Paraganglioma Research Articles

Dural Arteriovenous Fistula with Hypoglossal Nerve Paralysis

A male in his early 30s presented with a swelling just below the angle of his left mandible for the last 2 months. The swelling had insidious onset, progressive and painless. Examination found 4 × 4cm, diffuse, soft to firm, pulsatile swelling in the upper part of the neck on left side ([Fig. 1]). An oral examination revealed left hypoglossal nerve paralysis. Further examination was noncontributory, including vagus and accessory spinal nerve examination. A probable diagnosis of carotid body tumor was kept, and the patient was subjected to computerized angiography. Angiography revealed dural arteriovenous fistula (DAVF), a rare clinical entity leading to hypoglossal nerve paralysis ([Figs. 2 (A, B)]). Patient was advised to undergo digital subtraction angiography for further management, but he refused and chose conservative care with regular follow-up.

Application of the shear wave elastography in the assessment of carotid body tumors: A preliminary study.

To evaluate the elasticity of carotid body tumors (CBTs) by two-dimensional shear wave elastography (SWE). 22 pathologically or clinically confirmed CBTs in 16 patients were scanned by SWE. The maximum elasticity value (Emax) and its standard deviation (SDmax) in kPa and m/s for CBTs were obtained by placing a round ROI (2-3 mm) on the stiffest region of the CBTs. Elasticity value was compared between hard and soft groups at manual palpation, benign and malignant groups and among three Shamblin types. The area under the receiver operating characteristic curve (AUC) analysis was performed to evaluate the performance of SWE in the malignancy prediction of CBTs. Sensitivity, specificity and accuracy were calculated. The cut-off value was obtained by using the Youden index. There were 19 benign CBTs and 3 malignant CBTs. Emax (kPa and m/s) and SDmax (kPa) were significantly higher in the hard group than in the soft group at manual palpation (P<0.05); The distribution of Emax in kPa and m/s and SDmax in kPa were different in the three Shamblin types (P<0.05), Emax (kPa and m/s)increased from shambling I to Shambling II and Shambling III; Emax (kPa and m/s) were significantly higher in the malignant CBTs than in the benign ones (P<0.05). Emax in kPa and m/s had the similar AUC value (AUC=0.947, P=1.0000) for the prediction of malignant CBTs. Emax in kPa with the cut-off 124.9kPa showed a sensitivity of 100.0%, specificity of 94.7%, and an accuracy of 95.5% (Z=8.500, P<0.0001); Emax in m/s with the cut-off 5.9m/s showed a sensitivity of 100.0%, specificity of 89.5% and an accuracy of 90.9% for the prediction of malignant CBTs (Z=9.143, P<0.0001). Quantitative analysis of SWE obtained the good performance in the elasticity assessment of CBTs.

SDHB exon 1 deletion: A recurrent germline mutation in Colombian patients with pheochromocytomas and paragangliomas.

Pheochromocytomas (PCCs) and paragangliomas (PGLs) (known as PPGL in combination) are rare neuroendocrine tumors of the adrenal medulla and extra-adrenal ganglia. About 40% of the patients with PPGL have a hereditary predisposition. Here we present a case-series of 19 unrelated Colombian patients with a clinical diagnosis of PPGL tumors that underwent germline genetic testing as part of the Hereditary Cancer Program developed at the Instituto Nacional de Cancerología, Colombia (INC-C), the largest reference cancer center in the country. Ten of 19 patients (52.63%) were identified as carriers of a pathogenic/likely pathogenic (P/LP) germline variant in a known susceptibility gene. The majority of the P/LP variants were in the SDHB gene (9/10): one corresponded to a nonsense variant c.268C>T (p.Arg90*) and eight cases were found to be carriers of a recurrent CNV consisting of a large deletion of one copy of exon 1, explaining 42% (8/19) of all the affected cases. Only one additional case was found to be a carrier of a missense mutation in the VHL gene: c.355T>C (p.Phe119Leu). Our study highlights the major role of SDHB in Colombian patients with a clinical diagnosis of PGL/PCC tumors and supports the recommendation of including the analysis of large deletions/duplications of the SDHB gene as part of the genetic counselling to improve the detection rate of hereditary cases and their clinical care.

Bilateral Conjugating Carotid Body Tumor and Associated Jugular Vein Aneurysm.

