De novo malignant craniopharyngioma: case report and literature review

Abstract

Malignant or anaplastic craniopharyngioma, first described in 1987 by Akachi and coworkers, is a rare occurring craniopharyngioma characterized by cytologic atypia and poor prognosis. Fifteen cases have been previously reported, two of which have been defined de novo, i.e. not developing from a previously treated benign craniopharyngioma; both these patients died in the early post-operative period. Herein we describe the case of a 66-year-old female who presented with visual disturbance and radiological evidence of a sellar and suprasellar tumor. The patient underwent trans-sphenoidal biopsy followed by pterional craniotomy with partial tumor removal. Histological diagnosis documented a malignant adamantinomatous type craniopharyngioma. The patient received adjuvant radiotherapy with a significant tumor reduction. She remained in good clinical conditions for 10months; she deteriorated and died, due to tumor progression, 15months after diagnosis. Malignant craniopharyngioma is a rare primary malignant tumor of the sellar region. This is the first case of de novo malignant craniopharyngioma with significant follow-up.