The spectrum of malignancy in craniopharyngioma

Fausto J. Rodriguez, Bernd W. Scheithauer, Shigeru Tsunoda, Kalman Kovacs, Sergio Vidal, David G. Piepgras

Research output: Contribution to journalArticlepeer-review

Abstract

Craniopharyngiomas are low-grade epithelial neoplasms occurring almost exclusively in the sellar/suprasellar region. Histologic malignancy is extremely rare; the literature consists mostly of isolated case reports. Herein, we report 3 patients with craniopharyngiomas exhibiting histologic malignancy, 2 of which received radiation therapy before its appearance. Hematoxylin and eosin-stained slides and selected immunohistochemical stains were reviewed in all cases. Microvessel density analysis was performed in case 2. The patients included 2 men and 1 woman, age 14, 31, and 58 years at presentation, respectively. All patients expired 3 months to 9 years after first resection and 3 to 9 months after identification of histologic malignancy. The latter developed after multiple recurrences and radiation therapy in 2 cases, but seemed to arise de novo in 1 case resembling odontogenic ghost cell carcinoma and lacking any definite low-grade craniopharyngioma precursor. The malignant component of the other 2 cases resembled squamous cell carcinoma and low-grade myoepithelial carcinoma, respectively. The MIB-1 labeling index was markedly increased in the malignant component in comparison with the low-grade precursor. Malignant transformation in craniopharyngiomas, although rare, does exist. It assumes varied histologic appearances, usually after multiple recurrences and radiation therapy, and has a near uniformly fatal outcome. De novo malignancy in odontogenic tumors of the sella is even more unusual, but also has an ominous prognosis.

Original languageEnglish (US)
Pages (from-to)1020-1028
Number of pages9
JournalAmerican Journal of Surgical Pathology
Volume31
Issue number7
DOIs
StatePublished - Jul 1 2007
Externally publishedYes

Keywords

  • Craniopharyngioma
  • Ghost cell carcinoma
  • Malignant transformation
  • Myoepithelioma
  • Odontogenic
  • Squamous cell carcinoma
  • Suprasellar

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

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