Tumor progression in tympanojugular paragangliomas: the role of radiotherapy and wait and scan.

INTRODUCTION: Tympanojugular paragangliomas (TJ PGLs) are rare tumors characterized by bone infiltration and erosion and a close relationship with critical structures, such as cranial nerves and internal carotid artery. For these reasons, their management represents a tough challenge. Since the fifties, radio-therapy (RT) has been proposed as an alternative treatment aimed at avoiding tumor progression. However, the indolent nature of the tumor, characterized by slow growth, is a crucial factor that needs to be considered before offering radiation. METHODS: This study aims to examine tumor progression in RT patients through a systematic review of the literature and in TJ PGL patients who underwent solely wait and scan at our department. RESULTS: The rate of tumor progression in the RT group was 8.9%, while in the wait and scan cohort was 12.9%. This data suggests the innate slow growth of PGLs. However, it is not possible to draw certain conclusions because of the wide heterogeneity of the studies. CONCLUSION: When complete surgical excision of TJ PGLs is not feasible, appropriate counseling and patient selection, including comprehensive tumor classification, should be performed before proposing RT to control tumor progression, since wait and scan may represent a reasonable option in selected cases.

Stereotactic Radiosurgery for Glomus Jugulare Tumors: Systematic Review and Meta-Analysis.

BACKGROUND: Glomus jugulare tumors (GJTs) are benign paragangliomas of the jugular foramen. Traditional management of these tumors involves surgical resection; however, considering the proximity of these tumors to important neurovasculature, stereotactic radiosurgery (SRS) may be an appropriate noninvasive treatment to consider. The aim of this meta-analysis was to evaluate SRS as a treatment option for GJTs. METHODS: An online search using PubMed, Web of Science, Scopus, and Cochrane databases was performed in March 2019 for articles on radiosurgery treatment of GJTs. The screening process followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. RESULTS: The final analysis comprised 23 studies including 460 patients. Average rates of tinnitus, hearing loss, and lower cranial nerve deficit as presenting symptoms were 56% (95% confidence interval [CI], 46%-66%), 56% (95% CI, 44%-68%), and 42% (95% CI, 31%-54%), respectively. Overall clinical status improvement rate after treatment was 47% (95% CI, 37%-57%). Rates of tinnitus, hearing loss, and lower cranial nerve improvement after treatment were 54% (95% CI, 44%-63%), 28% (95% CI, 19%-40%), and 22% (95% CI, 11%-39%), respectively. The mean follow-up time across studies was 47 months (range, 4-268 months). The aggregate tumor control rate at the time of follow-up was 95% (95% CI, 93%-97%). CONCLUSIONS: The tumor control rate of 95% and 47% symptomatic improvement suggest that SRS may be a suitable treatment modality for these hypervascular skull base tumors. Future studies are warranted to further evaluate the potential role of SRS in management of GJTs.

Image-guided robotic radiosurgery for glomus jugulare tumors-Multicenter experience and review of the literature.

BACKGROUND: Glomus jugulare tumors (GJTs) are challenging to treat due to their vascularization and location. This analysis evaluates the effectiveness and safety of image-guided robotic radiosurgery (RRS) for GJTs in a multicenter study and reviews the existing radiosurgical literature. METHODS: We analyzed outcome data from 101 patients to evaluate local control (LC), changes in pretreatment deficits, and toxicity. Moreover, radiosurgical studies for GJTs have been reviewed. RESULTS: After a median follow-up of 35 months, the overall LC was 99%. Eighty-eight patients were treated with a single dose, 13 received up to 5 fractions. The median tumor volume was 5.6 cc; the median treatment dose for single-session treatments is 16 Gy, and for multisession treatments is 21 Gy. Fifty-six percentage of patients experienced symptom improvement or recovered entirely. CONCLUSIONS: RRS is an effective primary and secondary treatment option for GJTs. The available literature suggests that radiosurgery is a treatment option for most GJTs.

