Impact of endoscopy and related technologies on indications for transsphenoidal pituitary surgery for growth hormone-producing pituitary neuroendocrine tumors in McCune-Albright syndrome: illustrative case.

backgroundMcCune-Albright syndrome (MAS) is frequently associated with growth hormone (GH)-producing pituitary neuroendocrine tumors. However, transsphenoidal surgery is considered difficult because of skull base fibrous dysplasia, making complete tumor removal and endocrine remission challenging. OBSERVATIONS: Eighteen years earlier, a 31-year-old woman was diagnosed with MAS based on findings of GH hypersecretion, a -café--au-lait spot on the back of her right shoulder, and bone lesions in the ribs and craniofacial bones. She had a pituitary tumor, but craniofacial fibrous dysplasia involving the sphenoid bone contraindicated surgical removal of the GH-producing pituitary tumor. Medical treatment was prioritized, and she underwent triple-drug therapy (octreotide, cabergoline, and pegvisomant) for 18 years. Insulin-like growth factor-1 remained poorly controlled. Recent advances in endonasal endoscopic surgery prompted reconsideration of surgical treatment. Endoscopic transnasal pituitary tumor resection using neuronavigation and a high-speed drill was performed, and achieved complete tumor resection, and all medical therapy became unnecessary. LESSONS: Surgical treatment was previously considered difficult in cases of GH-producing pituitary tumor associated with MAS. However, recent advances in transnasal endoscopic surgery, including drilling and neuronavigation techniques, have made surgical endocrine remission achievable in some cases. Therefore, surgical indications should be reconsidered even in patients undergoing medical therapy. https://thejns.org/doi/10.3171/CASE26521.