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Kang-Du Liu, Wen-Yuh Chung, Hsiu-Mei Wu, Cheng-Ying Shiau, Ling-Wei Wang, Wan-You Guo and David Hung-Chi Pan

Object. The authors sought to determine the value of gamma knife surgery (GKS) in the treatment of cavernous hemangiomas (CHs).

Methods. Between 1993 and 2002, a total of 125 patients with symptomatic CHs were treated with GKS. Ninety-seven patients presented with bleeding and 45 of these had at least two bleeding episodes. Thirteen patients presented with seizures combined with hemorrhage, and 15 patients presented with seizures alone. The mean margin dose of radiation was 12.1 Gy and the mean follow-up time was 5.4 years.

In the 112 patients who had bled the number of rebleeds after GKS was 32. These rebleeds were defined both clinically and based on magnetic resonance imaging for an annual rebleeding rate of 32 episodes/492 patient-years or 6.5%. Twenty-three of the 32 rebleeding episodes occurred within 2 years after GKS. Nine episodes occurred after 2 years; thus, the annual rebleeding rate after GKS was 10.3% for the first 2 years and 3.3% thereafter (p = 0.0038). In the 45 patients with at least two bleeding episodes before GKS, the rebleeding rate dropped from 29.2% (55 episodes/188 patient-years) before treatment to 5% (10 episodes/197 patient-years) after treatment (p < 0.0001). Among the 28 patients who presented with seizures, 15 (53%) had good outcomes (Engel Grades I and II). In this study of 125 patients, symptomatic radiation-induced complications developed in only three patients.

Conclusions. Gamma knife surgery can effectively reduce the rebleeding rate after the first symptomatic hemorrhage in patients with CH. In addition, GKS may be useful in reducing the severity of seizures in patients with CH.

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David Hung-chi Pan, Wen-yuh Chung, Wan-yuo Guo, Hsiu-mei Wu, Kang-du Liu, Cheng-ying Shiau and Ling-wei Wang

Object. The aim of this study was to assess the efficacy and safety of radiosurgery for the treatment of dural arteriovenous fistulas (DAVFs) located in the region of the transverse—sigmoid sinus.

Methods. A series of 20 patients with DAVFs located in the transverse—sigmoid sinus, who were treated with gamma knife surgery between June 1995 and June 2000, was evaluated. According to the Cognard classification, the DAVF was Type I in four patients, Type IIa in seven, Type IIb in two, and combined Type IIa+b in seven. Nine patients had previously been treated with surgery and/or embolization, whereas 11 patients underwent radiosurgery alone. Radiosurgery was performed using multiple-isocenter irradiation of the delineated DAVF nidus. The target volume ranged from 1.7 to 40.7 cm3. The margin dose delivered to the nidus ranged from 16.5 to 19 Gy at a 50 to 70% isodose level.

Nineteen patients were available for follow-up review, the duration of which ranged from 6 to 58 months (median 19 months). Of the 19 patients, 14 (74%) were cured of their symptoms. At follow up, magnetic resonance imaging and/or angiography demonstrated complete obliteration of the DAVF in 11 patients (58%), subtotal obliteration (95% reduction of the nidus) in three (16%), and partial obliteration in another five (26%). There was no neurological complication related to the treatment. One patient experienced a recurrence of the DAVF 18 months after angiographic confirmation of total obliteration, and underwent a second course of radiosurgery.

Conclusions. Stereotactic radiosurgery provides a safe and effective option for the treatment of DAVFs involving the transverse and sigmoid sinuses. For some aggressive DAVFs with extensive retrograde cortical venous drainage, however, a combination of endovascular embolization and surgery may be necessary.