Paraganglioma yugular bilateral: reporte de un caso y revisión de la literatura / Bilateral jugular paraganglioma: case report and review of the literature

Resumen El paraganglioma (PG) es una neoplasia infrecuente originada de las células paraganglionares, embriológicamente derivadas de la cresta neural. Se localizan en la cabeza, base de cráneo, cuello, mediastino, abdomen y pelvis. La mayor parte de los PG muestran un curso clínico benigno, sin embargo, algunos casos pueden mostrar un comportamiento biológico agresivo con invasión local y metástasis a distancia. Un avance significativo en patología molecular ha sido el reconocimiento que el 30%-40% de estas neoplasias presentan alteraciones genéticas. Se han descrito más de 45 genes involucrados, incluyendo mutaciones de la línea germinal succinato deshidrogenasa. Actualmente se recomienda hacer test genético a todos los portadores de PG incluyendo los de presentación esporádica. El PG más frecuente se ubica en la glándula suprarrenal llamado feocromocitoma. El diagnóstico definitivo se realiza con histología, sin embargo, el estudio imagenológico puede entregar una aproximación diagnóstica certera. Debido a la aceptación actual que todos los PG tienen potencial metastásico, el concepto de PG benigno y maligno ha cambiado a uno de estimación de riesgo de metástasis, aunque no existe un esquema único aceptado para tal efecto. El tratamiento considera la cirugía, la radioterapia, la observación y terapias combinadas. Dado el lento crecimiento de este tipo de neoplasia y las potenciales complicaciones de la terapia quirúrgica, la observación es una opción especialmente para pacientes añosos dejando las otras opciones para pacientes más jóvenes. En este trabajo se presenta un caso de paraganglioma yugular bilateral gigante tratado con radioterapia de intensidad modulada incluyendo una revisión bibliográfica pertinente.

Abstract Paraganglioma (PG) is a rare neoplasm derived from paraganglionic cells of the neural crest. They are located in the head, skull base, neck, mediastinum, abdomen and pelvis. Most PGs show a benign clinical course, however, some cases may show aggressive biological behavior with local invasion and distant metastasis. A significant advance in molecular pathology has been the recognition that 30%-40% of these neoplasms present genetic alterations; more than 45 genes have been described, including mutations of the germline succinate dehydrogenase. Currently it is recommended to make genetic test to all patients with PG, including sporadic presentation. The most frequent PG is located in the adrenal gland called pheochromocytoma. The definitive diagnosis is made with histology; however, the imaging study can provide an accurate diagnostic approach. It is now accepted that all PG have a metastatic potential, therefore the concept of benign or malignant has been changed to a metastasis risk stratification approach however no single scheme is been widely used. The treatment considers surgery, radiotherapy, observation and combination therapies. Given the slow growth of this type of neoplasia and the potential complications of surgical therapy, observation is an option especially for elderly patients leaving the other options for younger patients. In this work we present a case of giant bilateral jugular paraganglioma treated with intensity modulated radiation therapy, including a pertinent literature review.

LINAC radiosurgery for glomus jugulare tumors: retrospective - cohort study of 23 patients.

BACKGROUND: Glomus jugulare tumors (GJTs) are uncommon and locally disruptive tumors that usually arise within the jugular foramen of the temporal bone. Surgery was the treatment of choice up until recently. In the last decades, however, radiosurgery has surfaced as a promising alternative treatment by providing excellent tumor control with low risk of cranial nerve injuries. Our aim was to examine the results of radiosurgery specifically, linear accelerator stereotactic radiosurgery (LINAC SRS) for GJT treatment. We hypothesized that radiosurgery will reduce the size of the tumor and improve neurological symptoms. DESIGN AND METHOD: Between January 1, 1994 and December 31, 2013, 30 patients with GJTs were treated in Sheba Medical Center using LINAC SRS treatment. Comprehensive clinical follow-up was available for 23 patients. Sixteen patients were female and seven males with a median age of 64 years, with a range of 18-87 years. In 19 of the patients, LINAC SRS was the primary treatment, whereas in the remaining four cases, surgery or embolization preceded radiosurgery. The median treated dose to tumor margin was 14 Gy (range 12-27 Gy), and the median tumor volume before treatment was 5 ml (range 0.5-15 ml). RESULTS: Following the LINAC SRS treatment, 14 of 23 patients (60%) showed improvement of previous neurological deficits, nine patients (40%) remained unchanged. At the end of a follow-up, tumor reduction was seen in 13 patients and a stable volume in eight (91% tumor control rate). Two cases of tumor progression were noted. Three patients (13%) had post- SRS complications during the follow-up, two of which achieved tumor control, while in one the tumor advanced. CONCLUSIONS: LINAC SRS is a practical treatment option for GJTs, with a high rate of tumor control and satisfactory neurological improvement.