Carotid body tumors (CBTs) originate from the paraganglionic tissue in the bifurcation of the common carotid artery. Magnetic resonance (MR) imaging is a commonly used diagnostic method in the preoperative diagnosis of these tumors. In this study, we demonstrated an isthmus between the right and left carotid body tumors in a patient with bilateral CBT. The left CBT also was associated with a saccular aneurysm of left external jugular vein.

A New Mutation Site of Succinate Dehydrogenase-Related Carney-Stratakis Syndrome: A Case Report

Carney-Stratakis Syndrome (CSS), first described in 2002 [1], encompasses Gastrointestinal Stromal Tumors (GISTs) and Paragangliomas (PGLs) and has autosomal dominant inheritance with incomplete penetrance [2]. Germline mutations of Succinate Dehydrogenase (SDH) complex subunits and consequent SDH functional deficiency have been identified as responsible for CSS [3]. Here, we present a case with a new mutation site in SDHB that has not yet been reported.

Clinical presentation of sporadic and hereditary pheochromocytoma/paraganglioma

Pheochromocytomas (PHEO) and paragangliomas (PGL) can occur sporadic or within genetic predisposition syndromes. Despite shared embryology, there are important differences between PHEO and PGL. The aim of this study was to describe the clinical presentation and disease characteristics of PHEO/PGL. A retrospective analysis of consecutively registered patients diagnosed with or treated for PHEO/PGL in a tertiary care centre was performed. Patients were compared according to anatomic location (PHEO vs PGL) and genetic status (sporadic vs hereditary). In total, we identified 38 women and 29 men, aged 50 ± 19 years. Of these, 42 (63%) had PHEO, and 25 (37%) had PGL. Patients with PHEO presented more frequently with sporadic than hereditary disease (45 years vs 27 (77%) vs 8 (23%)) than patients with PGL (9 (36%) vs 16 (64%), respectively) and were older at diagnosis (55 ± 17 vs 40 ± 18 years, P = 0.001), respectively). About half of the cases in both PHEO and PGL were diagnosed due to disease-related symptoms. In patients with PHEO, tumour diameter was larger (P = 0.001), metanephrine levels higher (P = 0.02), and there was more frequently a history of cardiovascular events than in patients with PGL. In conclusion, we found that patients with PGL more frequently have a hereditary predisposition than those with PHEO, contributing to the fact that diagnosis is generally made earlier in PGL. Although diagnosis in both PHEO and PGL was mostly due to related symptoms, patients with PHEO more often presented with cardiovascular comorbidities than those with PGL which might relate to a higher number of functionally active tumours in the former.

A new potential risk factor for permanent cranial nerve injury following carotid body tumor resection

To quantify the association between the free distal segment length of the internal carotid artery (FDS-ICA) and permanent cranial nerve injury (p-CNI) following carotid body tumor (CBT) resection. This study is a case-control study. We surveyed 109 consecutive patients who underwent CBT resection between June 2015 and June 2020 at our single center. A total of 89 patients met the inclusion criteria and were selected for analysis. The FDS-ICA was measured by image post-processing software for computed tomography angiography (CTA). Postoperative p-CNI complications were evaluated using comprehensive statistical approaches. The cohort was divided into 2 groups depending on the presence of p-CNI, namely the p-CNI group (n=17) and the non-CNI group (n=79). The average FDS-ICA of patients with p-CNI complications was shorter than that of those without p-CNI complications (P<0.001). For every 1 mm increase in FDS-ICA, there was an associated decrease of 8% in the risk of p-CNI (0.92, 95% CI: 0.85 to 0.98, P<0.05). Threshold effect analysis of the FDS-ICA on p-CNI identified that the FDS-ICA was 28.7 (95% CI: 23.8 to 30.9) mm. The results of this study revealed a significant independent association between FDS-ICA and permanent postoperative cranial nerve injury complications of CBTs. Further study is warranted to confirm these results in a larger patient cohort.

