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Lethally aggressive multiply recurrent adamantinomatous craniopharyngioma without histological malignancy:
Hirokuni Hashikata1, Masanori Goto1, Yasunori Nagai1
1Department of Neurosurgery, Medical Research Institute Kitano Hospital, Osaka, Osaka, Japan.
Background:
Mortality from craniopharyngioma (CP) is rare and typically results from surgical or endocrine-metabolic complications rather than tumor growth. Rapidly lethal regrowth without malignant transformation is uncommon and poorly characterized.
Observations:
The authors report on the cases of 2 patients with multiply recurrent adamantinomatous CP (ACP)-one childhood-onset case with a late-adolescent lethal recurrence and one adult-onset case that developed rapid, treatment-refractory regrowth within months despite multiple resections, radiosurgery, and cytotoxic chemotherapy, culminating in death. Neither tumor showed histological malignant transformation. Both tumors had a Ki-67 labeling index (LI) exceeding 10% with Ki-67-positive epithelial clusters. Imaging and intraoperative findings showed firm adherence to the hypothalamus and third ventricle with a cyst-predominant pattern, suggesting that residual adherent cyst-wall epithelium may contribute to aggressive relapse.
Lessons:
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.
