Jugulotympanic paragangliomas treated with Gamma Knife radiosurgery: a single-center review of 58 cases

Clinical article

Full access

Object

Jugulotympanic paragangliomas (JTPs) are rare benign tumors whose surgical treatment is usually associated with partial resection of the lesion, high morbidity, and even death. Gamma Knife radiosurgery (GKRS) has been reported as a useful treatment option. The goal of this retrospective study is to analyze the role of GKRS in tumor volume control and clinical outcomes of these patients.

Methods

A total of 75 patients with JTPs were treated with GKRS at the authors' center from 1995 to 2012. The authors analyzed those treated during this period to allow for a minimal observation time of 2 years. The MR images and clinical reports of these patients were reviewed to assess clinical and volumetric outcomes of the tumors. The radiological and clinical assessments, along with a group of prognostic factors measured, were analyzed using descriptive methods. The time to volumetric and clinical progression was analyzed using the Kaplan-Meier method. Prognostic factors were identified using log-rank statistics and multivariate Cox regression models.

Results

The mean follow-up was 86.4 months. The authors observed volumetric tumor control in 94.8% of cases. In 67.2% of cases, tumor volume decreased by a mean of 40.1% from the original size. Of patients with previous tinnitus, 54% reported complete recovery. Improvement of other symptoms was observed in 34.5% of cases. Overall, clinical control was achieved in 91.4% of cases. Previous embolization and familial history of paraganglioma were selected as significant prognostic factors for volumetric response to GKRS treatment in the univariate analysis. In multivariate analysis, no factors were significantly correlated with progression-free survival. No patient died of side effects related to GKRS treatment or tumor progression.

Conclusions

Gamma Knife radiosurgery is an effective, safe, and efficient therapeutic option for the treatment of these tumors as a first-line treatment or in conjunction with traditional surgery, endovascular treatment, or conventional fractionated radiotherapy.

Abbreviations used in this paper:CN = cranial nerve; GKRS = Gamma Knife radiosurgery; HR = hazard ratio; JTP = jugulotympanic paraganglioma; SRS = stereotactic radiosurgery.

Object

Jugulotympanic paragangliomas (JTPs) are rare benign tumors whose surgical treatment is usually associated with partial resection of the lesion, high morbidity, and even death. Gamma Knife radiosurgery (GKRS) has been reported as a useful treatment option. The goal of this retrospective study is to analyze the role of GKRS in tumor volume control and clinical outcomes of these patients.

Methods

A total of 75 patients with JTPs were treated with GKRS at the authors' center from 1995 to 2012. The authors analyzed those treated during this period to allow for a minimal observation time of 2 years. The MR images and clinical reports of these patients were reviewed to assess clinical and volumetric outcomes of the tumors. The radiological and clinical assessments, along with a group of prognostic factors measured, were analyzed using descriptive methods. The time to volumetric and clinical progression was analyzed using the Kaplan-Meier method. Prognostic factors were identified using log-rank statistics and multivariate Cox regression models.

Results

The mean follow-up was 86.4 months. The authors observed volumetric tumor control in 94.8% of cases. In 67.2% of cases, tumor volume decreased by a mean of 40.1% from the original size. Of patients with previous tinnitus, 54% reported complete recovery. Improvement of other symptoms was observed in 34.5% of cases. Overall, clinical control was achieved in 91.4% of cases. Previous embolization and familial history of paraganglioma were selected as significant prognostic factors for volumetric response to GKRS treatment in the univariate analysis. In multivariate analysis, no factors were significantly correlated with progression-free survival. No patient died of side effects related to GKRS treatment or tumor progression.

Conclusions

Gamma Knife radiosurgery is an effective, safe, and efficient therapeutic option for the treatment of these tumors as a first-line treatment or in conjunction with traditional surgery, endovascular treatment, or conventional fractionated radiotherapy.

Paragangliomas of the head and neck, also known as chemodectomas or glomus tumors, are highly vascular neuroendocrine neoplasms that arise from paraganglionic tissue.6,23 These entities comprise tumors arising in the carotid body, the jugular bulb, the tympanic branch of the glossopharyngeal nerve (Jacobson's nerve), and the auricular branch of the vagus nerve (Arnold's nerve).19 Those affecting the carotid body are by far the most common type, and surgery has been shown to be a safe treatment option for them.22

However, treatment of jugulotympanic paragangliomas (JTPs) is challenging. These tumors are not as frequent as carotid body paragangliomas, with an estimated incidence of only 1 in 1.3 million,10,13,21 and they are usually benign, slow-growing tumors.19,22 Malignant variations occur in about 3% of cases,9 and metastases can be observed in 1%–4% of cases.3,13 Both benign and malignant variations cause very disabling symptoms; patients may present with severe hearing loss, tinnitus, other cranial nerve deficits, dizziness, and occasionally venous thrombosis.23

