Efficacy and Safety of Preoperative Embolization in Glomus Jugulare Tumors: A Systematic Review and Meta-analysis of Clinical Outcomes and Complications.

PURPOSE: This study aims to provide a comprehensive review of the clinical benefits, complications, and safety profile associated with preoperative embolization in Glomus jugulare tumors (GJTs). MATERIALS AND METHODS: A comprehensive search in PubMed, Embase, and Web of Science was conducted for English articles published up to March 2023, focusing on GJTs and preoperative embolization. Included studies involved patients over 18 with GJTs. We excluded studies that explored embolization methods other than the standard endovascular approach, as well as studies involving paragangliomas that did not provide specific data related to GJTs. Key variables such as hemorrhage volume and surgical time, as well as clinical outcomes, were analyzed. Data were analyzed using a random-effects model meta-analysis, assessing heterogeneity with the I2 statistic. RESULTS: This review encompasses 19 studies with a total of 328 patients. The studies incorporated into our meta-analysis display considerable differences and inconsistencies in their data. The findings of the meta-analysis show a mean hemorrhage volume of 636 ml (95% confidence interval (CI) 473-799) following preoperative embolization, and a mean surgical duration of 487 min (95% CI 350-624). The study also notes potential complications: facial nerve deficits occurred in 20% of cases (95% CI 11-32%), and vagal nerve deficits in 22% (95% CI 13-31%). CONCLUSION: This study suggests that preoperative embolization could decrease surgery duration and blood loss, but emphasizes the importance of evaluating risks like nerve damage. However, the generalizability of these findings is restricted due to the diversity of available data.

Metastatic Malignant Glomus Jugulare Tumor: A Rare Case Report with Clinical Manifestations and Treatment Approach.

BACKGROUND Paragangliomas are neuroendocrine neoplasms derived from paraganglia of the sympathetic and parasympathetic nervous systems. Parasympathetic ganglia-derived tumors, also called non-chromaffin, are located almost exclusively in the neck and skull base and are usually non-secretory and inactive. A case of malignant glomus jugulare with a metastatic cervical lymph node is described here. CASE REPORT A 24-year-old woman was referred to an otolaryngology clinic for concern of voice change for 1 month, which was associated with right progressive hearing loss, pulsating tinnitus, and right facial weakness. A clinical examination revealed a reddish mass in the right ear behind an intact tympanic membrane with right facial weakness of House-Brackmann grade VI. A bedside flexible nasopharyngoscopy revealed an immobile right vocal fold. A computed tomography scan of the brain revealed a destructive lesion within the right jugular foramen. The patient underwent embolization followed by glomus tumor resection via infra-temporal fossa with Fisch type A approach. Pathology revealed that the tumor was an infiltrative epithelioid tumor with a spindle and nesting pattern separated by fibrovascular stroma. The submitted lateral neck lymph node revealed a metastatic tumor. CONCLUSIONS Glomus jugulare tumors are uncommon paragangliomas, and malignant behavior with metastasis is extremely rare. Metastatic tumors are often associated with facial and vagal nerves palsy. There are no histological features that distinguish malignant glomus jugulare tumors. Malignant neoplasms are characterized by the presence of metastases. Tumors of the glomus jugulare that are malignant are treated with surgery, radiotherapy, or both. However, our search of the literature revealed no clear guidelines, given the scarcity of cases. Moreover, the presence of metastasis increases the risk of death.

Trends and Impact of Treatment Modalities (Surgery and Radiation Therapy) on Health Care Utilization in Patients With Glomus Jugulare Tumors (GJTs): An Inverse Probability of Treatment Weight Analysis.

OBJECTIVES: The trend of practice pattern and impact on health care utilization for surgery and radiation therapy (RT) in patients with glomus jugulare tumors (GJTs) is not well defined. METHODS: The IBM (Armonk, NY) MarketScan database was queried using the ICD-9/10 and CPT 4th edition, 2000-2020. We included patients ≥18 years of age who underwent either surgery or RT with at-least 1-year follow-up. We compared the health care utilization at 3-month, 6-month, and 1-year follow up using the inverse probability of treatment weight technique. RESULTS: A cohort of 333 patients was identified. Of these, 72.7% (n = 242) underwent RT and 27.3% (n = 91) underwent surgery. RT use increased from 2002-2004 (50%) to 2017-2019 (91%). Patients in the surgery cohort were younger (median age 49 vs. 56 years, P < 0.0001) and had a higher 3+ comorbidity index (34% vs. 30%, P = 0.43) compared with patients in the RT cohort. Patients who underwent surgery had higher complications at index hospitalization (22% vs. 6%, P < 0.0001) and at 30 days (14% vs. 5%, P = 0.0042). No difference in combined index and 6- or 12-month payments were noted (6-months: surgery, $66m108, RT: $43m509, P = 0.1034; 12-months: surgery, $73,259, RT: $51,576, P = 0.1817). Only 4% of patients who had initial RT underwent RT and none underwent surgery at 12 months, whereas 6% of patients who had initial surgery underwent RT and 2% underwent surgery at 12 months. CONCLUSIONS: RT plays an increasingly important role in the treatment for patients with GJTs, with fewer complications and a comparable health care utilization at 1 year.

