Extended Transsphenoidal Approach for Gross Total Resection of Hypothalamic Tumor

Tumors of the optic pathway and/or hypothalamus are uncommon, and the majority occur in patients with neurofibromatosis type 1.1,2 These lesions can be managed with a "watch-and-wait" approach; however, when treatment is indicated, the optimal strategy is wholly case dependent. We present a 22year-old female patient with neurofibromatosis type 1 who had a partially cystic mass within the right mamillary body (Video 1). The mass was present on prior imaging but had increased in size and was newly enhancing with evidence of mass effect on the right optic tract. Given her history and the lesion's progression, treatment was recommended. The patient elected for surgery over radiation. Preoperatively, there were no visual field deficits or signs of hypopituitarism. We undertook a unilateral, extended transsphenoidal approach. Additional bony removal of the tuberculum sellae and planum sphenoidale enabled greater exposure of the anterior cranial fossa. The circular sinus was cauterized and divided for visualization of the pituitary stalk and clear identification of the hypothalamus. This provided an operative corridor superior to the pituitary gland and just beneath the optic nerves. The procedure proceeded without complication, and gross total resection was achieved. Postoperative imaging demonstrated gross total resection with a normal-appearing pituitary gland. Of note, the patient did experience mildly elevated postoperative sodium and was treated with desmopressin for transient diabetes insipidus. Twelve- and 24-month follow-up imaging showed no recurrence. The extended transsphenoidal approach used here offers a safe and suitable working corridor to achieve total resection of hypothalamic lesions without injury to the pituitary gland.