Department of Pathology, Catholic University School of Medicine, Largo A. Gemelli, 8, 00168, Rome, Italy.
J Neurooncol. 2011 Jun;103(2):381-6. doi: 10.1007/s11060-010-0382-7. Epub 2010 Sep 3.
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.
恶性或间变性颅咽管瘤，由 Akachi 及其同事于 1987 年首次描述，是一种罕见的颅咽管瘤，其特征为细胞学异型性和预后不良。此前已有 15 例报道，其中 2 例为新发病例，即不是由先前治疗的良性颅咽管瘤发展而来；这两例患者均在术后早期死亡。在此，我们描述了一位 66 岁女性患者的病例，她因视力障碍和鞍上及鞍旁肿瘤的影像学证据就诊。患者接受了经蝶窦活检，随后行翼点开颅术，部分切除肿瘤。组织学诊断为恶性造釉细胞瘤型颅咽管瘤。患者接受了辅助放疗，肿瘤明显缩小。她在诊断后 10 个月仍处于良好的临床状态；由于肿瘤进展，她在诊断后 15 个月恶化并死亡。恶性颅咽管瘤是鞍区罕见的原发性恶性肿瘤。这是首例具有显著随访结果的新发病例。