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Ming-liang Yang, Jian-jun Li, Shao-cheng Zhang, Liang-jie Du, Feng Gao, Jun Li, Yu-ming Wang, Hui-ming Gong and Liang Cheng

The authors report a case of functional improvement of the paralyzed diaphragm in high cervical quadriplegia via phrenic nerve neurotization using a functional spinal accessory nerve. Complete spinal cord injury at the C-2 level was diagnosed in a 44-year-old man. Left diaphragm activity was decreased, and the right diaphragm was completely paralyzed. When the level of metabolism or activity (for example, fever, sitting, or speech) slightly increased, dyspnea occurred. The patient underwent neurotization of the right phrenic nerve with the trapezius branch of the right spinal accessory nerve at 11 months postinjury. Four weeks after surgery, training of the synchronous activities of the trapezius muscle and inspiration was conducted. Six months after surgery, motion was observed in the previously paralyzed right diaphragm. The lung function evaluation indicated improvements in vital capacity and tidal volume. This patient was able to sit in a wheelchair and conduct outdoor activities without assisted ventilation 12 months after surgery.

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Wen-Yuh Chung, David Hung-Chi Pan, Cheng-Chia Lee, Hsiu-Mei Wu, Kang-Du Liu, Yu-Shu Yen, Wan-Yuo Guo, Cheng-Ying Shiau and Yang-Hsin Shih

Object

Although radiosurgery has been well accepted as a treatment for small- to medium-sized vestibular schwannomas (VSs), its application in the treatment of large VSs remains controversial because of unfavorable effects such as tumor swelling and potential compression of the brainstem. The authors present a retrospective study spanning 17 years, during which 21 patients underwent Gamma Knife surgery (GKS) for large VSs. Long-term outcomes are reported, and possible factors affecting tumor responses to GKS are analyzed.

Methods

Five hundred thirteen patients harboring VSs underwent GKS between March 1993 and October 2009. A large VS was defined as a tumor whose diameter was > 3 cm. This paper focuses on 21 patients who harbored large VSs ranging in volume from 12.7 to 25.2 cm3 (mean 17.3 cm3) and were treated by GKS. Fourteen of these patients had undergone 1 or more craniotomies previously to remove the tumor. Seven patients underwent GKS alone because of patient preference or a poor clinical condition that precluded microsurgery with general anesthesia. The mean radiation dose directed to the tumor ranged from 15 to 17.5 Gy. The mean radiation dose prescribed to the tumor margin was 11.9 Gy (range 11–14 Gy). The mean follow-up period was 66 months (range 12–155 months), and the median follow-up period was 53 months.

Results

The tumor control rate was 90.5% (19 of 21 lesions). No deterioration in facial nerve or trigeminal nerve function was noted. Disturbances in balance (some temporary) occurred in 5 patients. Three of the 21 patients developed initial tumor swelling, which required minor surgical interventions, including aspiration using an Ommaya reservoir or placement of a ventriculoperitoneal shunt. All 3 patients recovered satisfactorily after aspiration of an enlarging cyst or ventriculoperitoneal shunt placement. There was no significant correlation between tumor control and the following factors: patient age or sex, tumor volume, radiation dose, previous operation, presence of brainstem compression, petrous bone invasion, T2 signal ratio between tumor and brainstem, and presence of a cyst. However, there was a significant correlation between the T2 signal ratio between tumor and brainstem and the duration of tumor swelling (p = 0.003).

Conclusions

Treatment of large VSs remains a challenge to neurosurgeons regardless of whether they perform microsurgery or radiosurgery. Control of tumor growth and preservation of neurological function are the main goals of treatment. Although delayed microsurgery was required in 2 patients (9.5%), the satisfactory tumor control rate and excellent preservation of facial and trigeminal nerve function are the great advantages of radiosurgery. Radiosurgery is not only a practical treatment for patients with small- to medium-sized VSs, but it is also an excellent tool for treating larger tumors up to 25 cm3. In selected cases, radiosurgery plays an important role in treating large VSs with satisfactory results.

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Cheng-Chia Lee, Huai-Che Yang, Ching-Jen Chen, Yi-Chieh Hung, Hsiu-Mei Wu, Cheng-Ying Shiau, Wan-Yuo Guo, David Hung-Chi Pan, Wen-Yuh Chung and Kang-Du Liu

Object

Although craniopharyngiomas are benign intracranial tumors, their high recurrence rates and intimate associations with surrounding neurovascular structures make gross tumor resection challenging. Stereotactic radiosurgery has been introduced as a valuable adjuvant therapy for recurrent or residual craniopharyngiomas. However, studies with large patient populations documenting long-term survival and progression-free survival rates are rare in the literature. The current study aims to report the long-term radiosurgical results and to define the prognostic factors in a large cohort of patients with a craniopharyngioma.