Traditionally, the management of these tumors has involved microsurgical resection and/or radiation therapy, sometimes preceded by preoperative embolization.12 Microsurgical removal can be accomplished through reasonably safe approaches, but even the most recent surgical series reported incomplete tumor removal in up to 20% of patients.23 Furthermore, significant morbidity and reduced quality of life are common downsides of surgical therapy,11,14,16,23 with 50% of patients experiencing permanent lower cranial nerve palsies after surgery.23 Finally, it should also be acknowledged that, according to recent publications, surgery carries a mortality rate of about 4%.22,26

To avoid the significant morbidity associated with excision, conventional radiotherapy has been used increasingly in the treatment of JTPs.2,28 However, this treatment has side effects.23 Because of its efficacy and precision, Gamma Knife radiosurgery (GKRS) has been used successfully to treat JTPs in many centers,4,5,7–9,15,19,20,24,26,29 minimizing the radiation adverse effects.

Gamma Knife radiosurgery was performed in 75 patients with JTPs at our center. According to our review of the literature, it is one of the largest cohorts of patients reported to date. Unlike previous studies, where the short follow-up may have made it difficult to draw conclusions about these slow-growing tumors, we evaluated clinical and radiological outcomes during long-term follow-up in a large group of patients. Such a large cohort with long-term follow-up has not been reported previously in the literature.

Methods

Patient Population

We retrospectively reviewed the prospectively maintained database of the Gamma Knife Unit at the International Ruber Hospital of Madrid and searched for JTPs cases. During an 18-year period, from January 1995 to December 2012, 75 patients were radiosurgically treated for JTPs at our institution. Medical records, imaging studies, and operative reports were reviewed. To allow for an observation time of at least 2 years in these slow-growing tumors, 13 patients treated after 2010 were excluded. Four other patients were lost to follow-up. A final cohort of 58 patients with complete outcome data were analyzed, with a mean follow-up of 86.4 months and a median follow-up of 76.6 months.

Management

Patients were treated with GKRS after all therapeutic options were considered by a multidisciplinary team of neurosurgeons and radiation oncologists. All patients were treated with the Leksell Gamma Knife (Models B, 4C, and PFX; Elekta AB) using existing planning software.

Stereotactic radiosurgery (SRS) was performed in a single session in all cases. The mean marginal dose was 13.6 Gy (range 11–15 Gy), and the mean maximum dose was 25.2 Gy (range 18–55 Gy). The mean isodose line was 55.3% (range 40%–65%), the mean number of isocenters was 10.8 (range 2–29).

The doses received by critical structures were a maximal dose of 10 Gy to the brainstem and of 11 Gy to the facial nerve. Although we tried to minimize the dose received by cranial nerves (CNs) V and VIII and lower, in some cases they were included in the tumor, and they received the same dose as the tumor itself. All radiosurgical treatments were performed by the same team of neurosurgeons and radiation oncologists.

Clinical and Radiological Outcome Assessment

Clinical outcomes were assessed by a complete neurological examination that included cranial nerve function. Patients were assessed by an otolaryngology specialist, a neurologist, or a neurosurgeon who were not directly involved in the care of the patients. Patients underwent follow-up at annual intervals beginning 12 months after undergoing SRS. The patient's neurological status prior to treatment was used as a reference point, and outcomes were expressed in terms of change from this baseline as improved, unchanged, or worse. Asymptomatic patients without new neurological deficits were classified as unchanged.

To evaluate the tumor, MRI sequences (T1, T2, fat suppression, and T1 after gadolinium administration) were routinely acquired in all patients. Radiosurgical planning was performed using MR images obtained under stereotactic conditions. After SRS, the same MRI sequences were acquired to evaluate for variations in tumor size. Follow-up imaging was performed at annual intervals beginning 12 months after surgery to assess the radiographic response to treatment. A neuroradiologist not directly involved in the care of the patient classified the tumor size as decreased, increased, or unchanged. Change in tumor size was reported as cubic centimeters of volume. Tumors were considered controlled if they were reported as decreased or unchanged on the follow-up volume images.

Statistical Analysis

In a descriptive analysis, frequencies and percentages are given for the categorical variables, and mean, median, and ranges are given for continuous variables.

The following potential prognostic factors at diagnosis were studied in a univariate analysis: sex, age, right or left location, paraganglioma familial history, tumor volume, ataxia, lower cranial nerve neuropathy, previous treatment with radiotherapy, previous embolization treatment, and previously operated tumor at baseline. Marginal dose, maximal dose, and number of isocenters were selected as the most relevant variables related with SRS radiation for the univariate and multivariate analysis.

The correlation of all the baseline characteristics with the radiological and clinical outcomes in terms of time to progression was analyzed using the univariate log-rank test and proportional hazards Cox regression. Frequency tables and summary statistics were used to show the distribution of the baseline characteristics in the response groups (increased, unchanged, or decreased volume for volumetric outcomes; improved, unchanged, or worse for clinical outcomes).

The variables showing statistical significance at the 10% level in the univariate analysis along with other clinically relevant variables of known prognostic value were entered in a multivariate logistic regression model. The hazard ratios (HRs) and their confidence intervals are displayed for the variables included in the univariate and multivariate regression models.

