Endocrine Abstracts

JOINT3943

New developments in targeted oncological treatment of craniopharyngioma provide innovative perspectives in the management of this challenging pathology. We report a clinical case demonstrating the fundamental role of pathological confirmation and molecular analyses for an optimal up to date treatment of craniopharyngioma. A 53-year-old male presented in August 2024vwith seven months history of visual failure and eventual visual deterioration. The ophthalmology assessment detected reduced visual acuity in both eyes, with OCT and visual field patterns in keeping with chiasmatic lesion. An MRI head demonstrated a suprasellar lesion involving the optic chiasm and the hypothalamus, and extending into the third ventricle, with features suggestive for an optic pathway/hypothalamic glioma. Hormonal testing demonstrated normal pituitary function with no diabetes insipidus. Initial steroid treatment with dexamethasone provided partial improvement of vision and interruption of deterioration. The case was reviewed by a Skull Base MDT and accordingly, the patient underwent a right sided pterional craniotomy in September 2024 and partial resection of suprasellar lesion with intraoperative neuro-monitoring including visual evoked potentials. The microsurgical procedure was recorded with a high-definition images, demonstrating the tumour appearances of a mixed density lesion and multiple fragments were collected for pathological analysis. The early postoperative visual function was overall stable compared to the vision prior to surgery. The integrated histopathology and molecular analyses confirmed a papillary craniopharyngioma WHO grade 1, B-RAF p.V600E mutated. The patient was initiated on a targeted monoclonal treatment combining vemurafenib and cobimetinib, BRAF/MEK inhibitors. The patient had an improvement in vision and the repeat MRI head from January 2025 demonstrated early response to treatment. In conclusion, the case presented demonstrate the relevance of the recent changes in the clinical management of suprasellar lesions, for which currently a pathological molecular confirmation is a cardinal step to guarantee the best oncological and functional outcomes.