors. They arise from the cells along the pituitary stalk. They are classified by histology as benign;[4] however, as with many brain tumors, their treatment can be difficult, and significant morbidities are associated with both the tumor and treatment.

Craniopharyngioma is a rare, usually suprasellar[5] neoplasm, which may be cystic, that develops from rests of epithelium derived from Rathke's pouch.[6][7] Rathke's pouch is an embryonic precursor of the anterior pituitary.

Histology

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]

  • Adamantinomatous craniopharyngioma and,
  • Papillary craniopharyngioma.

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. In addition to a long list of possible symptoms, the most common presentation include: headaches, growth failure, and bitemporal hemianopsia.

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.

Prognosis

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

Treatment

Treatment generally consists of subfrontal or transsphenoidal excision. Adjuvant radiotherapy is also used if total removal is not possible. Due to the morbidities associated with damage to the pituitary and hypothalmus from surgical removal and radiation, experimental therapies using intracavitary P32, yttrium or bleomycin delivered via an external reservoir are frequently employed, especially in young patients.[10]

Possible symptoms

Additional images

  • Enhanced T1 weighted MRI's of craniopharyngiomas.

See also

  • Rathke's cleft cyst

References

  1. ^ "craniopharyngioma" at Dorland's Medical Dictionary
  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. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=1855047. 
  4. ^ 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. 
  5. ^ 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. 
  6. ^ Moore, Kraig D. (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 9780865778801. http://books.google.com/books?id=gbwSC43Eia4C&pg=PA409. Retrieved August 8, 2011. 
  7. ^ "Endocrine Pathology". http://library.med.utah.edu/WebPath/ENDOHTML/ENDO115.html. Retrieved 2009-05-08. 
  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. PMC 1850925. PMID 12466115. http://ajp.amjpathol.org/cgi/pmidlookup?view=long&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. http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0309-0167&date=2004&volume=45&issue=6&spage=573. 
  10. ^ 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

v · d · eNervous tissue tumors/NS neoplasm/Neuroectodermal tumor (ICD-O 9350–9589) (C70–C72, D32–D33, 191–192/225)
Endocrine/
sellar (9350–9379)
sellar: Craniopharyngioma · Pituicytoma
other: Pinealoma
CNS
(9380–9539)
Neuroepithelial
(brain tumors,
spinal tumors)
Astrocytoma (Pilocytic astrocytoma, Pleomorphic xanthoastrocytoma, Fibrillary (also diffuse or lowgrade) astrocytomas, Anaplastic astrocytoma, Glioblastoma multiforme)
Ependymoma · Subependymoma
Multiple/unknown
Mature
neuron
Primitive
Meningiomas
(meninges)
Hematopoietic
PNS: NST
(9540–9579)
note: not all brain tumors are of nervous tissue, and not all nervous tissue tumors are in the brain (see brain metastases)

M: CNS

anat(n/s/m/p/4/e/b/d/c/a/f/l/g)/phys/devp

noco(m/d/e/h/v/s)/cong/tumr, sysi/epon, injr

proc, drug(N1A/2AB/C/3/4/7A/B/C/D)

M: PNS

anat(h/r/t/c/b/l/s/a)/phys(r)/devp/prot/nttr/nttm/ntrp

noco/auto/cong/tumr, sysi/epon, injr

proc, drug(N1B)
v · d · eTumors: endocrine gland neoplasia (C73–C75/D34–D35, 193–194/226–227)
Pancreas/
islets of Langerhans
Hypothalamic/
pituitary axes
+parathyroid
Pituitary
Pituitary adenoma: Prolactinoma · ACTH-secreting pituitary adenoma · GH-secreting pituitary adenoma
Craniopharyngioma
Thyroid
Parathyroid
Gonads
see genital neoplasia
Pinealoma
Pinealoblastoma · Pineocytoma
MEN
1 · 2A · 2B

M: END

anat/phys/devp/horm

noco(d)/cong/tumr, sysi/epon

proc, drug (A10/H1/H2/H3/H5)

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Look at other dictionaries:

  • Craniopharyngioma — Klassifikation nach ICD 10 D44. Neubildung unsicheren oder unbekannten Verhaltens der endokrinen Drüsen D44.3 Hypophyse D44.4 …   Deutsch Wikipedia

  • craniopharyngioma — A suprasellar neoplasm, which may be cystic, that develops from the nests of epithelium derived from Rathke pouch; the histologic pattern, similar to that observed in adamantinomas, consists of nesting of squamous epithelium bordered by radially… …   Medical dictionary

  • craniopharyngioma — ▪ tumour       benign brain tumour arising from the pituitary gland. Although most common in children, it can occur at any age. As it grows, the tumour may compress the optic nerve and other nearby structures, causing loss of vision, headaches… …   Universalium

  • craniopharyngioma — cra·nio·pha·ryn·gi·oma …   English syllables

  • craniopharyngioma — n. a brain tumour, situated above the sella turcica, that is derived from remnants of Rathke s pouch, the earliest detectable embryonic precursor of the pituitary gland. The patient may show raised intracranial pressure and diabetes insipidus due …   The new mediacal dictionary

  • craniopharyngioma — A benign brain tumor that may be considered malignant because it can damage the hypothalamus, the area of the brain that controls body temperature, hunger, and thirst …   English dictionary of cancer terms

  • craniopharyngioma — …   Useful english dictionary

  • pituitary adamantinoma — craniopharyngioma …   Medical dictionary

  • pituitary ameloblastoma — craniopharyngioma …   Medical dictionary

  • suprasellar cyst — craniopharyngioma …   Medical dictionary

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