Results

There were 40 women and 18 men with a mean age of 52.4 years (range 19–82 years); 32 patients (55%) had left-sided tumors and 26 patients (45%) had right-sided tumors. The most common symptom at presentation was tinnitus. Four patients were known familial cases.

Of the 58 patients reviewed, 18 had undergone prior microsurgical treatment and had histological confirmation of their tumors. Four of these patients had also been treated previously with embolization and 3 patients had received radiotherapy. Forty patients had not been treated surgically before GKRS. In these patients, the diagnosis had been established on the basis of imaging studies (CT scanning, MRI, or angiography) and clinical presentation. Of the 40 patients not treated with surgery, 6 had received endovascular treatment and 1 had received only radiotherapy before being referred to our institution.

Nine percent of patients presented with ataxia. Hearing loss was present at diagnosis in 79.3% of 58 patients (94.4% in patients who had undergone previous surgery, 72.5% who had not undergone previous surgery). Overall, 72.4% patients presented with lower cranial nerve neuropathy before SRS (83.3% in operated and 67.5% in non-operated patients).

Images obtained before SRS demonstrated a tumor volume that ranged from 0.32 to 62.2 cm3 (mean 12 cm3, median 9.3 cm3). Follow-up imaging revealed an increased volume in 3 patients (5.2%). Thirty-nine patients (67.2%) exhibited a decrease in tumor volume, and 16 patients (27.6%) showed no change in tumor size. Thus, tumor control was achieved in 55 patients (94.8%) (Fig. 1). The decrease in tumor volume observed in 39 patients was accurately measured. The mean volume decrease was 5.6 cm3 (range 0.75–15.55 cm3), corresponding to a reduction in size of 40.1% (range 5%–95%).

Fig. 1.
Fig. 1.

Pie chart showing the tumor volume outcomes at the end of follow-up.

Five patients (8.6%) experienced clinical worsening after treatment. Causes of impairment included hearing loss in 4 patients and dysphagia in 1 patient. In 3 of these patients the impairment was related to tumor growth (2 with hearing loss and 1 with dysphagia). All patients reported improvement of their previous tinnitus, but only 54% of cases reported complete recovery. Most of these patients reported this improvement at the first control visit 12 months after treatment. In addition to tinnitus, 20 patients (34.5%) showed improvement in other symptoms after GKRS. Thirty-three patients (56.9%) showed no clinical changes according to the most recent follow-up data available (Fig. 2).

Fig. 2.
Fig. 2.

Pie chart showing the clinical outcomes at the end of follow-up (tinnitus is not included).

No patient died during the perioperative period. Three patients died later of other unrelated causes, and none experienced clinical deterioration in terms of JTP progression or treatment-related side effects before death.

In a univariate analysis using the Kaplan-Meier method and log-rank statistics, the independent risk factors with statistical significance less than 10% were previous embolization (p = 0.0603) and paraganglioma familial history (p = 0.0102) for the volumetric progression (Table 1). Lateral right location (p = 0.0833) was an independent risk factor for clinical progression (Table 2).

TABLE 1:

Prognostic factors related to volumetric tumor progression-free survival: univariate analysis*

Variablep ValueHR95% CI
lateral location (lt/rt)0.1051
sex (M/F)0.80270.7370.067–8.156
previous radiotherapy (yes/no)0.6084
previous embolization (yes)0.06030.1370.012–1.535
ataxia (yes/no)0.5863
familial antecedents (yes)0.01020.0650.004–1.055
previous surgery (yes/no)0.17140.2180.020–2.404
age (<55/≥55 yrs)0.61501.8430.164–20.716
tumor vol (<10/≥10 cm3)0.74531.4850.135–16.374
marginal dose (<13.5/≥13.5 Gy)0.1631
lower cranial nerve involvement (yes/no)0.3001
age0.70251.0150.941–1.094
tumor vol0.35350.9020.726–1.121
marginal dose0.17283.1450.606–16.337
maximal dose0.23620.7540.473–1.203
no. of isocenters0.87210.9810.772–1.246

Values in boldface are statistically significant. — = Hazard ratio could not be calculated due to the low number of progression events in some groups.

The categories with higher risk are shown for the significant prognostic factors.

Log-rank test Cox regression.

TABLE 2:

Prognostic factors related to clinical tumor progression-free survival: univariate analysis*

Variablep ValueHR95% CI
lateral location (rt)0.08330.1790.020–1.606
sex (M/F)0.59811.7890.200–16.023
previous radiotherapy (yes/no)0.5545
previous embolization (yes/no)0.2814
ataxia (yes/no)0.35140.3680.041–3.296
familial antecedents (yes/no)0.31980.3450.039–3.099
previous surgery (yes/no)0.1075
age (<55/≥55 yrs)0.65690.6680.112–4.005
tumor vol (<10/≥10 cm3)0.80931.2460.208–7.446
marginal dose (<13.5/≥13.5 Gy)0.30850.3370.038–3.029
lower cranial nerve involvement (yes/no)0.7066
age0.74711.0090.956–1.065
tumor vol0.35960.9110.746–1.112
marginal dose0.34701.6580.578–4.752
maximal dose0.52630.9160.698–1.202
no. of isocenters0.91051.0090.867–1.173

Values in boldface are statistically significant. — = Hazard ratio could not be calculated due to the low number of progression events in some groups.