Methods

A total of 137 consecutive patients who underwent 162 sessions of Gamma Knife surgery (GKS) treatments at the Taipei Veterans General Hospital between 1993 and 2012 were analyzed. The patients' median age was 30.1 years (range 1.5–84.9 years), and the median tumor volume was 5.5 ml (range 0.2–28.4 ml). There were 23 solid (16.8%), 23 cystic (16.8%), and 91 mixed solid and cystic (66.4%) craniopharyngiomas. GKS was indicated for residual or recurrent craniopharyngiomas. The median radiation dose was 12 Gy (range 9.5–16.0 Gy) at a median isodose line of 55% (range 50%–78%).

Results

At a median imaging follow-up of 45.7 months after GKS, the rates of tumor control were 72.7%, 73.9%, and 66.3% for the solid, cystic, and mixed tumors, respectively. The actuarial progression-free survival rates plotted by the Kaplan-Meier method were 70.0% and 43.8% at 5 and 10 years after radiosurgery, respectively. After repeated GKS, the actuarial progression-free survival rates were increased to 77.3% and 61.2% at 5 and 10 years, respectively. The overall survival rates were 91.5% and 83.9% at the 5- and 10-year follow-ups, respectively. Successful GKS treatment can be predicted by tumor volume (p = 0.011). Among the 137 patients who had clinical follow-up, new-onset or worsened pituitary deficiencies were detected in 11 patients (8.0%). Two patients without tumor growth had a worsened visual field, and 1 patient had a new onset of third cranial nerve palsy.

Conclusions

The current study suggests that GKS is a relatively safe modality for the treatment of recurrent or residual craniopharyngiomas, and it is associated with improved tumor control and reduced in-field recurrence rates. Acceptable rates of complications occurred.

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Cheng-Chia Lee, Jason P. Sheehan, Hideyuki Kano, Berkcan Akpinar, Roberto Martinez-Alvarez, Nuria Martinez-Moreno, Wan-Yuo Guo, L. Dade Lunsford and Kang-Du Liu

OBJECTIVE

Cavernous sinus hemangiomas (CSHs) are rare vascular tumors. A direct microsurgical approach usually results in massive hemorrhage and incomplete tumor resection. Although stereotactic radiosurgery (SRS) has emerged as a therapeutic alternative to microsurgery, outcome studies are few. Authors of the present study evaluated the role of SRS for CSH.

METHODS

An international multicenter study was conducted to review outcome data in 31 patients with CSH. Eleven patients had initial microsurgery before SRS, and the other 20 patients (64.5%) underwent Gamma Knife SRS as the primary management for their CSH. Median age at the time of radiosurgery was 47 years, and 77.4% of patients had cranial nerve dysfunction before SRS. Patients received a median tumor margin dose of 12.6 Gy (range 12–19 Gy) at a median isodose of 55%.

RESULTS

Tumor regression was confirmed by imaging in all 31 patients, and all patients had greater than 50% reduction in tumor volume at 6 months post-SRS. No patient had delayed tumor growth, new cranial neuropathy, visual function deterioration, adverse radiation effects, or hypopituitarism after SRS. Twenty-four patients had presented with cranial nerve disorders before SRS, and 6 (25%) of them had gradual improvement. Four (66.7%) of the 6 patients with orbital symptoms had symptomatic relief at the last follow-up.

CONCLUSIONS

Stereotactic radiosurgery was effective in reducing the volume of CSH and attaining long-term tumor control in all patients at a median of 40 months. The authors' experience suggests that SRS is a reasonable primary and adjuvant treatment modality for patients in whom a CSH is diagnosed.

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Ling-Wei Wang, Cheng-Ying Shiau, Wen-Yuh Chung, Hsiu-Mei Wu, Wan-Yuo Guo, Kang-Du Liu, Donald Ming-tak Ho, Tai-Tong Wong and David Hung-Chi Pan

Object

The authors report the long-term treatment results of Gamma Knife surgery (GKS) for patients with low-grade astrocytomas who underwent surgery at a single institution.

