Recurrent intracranial myoepithelioma: clinical course and long-term follow-up. Illustrative case.

backgroundIntracranial myoepithelial carcinomas are exceedingly rare tumors associated with a poor prognosis. A review of the literature revealed only 6 published cases, none of which include follow-up longer than 2 years. OBSERVATIONS: A 26-year-old female presented with headaches, fatigue, and vision changes. She was found to have a rare intracranial myoepithelioma in the parafalcine region. Over the next 7 years, she experienced multiple recurrences managed with surgery and radiation therapy. The patient eventually became pregnant, and a new lesion was identified in her first trimester. Following close observation and a cesarean section, the rapidly growing lesion wasresected, and consistent with prior myoepithelioma. LESSONS: The authors present the seventh case of primary, intracranial malignant myoepithelioma and the only such case with follow-up longer than 2.5 years. With aggressive management, patients navigating intracranial myoepithelial carcinoma can experience robust survival with conserved quality of life. https://thejns.org/doi/10.3171/CASE25136.