The categories with higher risk are shown for the significant prognostic factors.

Log-rank test/Cox regression.

Several multivariate Cox regression models have been tested. The predictive factors included in the models were the factors mentioned above that reached a p value < 0.10 in the univariate analysis. No independent predictive factors reached 5% statistical significance in the multivariate logistic regression for clinical or radiological outcomes alone (Figs. 3 and 4).

Fig. 3.
Fig. 3.

Kaplan-Meier curve for volumetric progression.

Fig. 4.
Fig. 4.

Kaplan-Meier curve for clinical progression.

Due to the high number of patients free from progression (55 for volumetric assessment and 53 for clinical assessment), the median time to progression was not reached at the time of the analysis, and high censoring made it impossible to calculate HRs for some variables.

Discussion

Paragangliomas of the head and neck are uncommon tumors. According to Neskey and colleagues,22 carotid paraganglioma is the most frequent among them, representing 63%–78% of cases followed by JTPs and vagal paragangiomas.

Jugulotympanic paragangliomas represent 0.03% of all neoplasms and 0.6% of all head and neck tumors.23 Their incidence in the general population is only 1 in 1.3 million, and they grow very slowly.13 Jansen and colleagues estimated the growth rate of these tumors to be 0.79 mm per year.17

Because JTPs are seen so infrequently, it is difficult to find large numbers of patients to study. In addition, because of their slow-growing nature, it is important to study patients with these tumors for many years after treatment. For these reasons, there have been few review articles that provide information on large number of patients with JTPs with long-term follow-up.

One of the largest cohorts was published by Liscák et al.19 in 1999. These authors described the results of a first multicenter study of 66 cases, of which 52 were followed during a median of 24 months. However, the largest multicenter series was recently reported by Sheehan et al.26 They observed 132 patients for a median of 50.5 months and found a high rate of long-term tumor control. In spite of our smaller number of patients, we assessed clinical and imaging outcomes of these patients for a longer median follow-up period, and our results are comparable to those in the large North American series.

In 2002 Eustacchio et al.4 reported a single-center review of 19 cases that had been observed for a median of 86.4 months. This follow-up is the longest in a single-center review to date. In 2008 Sharma et al.24 published the largest single-center series, in which 24 cases of paraganglioma treated using GKRS were analyzed during a mean of 26.1 months.

To the best of our knowledge, there has been no single-center study of a group of patients as large as the one we report here, with 75 patients treated and 58 analyzed. The patients we report on were followed for a mean of 86.4 months, a longer period than most of the series we found in the literature.

These tumors pose a difficult therapeutic challenge because of their location and highly vascular nature. Several management options, including surgical removal, endovascular embolization, and radiotherapy, have been described elsewhere.26 Their surgical management is associated with poor local control and a high incidence of recurrence, morbidity, and mortality.25 Surgical approaches are challenging because of the proximity of critical vascular and neural structures that are frequently invaded by the tumor.1,18 Jackson and colleagues16 reviewed 18 cases of incompletely resected tumors and found that 9 of them had progressed to the point that they required further interventions.

The proximity of these tumors to the cranial nerves, specifically, to CNs VII through XII, elevates the risk of postoperative deficits. Neskey and colleagues22 found a transient cranial nerve deficit during the 1st postoperative month in 96% of cases, with 50% experiencing permanent deficits. This is consistent with the rates of deficits reported in other series. In the one reported by Jackson et al.,16 new deficits were found in CNs IX, X, XI, and XII in 39%, 25%, 26%, and 21% of cases, respectively.

Green et al.11 reported on 52 patients who experienced complications such as pneumonia, pulmonary embolus, aspiration, and meningitis after microsurgery. In this series 19% of patients were subsequently treated for vocal cord paralysis, 8% required thyroplasty, and 8% required prolonged feedings via a nasogastric tube. Additionally, 29% of patients reported persistent hoarseness and 29% reported dysphagia. These deficits are very disabling and negatively affect the quality of life and psychological well being of the patient.

Another therapeutic option for the treatment of JTPs is radiotherapy, which was first used in the 1950s as adjuvant treatment. In 1990 Springate and Weichselbaum28 published the first meta-analysis of patients treated with radiotherapy alone and reported tumor control in 93% of cases. According to Bitaraf et al.,2 radiotherapy provided tumor control in 61%–94% of cases, using doses of 45–55 Gy. However, treatment of these tumors with radiotherapy is not without side effects. Acute complications such as mastoiditis, dermatitis, alopecia, mucositis, otitis, taste alterations, or cerebrovascular events have been reported in 4%–20% of patients.23 Facial palsy, hearing loss, temporal bone or brain radionecrosis, and radiation-induced tumors have been reported as chronic complications.23

In the 1990s, SRS began to be used for the treatment of these lesions. This is a minimally invasive therapeutic option that enables the patient to be treated in a single session with minimal radiation exposure to the surrounding healthy tissues. The precision and accuracy of GKRS minimizes adverse effects to the lowest possible level. The posttreatment morbidity is very low, and to date there have been no reports of treatment-associated mortality.