Safety and Efficacy of Primary Multisession Dose Fractionated Gamma Knife Radiosurgery for Jugular Paragangliomas.

BACKGROUND: While multisession dose fractionated Gamma Knife radiosurgery (DFGKS) is common, its use has never been described for jugular paragangliomas (JP), which are notoriously difficult to treat. OBJECTIVE: To define efficacy, safety, and complication profile of DFGKS in 2 or 3 consecutive sessions for the treatment of a cohort of 10 cases of JP. METHODS: Between 2012 and 2017, 10 patients with JP were treated with DFGKS in 2 or 3 sessions, because it was not safe to treat the lesion in a single session because of the large volume or proximity to organs at risk. The small to medium-sized JP are treated with 16-22 Gy radiation, but the large-volume JP were treated with 23-25 Gy radiation dose. The Leksell G frame was kept in situ during the whole procedure. The tumor volumes on pretreatment and posttreatment imaging were compared, using the Leksell Gamma Plan treatment plan software to assess tumor progression. The patients were regularly evaluated for their clinical outcome with radiologic correlation. RESULTS: The mean radiologic follow-up was 39 months (range, 12-78 months). The mean marginal dose for 3 fractions and 2 fractions was 7.64 Gy at 50% and 11.2 Gy at 50%, respectively. The mean tumor size was 29.9 cm3 (range, 9.95-47.63 cm3) at treatment and 21.9 cm3 (range, 8.83-37.5 cm3) at follow-up (suggestive of 26.7% reduction). Tumor control was achieved in all patients (100%). Of 110 potential neurologic problems (signs/symptoms) evaluated (11 in each patient), 56 (50.9%) were present preoperatively. Of them, 27 (48.2%) improved and 29 (51.8%) stabilized after treatment. There were 2 new-onset neurologic problems (of 110, 1.8%) attributable to treatment (new-onset headache and spinal accessory paresis). No patient had any permanent neurologic deterioration. CONCLUSIONS: DFGKS for large-volume JP leads to acceptable progression-free survival, tumor control rate, and symptomatic improvement. It may be preferred to surgery or fractionated radiotherapy given its better safety, efficacy, and complication profile.

Quality of Life in Jugular Paraganglioma Following Radiosurgery.

