De novo malignant craniopharyngioma: case report and literature review.
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 10 months; she deteriorated and died, due to tumor progression, 15 months 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.
Lauriola
Libero
L
Department of Pathology, Catholic University School of Medicine, Largo A. Gemelli, 8, 00168, Rome, Italy.
Doglietto
Francesco
F
Novello
Mariangela
M
Signorelli
Francesco
F
Montano
Nicola
N
Pallini
Roberto
R
Maira
Giulio
G
D000368:Aged; D003131:Combined Modality Therapy; D003397:Craniopharyngioma / Q000473:pathology* / Q000628:therapy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D019635:Neurosurgical Procedures; D010911:Pituitary Neoplasms / Q000473:pathology* / Q000628:therapy; D011878:Radiotherapy
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