Liscák and colleagues19 were the first to study a large cohort of 52 patients. In their multicenter study, the authors were able to demonstrate clinical tumor control in 90% of patients and tumor size control in 60% of patients. Successive review articles have since been published that demonstrate that GKRS provides good control of symptoms and tumor volume (Table 3).

TABLE 3:

Single- and multicenter studies of patients with JTPs treated with GKRS

Authors & YearType of ReviewNo. of PatientsFollow-Up (mos)Control (%)
MedianMeanTumorClinical
Liscák et al., 1999multicenter522410094.2
Eustacchio et al., 2002single center1986.494.794.7
Sheehan et al., 2005single center828100100
Varma et al., 2006single center17487188
Gerosa et al., 2006single center2050.910090
Sharma et al., 2008single center2426.1100100
Ganz & Abdelkarim, 2009single center1428100100
Miller et al., 2009single center53480100
Sheehan et al., 2012multicenter132 (134 procedures)50.59385
present studysingle center5876.686.494.891.4

The rate of control of tumor volume varies between 71% and 100% in these studies. The rate of tumor volume control that we achieved corroborates the rates previously reported in the literature (Fig. 5).

Fig. 5.
Fig. 5.

Magnetic resonance images obtained in a patient with a large left-sided JTP. A: Axial and coronal images obtained at the time of GKRS treatment. B: Axial and coronal images obtained 72 months after treatment. C: Axial and coronal images obtained 112 months posttreatment.

Previous embolization and familial history of paraganglioma were related to tumor growth after GKRS in the univariate analysis. However, these factors were not statistically correlated with progression-free survival in the multivariate analysis. The low rate of volumetric progression could explain why no factors have been identified as independent risk factors.

In terms of clinical control, the majority of patients did not experience changes after treatment. The rate of clinical control that we observed is similar to those previously reported by other authors.

One patient who experienced dysphagia as new deficit was found to have tumor growth. Another 2 patients experienced decreased hearing acuity, which was also associated with increased tumor volume. This supports the hypothesis described by Sheehan et al.26 that the development of new or worsening cranial nerve deficits is a good predictor of tumor growth.

According to our review and the reports of other authors, tinnitus and hearing loss are the symptoms found to improve most significantly after treatment with GKRS. All patients with tinnitus reported some degree of improvement, perhaps because they had become accustomed it; however, only 54% experienced complete recovery. Additionally, we found that several patients had improved symptoms of lower cranial nerve palsies, which has also been described by other authors.

None of the risk factors included in the multivariate analysis reached statistical significance for clinical progression-free survival, which may be due to the low rate of clinical progression. A larger series and a longer follow-up would be useful to uncover these factors.

The current study and the previous literature prove that GKRS is not only effective at controlling the growth of JTPs but is also effective at controlling the clinical symptoms of these tumors. In 2 cases, hearing loss was related to tumor growth. Tumor growth was not observed in the other 2 cases. Gamma Knife radiosurgery may have been responsible for this clinical worsening.

Sheehan et al.27 reported 2 cases of GKRS-induced meningiomas in patients treated for arteriovenous malformations. In the present series, we did not observe radiation-induced neoplasia, malignant transformation, or other symptoms or signs related to the GKRS treatment of JTPs; however, we recommend that these patients be followed for an extended period. Overall, the rate of reported side effects with GKRS is much lower than the rate of side effects with radiotherapy.

In 2004 Gottfried and colleagues10 published a study in which SRS and microsurgery were compared. They found that both methods achieved similar levels of tumor control. Even though they recognized that a big difference exists in morbidity favoring SRS, they expressed reservations about SRS due to the lack of long-term studies of such slow-growing tumors and were unable to evaluate the possibility of future recurrences.

Ivan and colleagues15 conducted a meta-analysis in which they compared the morbidity of surgery and SRS. In that study, 869 patients were separated into 4 groups according to the treatment they had received: subtotal resection, gross-total resection, subtotal resection combined with SRS, or SRS alone. Patients undergoing SRS alone experienced the lowest rates of recurrence of these 4 groups. In addition, the incidence of lower cranial nerve deficits was found to be higher in gross-total resection than in those who underwent SRS alone.

A larger number of patients and a longer follow-up are necessary to detect statistically significant risk factors related to the volumetric or clinical progression of JTPs; however, our review demonstrates that the rates of tumor control observed in previously published studies during periods of short-term follow-up are maintained when the follow-up interval is prolonged. Our review corroborates the findings of Eustacchio et al.4 and other authors7,9,19,20,24–26,29 that GKRS provides good clinical and tumor control that persists for long periods of time after treatment.