OBJECTIVE: The treatment paradigm for jugular paraganglioma (JP) has changed considerably over time with the wider adoption of stereotactic radiosurgery (SRS). To the best of the authors' knowledge, there are no published studies that use validated patient-reported outcome measures to ascertain quality of life (QoL) outcomes following SRS for JP when used in single or combined modality treatment regimens. STUDY DESIGN: Cross-sectional survey. SETTING: Tertiary referral center. PATIENTS: Adult patients with JP treated with primary SRS or SRS following primary surgery between 1990 and 2017. INTERVENTIONS(S): Surgery and/or Gamma Knife SRS. MAIN OUTCOME MEASURES: Global and treatment-related QoL and differences in QoL based on treatment approach. RESULTS: Sixty-nine surveys were distributed and a total of 26 completed surveys were received (38% response rate). Among respondents, the median age at SRS was 53 years and 16 of the 26 patients (62%) were female. Median follow-up was 97 months. Nineteen patients (73%) were treated with primary SRS or staged SRS following intentional subtotal resection (STR; hereafter referred to as "staged SRS"), while the remainder (n = 7, 27%) were treated with SRS for recurrent JP. Median physical and mental health QoL PROMIS-10 T-scores regardless of treatment strategy were 39.8 and 38.8, respectively, while median SF36 physical and mental component subscores were similar to national averages and non-tumor controls. When comparing general physical and mental health QoL scores, there was no significant difference between patients treated with primary or staged SRS and those treated with SRS for recurrent JP. However, age-adjusted swallowing function among patients treated with primary or staged SRS was better than in those patients treated with SRS for recurrent JP (p = 0.05). CONCLUSIONS: Patients treated with primary or staged SRS for JP tend to exhibit better swallowing outcomes than those treated with SRS for recurrent JP. However, the majority of overall and disease-specific quality of life measures were not different between groups. Based on the low incidence of new cranial neuropathy following SRS, it is likely that initial surgical morbidity is the primary contributor to this outcome. Disease-specific overall quality of life measures, akin to those already used for other benign skull base tumors, are necessary to better gauge physical and mental health outcomes following treatment for JP. Though limited by small sample size, this represents the first study to gauge QoL outcomes following treatment for JP.

Jugular foramen tumour resulting in hypoglossal denervation pseudohypertrophy: a rare and significant cause for tongue asymmetry.

Paragangliomas of the jugular foramen are rare. They may present with symptoms of compression of the glossopharyngeal or vagus nerves, or due to secretion of catecholamines from chromaffin cells within the tumour. This case describes a rare presentation of glomus tumour. A 67-year-old patient presented with a 2-month history of right-sided tongue swelling. She was found to have an obvious swelling on the right side of the tongue but no obvious weakness or fasciculation on initial examination. Ultrasound confirmed diffuse muscle swelling, but no lesion within the tongue. Magnetic resonance imaging of the neck revealed an ipsilateral glomus jugulare tumour that extended to the hypoglossal canal, and had resulted in ipsilateral denervation pseudohypertrophy of the lingual muscles. This paper reviews presentation of glomus jugulare tumours and contributes a novel presentation of a rare entity.

Salvage Radiosurgery After Subtotal Resection for Catecholamine-secreting Jugular Paragangliomas: Report of Two Cases and Review of the Literature.

OBJECTIVES: Catecholamine-secreting jugular paragangliomas (JPs) represent a rare subset of head and neck paragangliomas that may present with hypertension, arrhythmia, or syncopal episodes. Subtotal resection to protect critical neurovascular structures may result in persistent catecholamine excess from residual tumor. Herein, we report our experience with stereotactic radiosurgery (SRS) for salvage treatment of catecholamine-secreting JP following subtotal microsurgical resection. PATIENTS: Adult patients treated with SRS after subtotal microsurgical resection of catecholamine-secreting JP. INTERVENTIONS: SRS. MAIN OUTCOME MEASURES: Post-treatment catecholamine and metanephrine levels, clinical outcomes, and tumor control. RESULTS: Of 85 patients with JPs treated with primary or salvage radiosurgery between 1990 and 2017, 2 (2%) harbored nonmalignant secreting tumors. Patient 1 developed catecholamine excess with elevated norepinephrine (NE) at 475 mcg/24 hours (normal < 80 mcg/24 h). Following subtotal resection, she developed catecholamine excess with radiographic evidence of tumor growth and therefore underwent SRS. Three years post-SRS and beyond, catecholamine levels remained normalized (NE 62 mcg/24 h at 10 yr) and tumor volume remained stable on serial MRI studies over the 17-year follow-up period.Patient 2 developed symptomatic arrhythmia and was found to have a JP. Urine norepinephrine (NE) was elevated at 213 mcg/24 hours. She underwent nerve-sparing subtotal resection and upfront SRS was used to treat residual tumor. Twenty months following SRS, the area of residual JP had not enlarged in size and catecholamine levels remained normal (NE 46 mcg/24 h). CONCLUSIONS: Radiosurgery can be used in salvage treatment of catecholamine-secreting JP, providing durable tumor control and resolution of catecholamine excess. For patients with large catecholamine-secreting JP and normal lower cranial nerve function, aggressive nerve-sparing subtotal resection with adjuvant radiosurgery may offer a low-morbidity alternative to gross total resection. Further study of this subset of patients is warranted to substantiate these promising, yet preliminary findings.