Metastatic paraganglioma.

A man in his 70s, with a history of a glomus jugulare paraganglioma diagnosed 18 years ago, presented with an unprovoked deep vein thrombosis (DVT). The paraganglioma had been treated by radiotherapy, and yearly scans had not shown any progression since treatment. A sclerotic focus in L4 vertebral body was reported on a CT scan of the neck and trunk which was done to exclude a neoplastic process being the precipitating factor for the DVT. Nuclear imaging showed multiple areas of bony uptake, suggestive of metastases. A bone biopsy of the left femur resulted positive for metastatic paraganglioma. A monthly intramuscular injection of octreotide 30 mg was prescribed.

Management of Complex Jugular Paragangliomas: Surgical Resection and Outcomes.

BACKGROUND: This study aimed to review tumor control and cranial nerve function outcomes in patients with complex jugular paragangliomas and to refine the surgical strategies for complex jugular paragangliomas. METHODS: We describe our experience with 12 patients with complex jugular paragangliomas diagnosed in our institution from January 2013 to June 2020. The main outcomes included tumor control, complications, and function of facial nerve and lower cranial nerves, postoperatively. RESULTS: Gross-total resection was achieved for 9 (75%) patients, and subtotal resection was achieved for 3 (25%) patients. The surgical tumor control rate was 100% after a mean follow-up of 45.5 months (range, 13-111 months). Postoperatively, 10 patients (83.3%) obtained unchanged or improved facial nerve function. However, new lower cranial nerve deficits occurred in 2 patients (16.7%) due to surgical removal of the concurrent vagal paraganglioma and scar tissue enclosing the IX and XII nerves. CONCLUSION: Our refined surgical techniques, including tension-free anterior facial nerve rerouting, sigmoid sinus tunnel-packing, and pushpacking techniques, could be a choice for the treatment of complex jugular paragangliomas to achieve tumor control and cranial nerves preservation. A 2-stage surgery should be applied to minimize the risk of bilateral cranial neuropathies and the influence on cerebral circulation in patients with bilateral paragangliomas. The preoperative endovascular intervention such as coil embolization or internal carotid artery stenting can be employed for the management of paragangliomas with internal carotid artery-associated lesions.

Surgical treatment of selected tumors via the navigated minimally invasive presigmoidal suprabulbar infralabyrinthine approach without rerouting of the facial nerve.

The feasibility of a novel skull base approach - the navigated minimally invasive presigmoidal suprabulbar infralabyrinthine approach (NaMIPSI-A) without rerouting of the fallopian canal for selected jugular foramen tumors (JFTs) - has been demonstrated in a neuroanatomical laboratory study. Here, we present our clinical experience with the NaMIPSI-A for selected JFTs, with a particular focus on its efficacy and safety. All patients with JFTs who were treated via the NaMIPSI-A were included in this study. The JFTs were classified according to a modified Fisch classification. The neurological and neuroradiological outcome, the extent of tumor resection, and the approach-related morbidity were examined. Five patients (two women, three men; mean age 57 years, range 48-65) were available. According to the modified Fisch classification, two JFTs were graded as C1, one as De1, and two as De2. Gross total resection (GTR) was achieved in three patients and near-total resection (NTR) in two. Postsurgically, no new neurological deficits and no approach-related morbidity and mortality occurred. One case with a postoperative cerebrospinal fluid leak was managed successfully with lumbar drainage. During the follow-up period (mean 67.6 months, range 12-119 months), tumor recurrence was noted in the NTR group but not in the GTR group. The NaMIPSI-A to the jugular foramen without rerouting of the fallopian canal is highly valuable for selected tumors of the jugular foramen. It is less invasive than other skull base approaches, and it allows safe and complete tumor removal in appropriate patients.

Three-Corridors Procedure to Operate in the Infralabyrinthine Cervico-Jugulo-Carotico-Tympanic Area.