Additionally, death related to GKRS has not been described, and the morbidity associated with GKRS is very low and not disabling. Thus, GKRS should be considered a viable option in the primary treatment of these neoplasms, alone or in combination with other therapeutic treatment options.

Conclusions

Our experience with GKRS for JTPs confirms that it is an effective, safe, and efficient therapeutic option for these tumors, either as a first-line treatment or associated with surgery, endovascular treatment, or radiotherapy.

Acknowledgments

We would like to thank Drs. A. Rodríguez-Hernández and G. Palatucci for their collaboration with the preparation of the manuscript, A. de Campos Kusak for his assistance with the graphic material, and J. Gómez for his collaboration with the statistical content.

Disclosure

Dr. Martínez Álvarez is a consultant with Elekta AB. The authors report no other conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Author contributions to the study and manuscript preparation include the following. Conception and design: all authors. Acquisition of data: Gandía-González, Kusak. Analysis and interpretation of data: Gandía-González. Drafting the article: Gandía-González. Critically revising the article: all authors. Reviewed submitted version of manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Gandía-González. Statistical analysis: Gandía-González. Administrative/technical/material support: Gandía-González, Kusak, Martínez Moreno, Gutiérrez Sárraga, Martínez Álvarez. Study supervision: all authors.

Portions of this work were presented in poster form at the Congress of Neurological Surgeons Annual Meeting, San Francisco, California, October 16–21, 2010, and at the European Association of Neuro-Oncology, Maastricht, the Netherlands, September 16–19, 2011.

References

  • 1

    Al-Mefty OTeixeira A: Complex tumors of the glomus jugulare: criteria, treatment, and outcome. J Neurosurg 97:135613662002

  • 2

    Bitaraf MAAlikhani MTahsili-Fahadan PMotiei-Langroudi RZahiri AAllahverdi M: Radiosurgery for glomus jugulare tumors: experience treating 16 patients in Iran. J Neurosurg 105:Suppl1681742006

  • 3

    Brewis CBottrill IDWharton SBMoffat DA: Metastases from glomus jugulare tumours. J Laryngol Otol 114:17232000

  • 4

    Eustacchio STrummer MUnger FSchröttner OSutter BPendl G: The role of Gamma Knife radiosurgery in the management of glomus jugular tumours. Acta Neurochir Suppl 84:91972002

  • 5

    Feigl GCHorstmann GA: Intracranial glomus jugulare tumors: volume reduction with Gamma Knife surgery. J Neurosurg 105:Suppl1611672006

  • 6

    Forbes JABrock AAGhiassi MThompson RCHaynes DSTsai BS: Jugulotympanic paragangliomas: 75 years of evolution in understanding. Neurosurg Focus 33:2E132012

  • 7

    Ganz JCAbdelkarim K: Glomus jugulare tumours: certain clinical and radiological aspects observed following Gamma Knife radiosurgery. Acta Neurochir (Wien) 151:4234262009

  • 8

    Genç ABicer AAbacioglu UPeker SPamir MNKilic T: Gamma knife radiosurgery for the treatment of glomus jugulare tumors. J Neurooncol 97:1011082010

  • 9

    Gerosa MVisca ARizzo PForoni RNicolato ABricolo A: Glomus jugulare tumors: the option of gamma knife radiosurgery. Neurosurgery 59:5615692006

  • 10

    Gottfried ONLiu JKCouldwell WT: Comparison of radiosurgery and conventional surgery for the treatment of glomus jugulare tumors. Neurosurg Focus 17:2E42004

  • 11

    Green JD JrBrackmann DENguyen CDArriaga MATelischi FFDe la Cruz A: Surgical management of previously untreated glomus jugulare tumors. Laryngoscope 104:9179211994

  • 12

    Gstoettner WMatula CHamzavi JKornfehl JCzerny C: Long-term results of different treatment modalities in 37 patients with glomus jugulare tumors. Eur Arch Otorhinolaryngol 256:3513551999

  • 13

    Guss ZDBatra SLi GChang SDParsa ATRigamonti D: Radiosurgery for glomus jugulare: history and recent progress. Neurosurg Focus 27:6E52009

  • 14

    Henzel MHamm KGross MWSurber GKleinert GFailing T: Fractionated stereotactic radiotherapy of glomus jugulare tumors. Local control, toxicity, symptomatology, and quality of life. Strahlenther Onkol 183:5575622007

  • 15

    Ivan MESughrue MEClark AJKane AJAranda DBarani IJ: A meta-analysis of tumor control rates and treatment-related morbidity for patients with glomus jugulare tumors. Clinical article. J Neurosurg 114:129913052011

  • 16

    Jackson CGMcGrew BMForest JANetterville JLHampf CFGlasscock ME III: Lateral skull base surgery for glomus tumors: long-term control. Otol Neurotol 22:3773822001

  • 17

    Jansen JCvan den Berg RKuiper Avan der Mey AGZwinderman AHCornelisse CJ: Estimation of growth rate in patients with head and neck paragangliomas influences the treatment proposal. Cancer 88:281128162000