Temporary noncicatricial focal alopecia following Gamma knife radiosurgery: Case series and review of literature.

OBJECTIVE: The aim of this study is to report the pattern, timing, responsible radiation parameters, and dosimetric results on the outcome of alopecia following gamma knife radiosurgery (GKRS) for various intracranial pathologies. A literature review of radiation-induced alopecia and observation of this complication with GKRS are also included. MATERIALS AND METHODS: The authors report 6 cases of GKRS-induced focal temporary alopecia without dermal fibrosis or clinical scarring and with no long-term squeal. RESULTS: In all the cases, the scalp received ≥3 Gy radiation exposure. Post GKRS alopecia is a temporary, noncicatricial, focal, reversible phenomenon observed within 2 weeks of the treatment. This acute complication is dose-dependent and reflects damage to the hair follicle. It is only seen with treated volumes in the superficial location. Hair regrowth occurs within two months with no long-term complications and change in hair quality being noted. CONCLUSION: Compared to whole brain radiotherapy, chances of alopecia are significantly less after GKRS. Despite a sharp dose fallout, a single fraction high-dose radiosurgery with GKRS may expose the skin appendages to more than 3 Gy radiation exposures, which may injure the hair follicles irrespective of the growth phase (anagen, telogen, or catagen) they are in. A careful planning that includes sparing of the dermal appendages and hair roots up to 4-6 mm depth in the skin may prevent this complication. Patients with superficial lesions should be well informed about the reversible nature of this possible complication, with near-normal hair growth being established within 2 months of treatment.

Outcomes of Primary Radiosurgery Treatment of Glomus Jugulare Tumors: Systematic Review With Meta-analysis.

OBJECTIVE: 1) Determine tumor control, symptomatic control, and complication rates of primary radiosurgery (PRS) for treatment of glomus jugulare tumors (GJTs) via systematic review and meta-analysis. 2) Identify risk factors for these outcomes. DATA SOURCES: 1) Search of English articles in PubMed, Web of Science, Cochrane, and EBSCOhost databases from January 1950 to August 2017. STUDY SELECTION: Inclusion criteria: 1) treated GJT patients who had no previous treatment with radiosurgery, 2) follow-up with magnetic resonance imaging for at least 12 months, 3) reported pre and posttreatment symptoms, tumor control, or complications for individual PRS patients or for PRS patients as a cohort. DATA EXTRACTION: The following were extracted: number of patients, level of evidence, mean age, mean pretreatment tumor volume, tumor control rate, criteria for change in tumor size, symptom control rate, and complications. At the individual patient level the following were extracted: age, number of treatment fractions, total radiation dose to tumor margin, pretreatment tumor volume, Fisch or Glasscock-Jackson stage, pre- and posttreatment symptoms, tumor control, symptom control/improvement, length of follow-up, and complications. DATA SYNTHESIS: Fifteen studies encompassing 91 patients met criteria. Tumor control was achieved in 92% of patients, symptom control in 93%, and complications occurred in 8%. There was one major complication. Smaller tumor volume predicted improvement in symptoms with PRS. CONCLUSIONS: In the short term, PRS is safe and effective at controlling growth and clinical symptoms for patients with GJTs, though there exists significant selection bias, inconsistent reporting, and clinical heterogeneity among existing studies.

Long-term outcomes after radiosurgery for glomus jugulare tumors.