INTRODUCTION: The aim of the study was to present the results of our experience in three-corridors procedures applied for the tumors and inflammatory lesions of the infralabyrinthine cervico-jugulo-carotico-tympanic area. METHODS: The lesions located in the infralabyrinthine cervico-jugulo-carotico-tympanic area were operated in 13 patients using the 3-corridors technique. The anatomical and functional integrity of the external and middle ears and the facial nerve (FN) could be preserved. RESULTS: The diagnoses were glomus jugulare, infralabyrinthine petrous bone cholesteatoma, jugular foramen schwannoma, and giant-cell tumor. The follow-up duration ranged from 2 to 24 months. No tumor recurrence or growth was encountered in the follow-up. The operations were uneventful. Total surgical excision could be achieved in 10 patients. A second-stage retrosigmoid approach was performed for the total removal of the intracranial tumor remnant in two patients. A wait-and-scan policy has been considered in one patient who had partial resection for a glomus jugulare tumor. CONCLUSION: Three-corridors procedure seems to be a useful technique to operate in the infralabyrinthine, cervico-jugulo-carotico-tympanic area as it takes the advantage of hearing preservation, preservation of the anatomic and functional integrity of the external and middle ear structures as well as the FN.

Lateral Skull Base Chordoma Mimicking a Paraganglioma.

We present an unusual case of chordoma arising entirely from the lateral skull base with imaging features suggestive of a paraganglioma. Clinical history, management, histopathology, and imaging characteristics are described, including a review of gallium-dotate PET scanning somatostatinreceptor-positive tumors. We further provide a review of management options, including a summary of our approach with surgical biopsy via retrosigmoid and resection via transtemporal approaches. Based on radiologic characteristics and location, lateral skull base chordoma may arise with isolated lateral skull base involvement and has the potential to be misidentified as a glomus jugulare on initial workup.

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.

Involvement of the Cochlear Aqueduct by Jugular Paraganglioma Is Associated With Sensorineural Hearing Loss.

OBJECTIVE: The etiology of sensorineural hearing loss (SNHL) in patients with jugular paraganglioma (JP) whose tumors lack inner ear fistulae or vestibulocochlear nerve involvement is unknown. Recent literature has proposed that occlusion of the inferior cochlear vein may be causative. Herein, we assess the association between radiologic involvement of the cochlear aqueduct (CA) and the development of SNHL. STUDY DESIGN: Blinded, retrospective review of imaging and audiometry. SETTING: Tertiary center. PATIENTS: Adults with JP. INTERVENTION(S): None. MAIN OUTCOME MEASURES: Asymmetric SNHL was assessed continuously as the difference in bone conduction pure-tone average (BCPTA) between ears and as a categorical variable (≥15 dB difference at two consecutive frequencies, or a difference in speech discrimination score of ≥15%). Involvement of the CA was considered present if there was evidence of medial T2 fluid signal loss, contrast enhancement, or bony erosion/expansion. RESULTS: Of 30 patients meeting inclusion criteria, 15 (50%) had asymmetric SNHL. CA involvement was observed in 87% of patients with asymmetric SNHL compared with 13% in those with symmetric hearing (p = 0.0001). Univariate analysis demonstrated that age, sex, and tumor volume were not associated with asymmetric SNHL. The median difference in BCPTA between ears in patients with CA involvement was 21.3 dB HL compared to 1.2 dB HL in those without CA involvement (p < 0.0001). Regression analysis demonstrates that enhancement within the CA is associated with a BCPTA difference of 19.4 dB HL (p = 0.0006). CONCLUSIONS: Cochlear aqueduct involvement by JP is associated with SNHL in the absence of inner ear fistula, vestibulocochlear nerve involvement, or brainstem compression. Correlation with operative findings or histopathologic evidence of tumor involvement may validate this intriguing imaging finding.

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.

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.

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.

The Application of Sigmoid Sinus Tunnel-packing or Push-packing of the Inferior Petrous Sinus in the Microsurgical Management of Jugular Paragangliomas.

OBJECTIVE: To analyze the outcomes of microsurgically treated jugular paragangliomas with control of bleeding from the inferior petrous sinus. STUDY DESIGN: Retrospective patient review. SETTING: A single university hospital. PATIENTS: Forty-three patients with jugular paragangliomas were diagnosed in the past 7 years in our clinic. MAIN OUTCOME MEASURES: Surgical tumor control, intraoperative blood loss, intraoperative management of the facial nerve, and the preoperative and postoperative function of the lower central nerves. RESULTS: Twenty-six patients underwent microsurgical treatment. Eighteen tumors were class C2 (69.2%), six were class C3 (23.1%), and two were class CDe1 (7.7%). Gross total tumor resection was achieved in 92.3% of the patients. The mean blood loss during surgery was 438.5 ml. The recurrence rate was 3.8% over a mean follow-up of 29.2 months. New facial palsy and lower cranial nerve deficit occurred in 42.3 and 11.5% of the patients, respectively. CONCLUSION: The infratemporal fossa approach type A with sigmoid sinus tunnel-packing or push-packing technique facilitated the control of bleeding from the inferior petrous sinus and improved the outcomes of microsurgical treatment for jugular paragangliomas.

