Late development of craniopharyngioma following surgery for Rathke's cleft cyst.
OBJECTIVE
Rathke's cleft cyst (RCC) may transform to papillary type craniopharyngioma (CP) after squamous metaplasia: this is referred to as ciliated CP. We encountered a case involving a patient who had late development of adamantinomatous CP following surgery for RCC, the details of which may shed light on the histogenesis of CP in general.


METHODS
A 41-year-old man presented to our institution with visual disturbance, and magnetic resonance imaging (MRI) showed a cystic mass in the suprasellar region. The patient underwent a biopsy via a transsphenoidal approach and was diagnosed as having a RCC. 34 months after the initial surgery, the patient revisited our hospital for a rapidly aggravating visual disturbance and underwent neuroendoscopic biopsy and tumor removal via a bifrontal craniotomy. Histologically, the tumor was shown to be an adamantinomatous CP. No nuclear beta-catenin accumulation was detectable in the previous RCC specimen, but nuclear beta-catenin accumulation was found in the recent CP specimen, restricted to whorl-like structures or surrounding ghost cells.


CONCLUSIONS
Our case of adamantinomatous CP that developed long after removal of the RCC, diagnosed by beta-catenin staining, supports the hypothesis that CPs may develop from RCCs directly due to beta-catenin mutations. However, it still does not prove that a histogenetic connection can be shown between the two lesions which are clonally unrelated. Our case is reported as two consecutive lesions; this in itself is a rare situation.
Park
Y S
YS
Department of Neurosurgery, Brain Research Institute, Seoul, Republic of Korea.
Ahn
J Y
JY
Kim
D S
DS
Kim
T S
TS
Kim
S H
SH
D000328:Adult; D020863:Central Nervous System Cysts / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D003397:Craniopharyngioma / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D016609:Neoplasms, Second Primary / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D019635:Neurosurgical Procedures; D010911:Pituitary Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D051176:beta Catenin / Q000378:metabolism
D051176:beta Catenin