  • 18

    Kemeny AA: Contemporary management of jugular paragangliomas (glomus tumours): microsurgery and radiosurgery. Acta Neurochir (Wien) 151:4194212009

  • 19

    Liscák RVladyka VWowra BKemeny AForster DBurzaco JA: Gamma Knife radiosurgery of the glomus jugulare tumour – early multicentre experience. Acta Neurochir (Wien) 141:114111461999

  • 20

    Miller JPSemaan MEinstein DMegerian CAMaciunas RJ: Staged Gamma Knife radiosurgery after tailored surgical resection: a novel treatment paradigm for glomus jugulare tumors. Stereotact Funct Neurosurg 87:31362009

  • 21

    Navarro Martín AMaitz AGrills ISBojrab DKartush JChen PY: Successful treatment of glomus jugulare tumours with gamma knife radiosurgery: clinical and physical aspects of management and review of the literature. Clin Transl Oncol 12:55622010

  • 22

    Neskey DMHatoum GModh RCivantos FTelischi FFAngeli SI: Outcomes after surgical resection of head and neck paragangliomas: a review of 61 patients. Skull Base 21:1711762011

  • 23

    Poznanovic SACass SPKavanagh BD: Short-term tumor control and acute toxicity after stereotactic radiosurgery for glomus jugulare tumors. Otolaryngol Head Neck Surg 134:4374422006

  • 24

    Sharma MSGupta AKale SSAgrawal DMahapatra AKSharma BS: Gamma knife radiosurgery for glomus jugulare tumors: therapeutic advantages of minimalism in the skull base. Neurol India 56:57612008

  • 25

    Sheehan JPKondziolka DFlickinger JLunsford LD: Gamma knife surgery for glomus jugulare tumors: an intermediate report on efficacy and safety. J Neurosurg 102:Suppl2412462005

  • 26

    Sheehan JPTanaka SLink MJPollock BEKondziolka DMathieu D: Gamma Knife surgery for the management of glomus tumors: a multicenter study. Clinical article. J Neurosurg 117:2462542012

  • 27

    Sheehan JPYen CPSteiner L: Gamma Knife surgery–induced meningioma. Report of two cases and review of the literature. J Neurosurg 105:3253292006

  • 28

    Springate SCWeichselbaum RR: Radiation or surgery for chemodectoma of the temporal bone: a review of local control and complications. Head Neck 12:3033071990

  • 29

    Varma ANathoo NNeyman GSuh JHRoss JPark J: Gamma knife radiosurgery for glomus jugulare tumors: volumetric analysis in 17 patients. Neurosurgery 59:103010362006

If the inline PDF is not rendering correctly, you can download the PDF file here.

Article Information

Address correspondence to: Maria Luisa Gandía González, M.D., Department of Gamma Knife Radiosurgery, Hospital Ruber Internacional, Calle La Masó, 38, Madrid 28034, Spain. email: marisagg4@hotmail.com.

Please include this information when citing this paper: published online June 13, 2014; DOI: 10.3171/2014.5.JNS131880.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Pie chart showing the tumor volume outcomes at the end of follow-up.

  • View in gallery

    Pie chart showing the clinical outcomes at the end of follow-up (tinnitus is not included).

  • View in gallery

    Kaplan-Meier curve for volumetric progression.

  • View in gallery

    Kaplan-Meier curve for clinical progression.

  • View in gallery

    Magnetic resonance images obtained in a patient with a large left-sided JTP. A: Axial and coronal images obtained at the time of GKRS treatment. B: Axial and coronal images obtained 72 months after treatment. C: Axial and coronal images obtained 112 months posttreatment.

References

  • 1

    Al-Mefty OTeixeira A: Complex tumors of the glomus jugulare: criteria, treatment, and outcome. J Neurosurg 97:135613662002

  • 2

    Bitaraf MAAlikhani MTahsili-Fahadan PMotiei-Langroudi RZahiri AAllahverdi M: Radiosurgery for glomus jugulare tumors: experience treating 16 patients in Iran. J Neurosurg 105:Suppl1681742006

  • 3

    Brewis CBottrill IDWharton SBMoffat DA: Metastases from glomus jugulare tumours. J Laryngol Otol 114:17232000

  • 4

    Eustacchio STrummer MUnger FSchröttner OSutter BPendl G: The role of Gamma Knife radiosurgery in the management of glomus jugular tumours. Acta Neurochir Suppl 84:91972002

  • 5

    Feigl GCHorstmann GA: Intracranial glomus jugulare tumors: volume reduction with Gamma Knife surgery. J Neurosurg 105:Suppl1611672006

  • 6

    Forbes JABrock AAGhiassi MThompson RCHaynes DSTsai BS: Jugulotympanic paragangliomas: 75 years of evolution in understanding. Neurosurg Focus 33:2E132012