Results of a systematic literature review of treatment modalities for jugulotympanic paraganglioma, stratified per Fisch class.

OBJECTIVE: Key for successful jugulotympanic paraganglioma management is a personalised approach aiming for the best practice for each individual patient. To this end, a systematic review is performed, evaluating the local control and complication rates for the different treatment modalities stratified by the broadly accepted Fisch classification. DESIGN: A systematic literature review according to the PRISMA statement was performed. A detailed overview of individual treatment outcomes per Fisch class is provided. MAIN OUTCOME MEASURES: Local control, cranial nerve damage, complications, function recovery. RESULTS: Eighteen studies were selected, resembling 83 patients treated with radiotherapy and 299 with surgery. Excellent local control was found post-surgery for class A and B tumours, and risk of cranial nerve damage was <1%. For class C1-4 tumours, local control was 80%-95% post-surgery (84% post-radiotherapy), and cranial nerve damage was found in 71%-76% (none post-radiotherapy; P < .05). There was no difference in treatment outcomes between tumours of different C class. For class C1-4De/Di tumours, local control was 38%-86% (98% post-radiotherapy; P < .05) and cranial nerve damage/complication rates were 67%-100% (3% post-radiotherapy; P < .05). C1-4DeDi tumours showed lesser local control and cranial nerve damage rates when compared to C1-4De tumours. CONCLUSIONS: An individual risk is constituted for surgery and radiotherapy, stratified per Fisch class. For class A and B tumours, surgery is a suitable treatment option. For class C and D tumours, radiotherapy results in lower complication rates and similar or better local control rates when compared to the surgical group.

Hearing Outcomes After Stereotactic Radiosurgery for Jugular Paraganglioma.

OBJECTIVE: To describe audiometric outcomes following stereotactic radiosurgery (SRS) for jugular paraganglioma (JP). STUDY DESIGN: Retrospective review. SETTING: Tertiary referral center. PATIENTS: Patients with pretreatment serviceable hearing (American Academy of Otolaryngology-Head and Neck Surgery [AAO-HNS] Class A or B) and serial post-SRS audiometric follow-up who underwent Gamma Knife SRS for JP between 1990 and 2017. INTERVENTION(S): Gamma Knife SRS. MAIN OUTCOME MEASURES: Progression to nonserviceable hearing; correlation between baseline hearing and treatment parameters with audiometric outcomes. RESULTS: Of 85 patients with JP who underwent SRS during the study period, 35 (66% female, median age 53) had pretreatment serviceable hearing and serial post-treatment audiometry available for review. Median tumor volume at the time of treatment was 7,080 mm, median cochlear point dose was 5.8 Gy (interquartile range [IQR] 4.1 to 7.3 Gy), and median marginal and maximum tumor doses were 16 and 32 Gy, respectively. After a median follow-up of 37 months (IQR 16 to 77 mo), the median change in pure-tone average and speech discrimination score in the treated ear was -1.2 dB HL/yr (IQR -4.5 to 0.3) and 0%/yr (IQR 0-3.5%), compared with 0.07 dB HL/yr (IQR -0.03 to 0.12) and 0 %/yr (IQR 0 to 0%) in the contralateral untreated ear. Seven patients developed nonserviceable hearing (AAO-HNS Class C or D) at a median of 13.2 months following SRS (IQR 4.8 to 24 mo). Among those who maintained serviceable hearing, median audiometric follow-up was 42 months (IQR 18 to 77 mo). The Kaplan-Meier estimated rates of serviceable hearing at 1, 3, and 5 years following SRS were 91%, 80%, and 80%, respectively. Sixty percent of patients with pulsatile tinnitus who underwent SRS experienced varying levels of symptomatic improvement following treatment. CONCLUSION: The short- and intermediate-term risk of progression to nonserviceable hearing following SRS for JP is low. Data regarding the impact of cochlear dose from the vestibular schwannoma literature should not be freely applied to JP, since the impact of SRS parameters on hearing preservation seems to be less significant.