E-Poster Presentation ESA-SRB-ANZBMS 2021

A 27 year-old male overseas student presented to his GP with new headaches and blurred vision, developing over a two month period. Bilateral inferior quadrantanopia was identified and a 13.8 x 12.3 x 12.6mm solid/cystic suprasellar mass was seen on MRI inseparable from the optic chiasm. No endocrine dysfunction was present. Transcranial pituitary surgery successfully debulked the optic apparatus in a subtototal resection. Replacement thyroxine, hydrocortisone and desmopressin were required postoperatively and vision improved. Histopathology showed papillary craniopharyngioma positive for BRAF V600E with Ki-67 <2%. Attempts were made to secure a BRAF/MEK inhibitor to delay repeat intervention however the patient’s insurer was not forthcoming. An MRI at three months showed rapid tumour re-growth at 26.0 x 29.0 x 22.6mm with new cystic components compressing the optic apparatus and new extension into the third ventricle. The patient was reluctant to proceed to further surgery however this was initiated after an acute deterioration in vision. Gross total resection was achieved without complication via trans-sphenoidal approach and vision again stabilised.

This case exhibits an aggressive phenotype of craniopharyngioma with significant threat to vision. Given the patient’s young age attempts were made to treat with targeted BRAF/MEK which have shown promise in the treatment of BRAF V600E positive craniopharyngiomas¹ though this was not possible. This case highlights the need for close follow up with interval imaging and visual assessment in the case of subtotal resection.