Craniopharyngiomas are histologically and cytologically benign epithelial t
umors of the central nervous system that may be locally aggressive and tend
to recur after excision. Malignant change in craniopharyngiomas is extreme
ly rare; we found only 4 such reports in the literature. In this report, we
describe a case of squamous cell carcinoma arising in a previously benign
craniopharyngioma in a 42-year-old woman. The patient was diagnosed with cr
aniopharyngioma in 1982; during the subsequent 15 years she experienced 7 t
umor recurrences, for which surgical resections and 3 courses of radiothera
py were performed. In 1998, the tumor recurred with involvement of the nasa
l cavity and sphenoid and ethmoid sinuses. Histologic evaluation revealed f
oci of typical adamantinomatous craniopharyngioma associated with a moderat
ely differentiated squamous cell carcinoma. The transition of typical crani
opharyngioma to squamous cell carcinoma was well demonstrated, suggesting t
hat carcinoma arose from the underlying craniopharyngioma. Radiation may ha
ve been a contributing factor to carcinogenesis in this case.