Malignant Transformation of Craniopharyngioma: A rare case after 32 years of follow-up.

Craniopharyngiomas are rare, benign epithelial tumors of the sellar region, with malignant transformation being exceedingly uncommon. We report a case of squamous cell carcinoma arising 32 years after the initial diagnosis of adamantinomatous craniopharyngioma. The patient underwent four surgical resections and two courses of radiotherapy. Following seven years of stable imaging, a rapidly enlarging lesion emerged, invading adjacent skull base structures. Histopathological examination confirmed malignant transformation, characterized by squamous cell carcinoma with beta-catenin positivity. This case highlights the diagnostic and therapeutic challenges posed by such transformation and underscores the importance of long-term follow-up in craniopharyngioma patients. Although radiation therapy has been speculated as a risk factor, its causal relationship remains unproven. Malignant craniopharyngiomas carry a poor prognosis, with limited benefit from conventional therapies. Increased awareness and documentation of such rare cases are essential to improve understanding of underlying mechanisms and guide future management strategies.