AIMS AND BACKGROUND: The treatment of glomus jugulare tumors (GJT) remains controversial due to high morbidity. Historically, these tumors have primarily been managed surgically. The purpose of this retrospective review was to assess the tumor and clinical control rates as well as long-term toxicity of GJT treated with radiosurgery. METHODS: Between 1993 and 2014, 30 patients with GJT (31 tumors) were managed with radiosurgery. Twenty-one patients were female and the median age was 59 years. Twenty-eight patients (93%) were treated with radiosurgery, typically at 14 Gy ( n = 26), and 2 patients (7%) with stereotactic radiosurgery. Sixteen cases (52%) had undergone prior surgery. RESULTS: The mean follow-up was 4.6 years (range 1.5-12). Crude overall survival, tumor control, clinical control, and long-term grade 1 toxicity rates were 97%, 97%, 97%, and 13% (4/30), respectively. No statistically significant risk factor was associated with lower tumor control in our series. Univariate analysis showed a statistically significant association between patients having 1 cranial nerve (CN) involvement before radiosurgery and a higher risk of lack of improvement of symptoms (odds ratio 5.24, 95% confidence interval 1.06-25.97, p = .043). CONCLUSIONS: Radiosurgery is an effective and safe treatment modality for GJT. Patients having 1 CN involvement before radiosurgery show a higher risk of lack of improvement of symptoms.

Long-Term Outcome Following Stereotactic Radiosurgery for Glomus Jugulare Tumors: A Single Institution Experience of 20 Years.

BACKGROUND: Glomus jugulare tumors (GJTs) are rare benign tumors, which pose significant treatment challenges due to proximity to critical structures. OBJECTIVE: To evaluate the long-term clinical and radiological outcome in patients undergoing stereotactic radiosurgery (SRS) for GJTs through retrospective study. METHODS: Forty-two patients with 43 GJTs were treated using Gamma Knife radiosurgery (GKRS; Elekta AB, Stockholm, Sweden) at our institute from 1997 to 2016. Clinical, imaging, and radiosurgery data were collected from an institutional review board approved database. RESULTS: Most patients were females (n = 35, 83.3%) and median age was 61 yr (range 23-88 yr). Median tumor volume and diameter were 5 cc and 3 cm, respectively, with a median follow-up of 62.3 mo (3.4-218.6 mo). Overall, 20 patients (47.6%) improved clinically and 14 (33.3%) remained unchanged at last follow-up. New onset or worsening of hearing loss was noted in 6 patients (17.2%) after SRS. The median prescription dose to the tumor margin was 15 Gy (12-18 Gy). Median reduction in tumor volume and maximum tumor diameter at last follow-up was 33.3% and 11.54%, respectively. The 5-yr and 10-yr tumor control rates were 87% ± 6% and 69% ± 13%, respectively. There was no correlation between maximum or mean dose to the internal acoustic canal and post-GK hearing loss (P > .05). CONCLUSION: SRS is safe and effective in patients with GJTs and results in durable, long-term control. SRS has lower morbidity than that associated with surgical resection, particularly lower cranial nerve dysfunction, and can be a first-line management option in these patients.

Gamma Knife radiosurgery for glomus jugulare tumors: a single-center series of 75 cases.

OBJECTIVE Glomus jugulare tumors are rare indolent tumors that frequently involve the lower cranial nerves (CNs). Complete resection can be difficult and associated with lower CN injury. Gamma Knife radiosurgery (GKRS) has established its role as a noninvasive alternative treatment option for these often formidable lesions. The authors aimed to review their experience at the National Centre for Stereotactic Radiosurgery, Sheffield, United Kingdom, specifically the long-term tumor control rate and complications of GKRS for these lesions. METHODS Clinical and radiological data were retrospectively reviewed for patients treated between March 1994 and December 2010. Data were available for 75 patients harboring 76 tumors. The tumors in 3 patients were treated in 2 stages. Familial and/or hereditary history was noted in 12 patients, 2 of whom had catecholamine-secreting and/or active tumors. Gamma Knife radiosurgery was the primary treatment modality in 47 patients (63%). The median age at the time of treatment was 55 years. The median tumor volume was 7 cm3, and the median radiosurgical dose to the tumor margin was 18 Gy (range 12-25 Gy). The median duration of radiological follow-up was 51.5 months (range 12-230 months), and the median clinical follow-up was 38.5 months (range 6-223 months). RESULTS The overall tumor control rate was 93.4% with low CN morbidity. Improvement of preexisting deficits was noted in 15 patients (20%). A stationary clinical course and no progression of symptoms were noted in 48 patients (64%). Twelve patients (16%) had new symptoms or progression of their preexisting symptoms. The Kaplan-Meier actuarial tumor control rate was 92.2% at 5 years and 86.3% at 10 years. CONCLUSIONS Gamma Knife radiosurgery offers a risk-versus-benefit treatment option with very low CN morbidity and stable long-term results.