Methods

A series of 21 patients (median age 20 years) with 25 intracranial low-grade astrocytomas (World Health Organization Grades I and II) were treated with GKS between 1993 and 2003. Among them, four underwent GKS as a primary treatment. Two underwent GKS as a treatment boost after radiotherapy. In the other 15 patients, GKS was performed as an adjuvant or salvage treatment for residual/recurrent tumors after the patients had undergone craniotomy. Tumor volumes ranged from 0.2 to 13.3 ml (median 2.4 ml). Prescription margin doses ranged from 8 to 18 Gy (median 14.5 Gy). Radiation volumes were 1.3 to 21.6 ml (median 3.6 ml). Patients underwent regular follow up, with neurological evaluation and magnetic resonance imaging studies obtained at 6-month intervals.

One patient was lost to follow-up. The clinical follow-up time was 5 to 144 months (median 67 months). Complete tumor remission was seen in three patients. The 10-year progression-free patient survival rate after GKS was 65%. Tumor progression was found in six patients of whom five received further salvage treatment. All the tumor progression occurred within the GKS-treated volumes. Mild-to-moderate adverse radiation effects (AREs) were found in eight patients. Both of the patients who had undergone GKS as a treatment boost after radiotherapy developed AREs, but with good shrinkage of tumors.

Conclusions

Gamma Knife surgery provides durable long-term local tumor control with acceptable toxicity for some patients with highly selected low-grade astrocytomas.

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Cheng-Chia Lee, David Hung-Chi Pan, Wen-Yuh Chung, Kang-Du Liu, Huai-Che Yang, Hsiu-Mei Wu, Wan-Yuo Guo and Yang-Hsin Shih

Object

The authors retrospectively reviewed the efficacy and safety of Gamma Knife surgery (GKS) in patients with brainstem cavernous malformations (CMs). The CMs had bled repeatedly and placed the patients at high risk with respect to surgical intervention.

Methods

Between 1993 and 2010, 49 patients with symptomatic CMs were treated by GKS. The mean age in these patients was 37.8 years, and the predominant sex was female (59.2%). All 49 patients experienced at least 2 instances of repeated bleeding before GKS; these hemorrhages caused neurological deficits including cranial nerve deficits, hemiparesis, hemisensory deficits, spasticity, chorea or athetosis, and consciousness disturbance.

Results

The mean size of the CMs at the time of GKS was 3.2 cm3 (range 0.1–14.6 cm3). The mean radiation dose directed to the lesion was 11 Gy with an isodose level at 60.0%. The mean clinical and imaging follow-up time was 40.6 months (range 1.0–150.7 months). Forty-five patients participated in regularly scheduled follow-up. Twenty-nine patients (59.2%) were followed up for > 2 years, and 16 (32.7%) were followed up for < 2 years. The pre-GKS annual hemorrhage rate was 31.3% (69 symptomatic hemorrhages during a total of 220.3 patient-years). After GKS, 3 episodes of symptomatic hemorrhage were observed within the first 2 years of follow-up (4.29% annual hemorrhage rate), and 3 episodes of symptomatic hemorrhage were observed after the first 2 years of follow-up (3.64% annual hemorrhage rate). In this study of 49 patients, symptomatic radiation-induced complications developed in only 2 patients (4.1%; cyst formation in 1 patient and perifocal edema with neurological deficits in the other patient). There were no deaths in this group.

Conclusions

Gamma Knife surgery is effective in reducing the rate of recurrent hemorrhage. In the authors' experience, it was possible to control bleeding using a low-dose treatment. In addition, there were few symptomatic radiation-induced complications. As a result, the authors believe that GKS is a good alternative treatment for brainstem CMs.

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Wen-Yuh Chung, Cheng-Ying Shiau, Hsiu-Mei Wu, Kang-Du Liu, Wan-Yuo Guo, Ling-Wei Wang and David Hung-Chi Pan

Object

The effectiveness and safety of radiosurgery for small- to medium-sized cerebral arteriovenous malformations (AVMs) have been well established. However, the management for large cerebral AVMs remains a great challenge to neurosurgeons. In the past 5 years the authors performed preplanned staged radiosurgery to treat extra-large cerebral AVMs.

