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Childhood Craniopharyngioma Treatment (PDQ®)

Health Professional Version

Histopathologic Classification of Childhood Craniopharyngioma

Craniopharyngiomas are histologically benign and often occur in the suprasellar region, with an intrasellar portion. They may be locally invasive and typically do not metastasize to remote brain locations; however, craniopharyngiomas may recur after initial therapy.

Craniopharyngiomas are classified as one of the following:

  • Adamantinomatous: Adamantinomatous craniopharyngioma is the most frequent type in children.[1] These tumors are typically composed of a solid portion formed by nests and trabeculae of epithelial tumor cells, with an abundance of calcification, and a cystic component that is filled with a dark, oily fluid. Wet keratin is also characteristic. Adamantinomatous craniopharyngiomas are more locally aggressive than are papillary tumors and have a significantly higher rate of recurrence.[2] Activating beta-catenin gene mutations are found in virtually all adamantinomatous tumors.[3,4]
  • Papillary: BRAF V600E mutations are observed in nearly all papillary craniopharyngiomas.[4] Papillary craniopharyngiomas occur primarily in adults.

References

  1. Karavitaki N, Wass JA: Craniopharyngiomas. Endocrinol Metab Clin North Am 37 (1): 173-93, ix-x, 2008. [PUBMED Abstract]
  2. Pekmezci M, Louie J, Gupta N, et al.: Clinicopathological characteristics of adamantinomatous and papillary craniopharyngiomas: University of California, San Francisco experience 1985-2005. Neurosurgery 67 (5): 1341-9; discussion 1349, 2010. [PUBMED Abstract]
  3. Sekine S, Shibata T, Kokubu A, et al.: Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations. Am J Pathol 161 (6): 1997-2001, 2002. [PUBMED Abstract]
  4. Brastianos PK, Taylor-Weiner A, Manley PE, et al.: Exome sequencing identifies BRAF mutations in papillary craniopharyngiomas. Nat Genet 46 (2): 161-5, 2014. [PUBMED Abstract]
  • Updated: January 15, 2015