Systemic Inflammatory Syndrome Associated with a Case of Jugular Paraganglioma.

Jugular paraganlioma is a benign, slow-growing tumor originating from the paraganglion cells and it is associated with catecholamine secretion. Paragangliomas can secrete Interleukin-6 (IL-6) and present as a systemic inflammatory syndrome; these characteristics have not been previously associated with jugular paragangliomas. A 63-year-old man with a jugular tumor in the skull base was referred to our hospital for an evaluation of pyrexia, back pain, and acute inflammation. His serum IL-6 level was elevated on admission and it decreased after radiotherapy. This is the first known case of a jugular paraganglioma exhibiting systemic inflammatory syndrome.

Treatment Outcomes for Single Modality Management of Glomus Jugulare Tumors With Stereotactic Radiosurgery.

OBJECTIVES: The objectives were to evaluate the audiological outcomes, response of symptoms, and response of tumor volume in patients with glomus jugulare tumors treated solely with single fraction gamma knife radiosurgery. STUDY DESIGN: Single institution retrospective review. SETTING: Academic, tertiary referral center. PATIENTS: The diagnosis code for glomus jugulare was used to identify patients. Only those who underwent gamma knife radiosurgery were included. Those previously treated with any modality were excluded. A total of 12 patients were included for the tumor response and symptom response data and 7 of those were included in the audiometric analysis. MAIN OUTCOMES MEASURES: Audiometric data at most recent follow-up compared with presentation, subjective improvement in pulsatile tinnitus, and change in tumor volume at most recent follow-up compared with pretreatment. RESULTS: The average time to most recent follow-up was 27.6 months. There was no significant change in pure-tone average or word recognition. Pulsatile tinnitus completely resolved or improved in 80% of patients. Cranial neuropathies were stable or improved. A single patient experienced facial nerve paresis 2 years after treatment, which resolved with steroid treatment. Tumor control was 100% and the average change in tumor volume was a decrease of 37%. CONCLUSION: Single modality gamma knife radiosurgery treatment of glomus jugulare tumors seems to be safe. Treatment results in decreased tumor volume and improved pulsatile tinnitus in most patients. There was no significant progression of hearing loss after treatment. Lower cranial nerve function remains stable in all patients.

Strategies and long-term outcomes in the surgical management of tympanojugular paragangliomas.

BACKGROUND: The purpose of this study was to share our review of surgical strategies and long-term outcomes in the management of tympanojugular paragangliomas. METHODS: This was a retrospective study with a literature review. The records of 184 patients with 185 tympanojugular paragangliomas were analyzed for tumor class, surgical procedure, preoperative vascular management, and perioperative sequelae. RESULTS: Of class C1, C2, C3, and C4 tumors, we found 46 (24.9%), 95 (51.3%), 41 (22.2%), and 3 (1.6%), respectively. One hundred four (56.2%) tumors had intracranial extensions and 8 (4.3%) involved the vertebral artery. A single-stage procedure was adopted in 158 (85.4%) tumors. The infratemporal fossa type A approach was used in all cases. In 17 patients (9.7%), an intra-arterial stenting of the internal carotid artery was performed. Gross-total tumor removal was achieved in 166 cases (89.7%) and 4 (2.4%) among them developed a recurrence. CONCLUSION: A thorough understanding of skull base techniques and a logical decision-making process in the management of tympanojugular paragangliomas can achieve a high rate of success in terms of recurrences and complications. © 2015 Wiley Periodicals, Inc. Head Neck 38: 871-885, 2016.