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

BACKGROUND

Intracranial 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.