Sarah E. Squire

Background: Intracranial neoplasms can cause pain similar to trigeminal neuralgia. Literature regarding radiosurgery for this is limited. We present a retrospective review of patients with tumor-related facial pain from benign lesions treated with gamma knife radiosurgery (GKRS) at Wake Forest University. Objectives: The primary objectives were to determine long-term pain relief and predictive factors for pain alleviation. Methods: We reviewed 515 patients treated with GKRS for benign meningioma, vestibular schwannoma or trigeminal schwannoma between August 1999 and August 2010. Twenty-one eligible patients had tumor-related facial pain prior to GKRS. The median marginal tumor dose was 12 Gy. Long-term pain relief data were obtained by chart review and telephone interview. Results: The median follow-up for symptom evaluation was 3.8 years. Seventeen of 21 patients (81%) experienced a Barrow Neurological Institute (BNI) score of I–III at 6 months following GKRS. Kaplan-Meier estimates of freedom from BNI IV–V relapse were 66% at 1 year and 53% at 2 years. No pain relapses occurred after 2 years. Conclusion: GKRS of benign lesions is a noninvasive option for patients with tumor-related facial pain. Pain relief is modest, with the majority of pain relapses occurring within 2 years and approximately one half of patients maintaining relief beyond 2 years.

To date, approximately 200 cases of atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system have been described in the literature. This CNS tumor tends to present at an age of less than 3 years, and most patients succumb to their disease within 1 year of diagnosis. Prior to the rise in utilization of immunohistochemical (IHC) testing in the late 1990s, this tumor was likely mistaken as medulloblastoma and treated as such. However, lessons learned from regimens based upon medulloblastoma have revealed that AT/RT requires more aggressive treatment. A significant portion of patients die of local recurrence in spite of aggressive surgery and chemotherapy. As most patients with AT/RT present as infants or young children, radiation therapy has been a less than standard treatment option. However, recent evidence suggests that long-term survival can occur with use of more aggressive treatment approaches including dose-intense chemotherapy as well as adjuvant radiation therapy. A standardized and effective approach to treating this usually fatal tumor remains elusive, and the role of radiation therapy presents a particular dilemma as young patients with this disease may experience devastating late effects of therapy if they achieve a long-term survival. Review of the literature reveals an association between initial radiation therapy and the ability to achieve a prolonged survival. Our review underscores the importance or enrolling patients in multi-institutional prospective studies to further investigate the value of radiation to treat this pediatric neoplasm.