  • 7

    Ganz JCAbdelkarim K: Glomus jugulare tumours: certain clinical and radiological aspects observed following Gamma Knife radiosurgery. Acta Neurochir (Wien) 151:4234262009

  • 8

    Genç ABicer AAbacioglu UPeker SPamir MNKilic T: Gamma knife radiosurgery for the treatment of glomus jugulare tumors. J Neurooncol 97:1011082010

  • 9

    Gerosa MVisca ARizzo PForoni RNicolato ABricolo A: Glomus jugulare tumors: the option of gamma knife radiosurgery. Neurosurgery 59:5615692006

  • 10

    Gottfried ONLiu JKCouldwell WT: Comparison of radiosurgery and conventional surgery for the treatment of glomus jugulare tumors. Neurosurg Focus 17:2E42004

  • 11

    Green JD JrBrackmann DENguyen CDArriaga MATelischi FFDe la Cruz A: Surgical management of previously untreated glomus jugulare tumors. Laryngoscope 104:9179211994

  • 12

    Gstoettner WMatula CHamzavi JKornfehl JCzerny C: Long-term results of different treatment modalities in 37 patients with glomus jugulare tumors. Eur Arch Otorhinolaryngol 256:3513551999

  • 13

    Guss ZDBatra SLi GChang SDParsa ATRigamonti D: Radiosurgery for glomus jugulare: history and recent progress. Neurosurg Focus 27:6E52009

  • 14

    Henzel MHamm KGross MWSurber GKleinert GFailing T: Fractionated stereotactic radiotherapy of glomus jugulare tumors. Local control, toxicity, symptomatology, and quality of life. Strahlenther Onkol 183:5575622007

  • 15

    Ivan MESughrue MEClark AJKane AJAranda DBarani IJ: A meta-analysis of tumor control rates and treatment-related morbidity for patients with glomus jugulare tumors. Clinical article. J Neurosurg 114:129913052011

  • 16

    Jackson CGMcGrew BMForest JANetterville JLHampf CFGlasscock ME III: Lateral skull base surgery for glomus tumors: long-term control. Otol Neurotol 22:3773822001

  • 17

    Jansen JCvan den Berg RKuiper Avan der Mey AGZwinderman AHCornelisse CJ: Estimation of growth rate in patients with head and neck paragangliomas influences the treatment proposal. Cancer 88:281128162000

  • 18

    Kemeny AA: Contemporary management of jugular paragangliomas (glomus tumours): microsurgery and radiosurgery. Acta Neurochir (Wien) 151:4194212009

  • 19

    Liscák RVladyka VWowra BKemeny AForster DBurzaco JA: Gamma Knife radiosurgery of the glomus jugulare tumour – early multicentre experience. Acta Neurochir (Wien) 141:114111461999

  • 20

    Miller JPSemaan MEinstein DMegerian CAMaciunas RJ: Staged Gamma Knife radiosurgery after tailored surgical resection: a novel treatment paradigm for glomus jugulare tumors. Stereotact Funct Neurosurg 87:31362009

  • 21

    Navarro Martín AMaitz AGrills ISBojrab DKartush JChen PY: Successful treatment of glomus jugulare tumours with gamma knife radiosurgery: clinical and physical aspects of management and review of the literature. Clin Transl Oncol 12:55622010

  • 22

    Neskey DMHatoum GModh RCivantos FTelischi FFAngeli SI: Outcomes after surgical resection of head and neck paragangliomas: a review of 61 patients. Skull Base 21:1711762011

  • 23

    Poznanovic SACass SPKavanagh BD: Short-term tumor control and acute toxicity after stereotactic radiosurgery for glomus jugulare tumors. Otolaryngol Head Neck Surg 134:4374422006

  • 24

    Sharma MSGupta AKale SSAgrawal DMahapatra AKSharma BS: Gamma knife radiosurgery for glomus jugulare tumors: therapeutic advantages of minimalism in the skull base. Neurol India 56:57612008

  • 25

    Sheehan JPKondziolka DFlickinger JLunsford LD: Gamma knife surgery for glomus jugulare tumors: an intermediate report on efficacy and safety. J Neurosurg 102:Suppl2412462005

  • 26

    Sheehan JPTanaka SLink MJPollock BEKondziolka DMathieu D: Gamma Knife surgery for the management of glomus tumors: a multicenter study. Clinical article. J Neurosurg 117:2462542012

  • 27

    Sheehan JPYen CPSteiner L: Gamma Knife surgery–induced meningioma. Report of two cases and review of the literature. J Neurosurg 105:3253292006

  • 28

    Springate SCWeichselbaum RR: Radiation or surgery for chemodectoma of the temporal bone: a review of local control and complications. Head Neck 12:3033071990

  • 29

    Varma ANathoo NNeyman GSuh JHRoss JPark J: Gamma knife radiosurgery for glomus jugulare tumors: volumetric analysis in 17 patients. Neurosurgery 59:103010362006

TrendMD

Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 0 0 0
Full Text Views 425 425 21
PDF Downloads 145 145 11
EPUB Downloads 0 0 0

PubMed

Google Scholar