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Lawrence S. Chin, Nicholas J. Szerlip and William F. Regine

Meningiomas are benign tumors attached to the dura that typically have a slow growth rate. After gliomas, they are the most common primary tumor of the brain. They are ideal radiobiological targets because single-fraction radiation has a high biologically effective dose. Furthermore, a highly conformal radiation plan can provide effective treatment to the tumor while sparing the surrounding brain. Meningioma control rates range from 90 to 95%, and the risk of morbidity is low. Radiosurgery is an excellent treatment for asymptomatic, small- to moderate-sized meningiomas. It is also ideal for patients with incompletely resected meningiomas, recurrent meningiomas, and risk factors precluding conventional surgery.

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Cerebellar liponeurocytoma

Case report and review of the literature

Thad R. Jackson, William F. Regine, Dianne Wilson and Daron G. Davis

✓ Cerebellar liponeurocytoma is a rare tumor of the posterior fossa that has many morphological similarities to medulloblastoma and neurocytoma. Recently the World Health Organization working group for classification of central nervous system neoplasms adopted the term “cerebellar liponeurocytoma” to provide a unified nomenclature for a tumor variously labeled in the literature as lipomatous medulloblastoma, lipidized medulloblastoma, medullocytoma, neurolipocytoma, lipomatous glioneurocytoma, and lipidized mature neuroectodermal tumor of the cerebellum. The rarity of this tumor and paucity of pertinent information regarding its biological potential and natural history have resulted in the application of various treatment modalities. It is suggested in the available literature that these lesions have a much more favorable prognosis than typical medulloblastomas, and that adjuvant therapy for liponeurocytoma need not be as extensive as that administered for medulloblastomas.

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William F. Regine, Roy A. Patchell, James M. Strottmann, Ali Meigooni, Michael Sanders and Byron Young

Object. This investigation was performed to determine the tolerance and toxicities of split-course fractionated gamma knife radiosurgery (FSRS) given in combination with conventional external-beam radiation therapy (CEBRT).

Methods. Eighteen patients with previously unirradiated, gliomas treated between March 1995 and January 2000 form the substrate of this report. These included 11 patients with malignant gliomas, six with low-grade gliomas, and one with a recurrent glioma. They were stratified into three groups according to tumor volume (TV). Fifteen were treated using the initial FSRS dose schedule and form the subject of this report. Group A (four patients), had TV of 5 cm3 or less (7 Gy twice pre- and twice post-CEBRT); Group B (six patients), TV greater than 5 cm3 but less than or equal to 15 cm3 (7 Gy twice pre-CEBRT and once post-CEBRT); and Group C (five patients), TV greater than 15 cm3 but less than or equal to 30 cm3 (7 Gy once pre- and once post-CEBRT). All patients received CEBRT to 59.4 Gy in 1.8-Gy fractions. Dose escalation was planned, provided the level of toxicity was acceptable. All patients were able to complete CEBRT without interruption or experiencing disease progression. Unacceptable toxicity was observed in two Grade 4/Group B patients and two Grade 4/Group C patients. Eight patients required reoperation. In three (38%) there was necrosis without evidence of tumor. Neuroimaging studies were available for evaluation in 14 patients. Two had a partial (≥ 50%) reduction in volume and nine had a minor (> 20%) reduction in size. The median follow-up period was 15 months (range 9–60 months). Six patients remained alive for 3 to 60 months.

Conclusions. The imaging responses and the ability of these patients with intracranial gliomas to complete therapy without interruption or experiencing disease progression is encouraging. Excessive toxicity derived from combined FSRS and CEBRT treatment, as evaluated thus far in this study, was seen in patients with Group B and C lesions at the 7-Gy dose level. Evaluation of this novel treatment strategy with dose modification is ongoing.

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Bradley Nicol, William F. Regine, Claire Courtney, Ali Meigooni, Michael Sanders and Byron Young

Object. The purpose of this paper was to assess the treatment of trigeminal neuralgia (TN) with the higher than normal dose of 90 Gy.

Methods. Forty-two patients with typical TN were treated over a 3-year period with gamma knife radiosurgery. Every patient received a maximum dose of 90 Gy in a single 4-mm isocenter targeted to the root entry zone of the trigeminal nerve. Thirty of 42 patients had undergone no prior treatments. The median follow-up period was 14 months (range 2–30 months).

Thirty-one patients (73.8%) achieved complete relief of pain. Nine patients (21.4%) obtained good pain control. Complications were limited to increased facial paresthesia in seven patients (16.7%) and dysgeusia in four patients (9.5%).

Conclusions. The authors conclude that the use of 90 Gy is a safe and effective dose for the treatment of TN.

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Anil A. Dhople, Jared R. Adams, William W. Maggio, Shahid A. Naqvi, William F. Regine and Young Kwok

Object

Few long-term studies of Gamma Knife surgery (GKS) for trigeminal neuralgia (TN) exist. The authors report their long-term experience with the use of GKS in a previously reported cohort of patients with TN that has now been followed since 1996.

Methods

One hundred twelve patients with TN were treated with GKS at the University of Maryland between June 1996 and July 2001. Of these, 67% had no invasive operations for TN prior to GKS, 13% had 1, 4% had 2, and 16% had ≥ 3. The right side was affected in 56% of cases, predominantly involving V2 (26%), V3 (24%), or a combination of both (18%) branches. The median age at diagnosis was 56 years, and median age at GKS was 64 years. The median prescription dose of 75 Gy (range 70–80 Gy) was delivered to the involved trigeminal nerve root entry zone. The authors assessed the degree of pain before and after GKS by using the Barrow Neurological Institute (BNI) pain scale.

Results

In total, 102 patients took the survey at least once, for a response rate of 91%. Although not found to alter the conclusions of this study, 7 cases of atypical TN were found and these patients were removed, for a total of 95 cases herein analyzed. The median follow-up was 5.6 years (range 13–115 months). Before GKS, 88% of patients categorized their pain as BNI IV or V (inadequate control or severe pain on medication), whereas the remainder described their pain as BNI III (some pain, but controlled on medication). After GKS, 64% reported a BNI score of I (no pain, no medications), 5% had BNI II (no pain, still on medication), 12% had BNI III, and 19% reported a BNI score of IV or V. The median time to response was 2 weeks (range 0–12 weeks) and the median response duration was 32 months (range 0–112 months). Eighty-one percent reported initial pain relief, and actuarial rates of freedom from treatment failure at 1, 3, 5, and 7 years were 60, 41, 34, and 22%, respectively. Response duration was significantly better for those who had no prior invasive treatment versus those in whom a previous surgical intervention had failed (32 vs 21 months, p < 0.02). New bothersome facial numbness was reported in 6% of cases.

Conclusions

This study represents one of the longest reported median follow-up periods and actuarial results for a cohort of patients with classic TN treated with GKS. Although GKS achieves excellent rates of initial pain relief, these results suggest a steady rate of late failure, particularly among patients who had undergone prior invasive surgical treatment. Despite a higher than expected recurrence rate, GKS remains a viable treatment option, particularly for patients who have had no prior invasive procedures. Patients with recurrences can still be offered salvage therapy with either repeat GKS, microvascular decompression, or rhizotomy.