Methods

An extra-large cerebral AVM is defined as one with nidus volume > 40 ml. The nidus volume of cerebral AVM is measured from the dose plan—that is, as being the volume contained within the best-fit prescription isodose. From January 2003 to December 2007, the authors treated 6 patients with extra-large AVMs by preplanned staged GKS. Staged radiosurgery is implemented by rigid transformation with translation and rotation of coordinates between 2 stages. The average radiation-targeted volume was 60 ml (range 47–72 ml). The presenting symptoms were seizure in 4 patients and a bleeding episode in 2. One patient had undergone a previous craniotomy and evacuation of hematoma. The mean interval between the 2 radiosurgical sessions was 6.9 months (range 4.5–9.1 months). The prescribed marginal dose given to the nidus volume in each stage ranged from 16 to 18.6 Gy. The expected marginal dose of total nidus was 17–19 Gy. Regular follow-up MR imaging was performed every 6 months. The mean follow-up period was 28 months (range 12–54 months).

Results

Most of the patients exhibited clinical improvement: relief of headache and reduced frequency of seizure attack. All patients had significant regression of nidus observed on MR imaging follow-up. Two patients had angiogram-confirmed complete obliteration of the nidus 45 and 60 months after the second-stage radiosurgical session. One patient experienced minor bleeding 8 months after the second-stage radiosurgery with mild headache. She had satisfactory recovery without clinical neurological deficit after conservative treatment.

Conclusions

These preliminary results indicate that staged radiosurgery is a practical strategy to treat patients with extra-large cerebral AVMs. It takes longer to obliterate the AVMs. The observed high signal T2 changes after the radiosurgery appeared clinically insignificant in 6 patients followed up for an average of 28 months. Longer follow-up is necessary to confirm its long-term safety.

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Hsiu-Mei Wu, David Hung-Chi Pan, Wen-Yuh Chung, Wan-Yuo Guo, Kang-Du Liu, Cheng-Ying Shiau, Ling-Wei Wang and Shih-Jen Chen

Object

The purpose of this study was to assess the efficacy and safety of Gamma Knife surgery (GKS) for the treatment of cavernous sinus dural arteriovenous fistulas (CSDAVFs) and other intracranial dural arteriovenous fistulas (ODAVFs).

Methods

Among the 238 GKS procedures performed for intracranial DAVFs in the authors' institute, 227 cases (146 CSDAVFs and 81 OIDAVFs) with clinical follow up formed the database from which the authors determined clinical outcome and the incidence of untoward events. One hundred ninety-five cases (118 CSDAVFs and 77 ODAVFs) with imaging follow up formed the database from which the authors determined the imaging results.

Older age, female sex, higher incidence of diabetes, and shorter duration of symptoms were noted more in cases of CSDAVF than in ODAVFs. Most patients had symptomatic improvement after GKS. A symptomatic cure was observed in one patient with CSDAVFs as early as 6 weeks. The cumulative cure rate based on follow-up angiography of CSDAVFs approached 75% at 24 months, which was much better than that of ODAVFs (approximately 50% at 24 months). A neuroimaging-based cure lagged behind that of the clinical symptoms. Overall, there were only two nonfatal intracerebral hemorrhages during the follow-up period, both occurring less than 1 week after GKS and both being Cognard Type IIa+b with initial aggressive symptoms. Transient deterioration of neurological status without hemorrhage was noted in six patients with ODAVFs. Thrombosis of the superior ophthalmic vein occurred in 11 patients with CSDAVFs, in two of whom there were unilateral visual impairments. There were three cranial nerve neuropathies: transient in one CSDAVF and one ODAVF involving the jugular foramen, and another one was a CSDAVF previously treated by conventional radiotherapy.

Conclusions

Gamma Knife surgery provides a safe and effective option for treatment of intracranial DAVFs with a low risk of complications. In cases of DAVFs with benign clinical presentation, GKS can serve as a primary treatment. In some cases of aggressive DAVFs in which there is extensive retrograde cortical vein drainage, combined treatment with embolization or surgery is suggested.