What question did this study set out to answer?

To highlight the aggressive nature of recurrent adamantinomatous craniopharyngiomas with benign histology.

March 26, 20260 citations

Lethally aggressive multiply recurrent adamantinomatous craniopharyngioma without histological malignancy: illustrative cases.

HHHirokuni Hashikata MGMasanori Goto YNYasunori Nagai

Key Points

To highlight the aggressive nature of recurrent adamantinomatous craniopharyngiomas with benign histology.
Case illustrations of patients with multiply recurrent adamantinomatous craniopharyngioma.
Short-interval MRI surveillance employed for early detection.
Goal-directed interventions focused on cyst control and preservation of the hypothalamus.
High Ki-67 labeling index associated with rapid disease progression.
Standard treatment options often fail, necessitating consideration of clinical trials.
Emerging therapies like IL-6 blockade and EGFR-axis treatments showed promise.

Abstract

Multiply recurrent ACP with a high Ki-67 LI can follow a rapidly lethal course despite benign histology. Early recognition of this aggressive phenotype should prompt short-interval MRI surveillance and timely, goal-directed salvage focused on proactive cyst control, hypothalamus-sparing strategies, and carefully selected reirradiation. When standard options fail, enrollment in clinical trials of emerging biological therapies, such as IL-6 blockade, intracystic peginterferon-α2a, and EGFR-axis treatments, should be considered. https://thejns.org/doi/10.3171/CASE25840.

AI से पूछें

Bookmark

View Full Paper