Malignant transformation of vestibular schwannoma after radiation therapy

Stereotactic radiosurgery (SRS) is an effective treatment for vestibular schwannomas, offering high rates of tumor control and low neurological risks. Long-term complications of SRS are not fully understood, with several cases of malignant transformation reported in the literature. We report the case of a 50-year-old female with no prior history of neurofibromatosis who presented in 2013 with MRI evidence of a benign vestibular schwannoma. Despite treatment with CyberKnife SRS, she presented 6 years later with new onset neurologic symptoms. Further investigation showed stable lesion size with increasing vasogenic edema and a new area of enhancement in the brainstem, suspicious for malignant transformation. Subsequent treatment with partial craniectomy and histopathologic analysis was consistent with a malignant peripheral nerve sheath tumor diagnosis. Our case adds to a series of 24 similar cases in the literature, details of which have been summarized in our study. Overall, findings support the need for lifelong surveillance following SRS treatment of benign vestibular schwannomas. Patients should be educated on the potential risk of this complication, and clinicians must maintain a high level of suspicion for potential radiation-induced malignancy during the patient's clinical course.