Carotid Body Tumor Presentation, Management, and Outcome an Institutional Experience – 12-Year Experience

Objective: This study aimed to analyze the presentation and treatment modalities of carotid body tumor (CBT) and evaluate the outcomes and complications of surgical treatment of CBT. Methodology: Twenty-seven CBT patients treated surgically from January 2012 to August 2022 were included in the study. Patient presentation, diagnostic modalities, surgical and radiological interventions, outcomes, and complications were analyzed. Results: (1) A total of 27 patients were studied, with an age ranging from 13 to 65 years (median age - 45 years), in which male preponderance was found (male: female = 2.5:1), (2) Local swelling was the most common symptom (n = 27), with other symptoms being hoarseness of voice, dysphagia, and ear/neck pain, (3) Left laterality was found in 76.2% of patients, whereas 17.3% of them were right sided with 6.5% of bilateral cases, (4) 15 patients (55.5%) out of the 27 total patients, were classified in Shamblin’s type II, 8 patients (29.6%) in Shamblin’s type I, and the remaining 4 patients (14.8%) as Shamblin’s type III CBTs, (5) Thirteen (48.1%) patients underwent preoperative angioembolization. All (100%) patients underwent complete excision (6) Operative site hematoma was the most common postoperative complication encountered. All patients are asymptomatic with the minimum of 6-month follow-up. Conclusion: Careful preoperative evaluation, which included appropriate modality for diagnosis, preoperative angioembolization for large tumors, meticulous dissection, and good postoperative care, resulted in complete excision of all the tumors and with less complications postoperatively. Shamblin III had a high risk of postoperative neurovascular complications. Therefore, early detection and prompt surgical resection of CBTs will decrease surgical morbidity.

External Auditory Canal Paraganglioma Causing Chronic Mastoiditis with Complications of Bezold and Luc’s Abscesses

Paragangliomas (PGs) are rare, benign neuroendocrine tumors that originate from neuroectodermal tissues. PGs arising from the ear canal are extremely rare, with only a few cases reported to date. The common symptoms of ear canal PGs include ear discharge, ear fullness, and hearing loss. This article describes unusual manifestations of ear canal PGs otitis media with Bezold and Luc’s abscesses, in an immunocompromised young adult. The pathogenesis of the disease is described. In addition, determining the origin of PG in this case was not straightforward compared to previously reported ear canal PGs. The derivation of the diagnosis is further discussed.

Unilateral Carotid Body Tumour: A Case Report

The carotid body, which is located bilaterally on the medial side of the carotid bifurcation, is the largest group of paraganglia in the head and neck. Chemoreceptor cells in the carotid bulb give rise to uncommon tumours known as Carotid Body Tumours (CBTs). The authors describe a case of a CBT in a 40-year-old woman who had a painful, pulsatile, lateral neck enlargement on the right side that had been developing over time. Due to the numerous difficulties and problems brought on by their high vascularity and close proximity to cranial nerves in the neck near the carotid bifurcation, they pose a surgical challenge. Based on the patient's medical history, the results of clinical and radiological examinations, and the successful surgical removal of the tumour, the diagnosis was suspected. The results of histological analysis supported the diagnosis of a CBT.

A Study of Arterial Supply to Carotid Body Tumor and Its Prospective Implication in Clinical Practice

Introduction and Objectives: Arterial supply to carotid body tumor is primarily from ascending pharyngeal artery (APA), a branch of the external carotid artery, and with infrequent feeders from other branches of the latter described in the literature. The present study attempted in describing other sources of arterial supply to carotid body tumor and its implication in clinical practice. Materials and Methods: This study was retrospective to start with turning a prospective midway for 5 years from July 2017 to July 2022. The data of all the 12 patients, in whom seven males and five females with a mean average of 45 years, were analyzed, There were seven males and five females with the mean age of 45 years; the average size of the tumor documented was 5 cm. All the patients underwent computed tomography angiography for confirming the diagnosis. Three-dimensional volume-rendered images from the rotational angiogram were analyzed for assessing the arterial supply. All patients underwent surgical excision without embolization. Results: In all 12 patients, APA was found primarily the main source of blood supply. Four patients, in addition, had occipital artery and superior thyroid in one. Two patients were identified to have glomic artery arising from the bulb of the common carotid artery supplying the tumor. The thyrocervical trunk was also supplying the tumor in a patient. Anomalous branches from the internal carotid artery were found to be the sole source of arterial supply to the tumor in a patient. Conclusion: Surgical excision of carotid body tumors still continues to be a challenge due to multiple arterial feeders leading to hypervascularity. A detailed knowledge of arterial supply helps in proper planning and reducing perioperative bleeding. The glomic artery, and in rare cases, the internal carotid artery, also contribute as a feeder to the tumor. These unusual arterial feeders give an important warning before routine embolization to avoid cerebrovascular events.

Correlation between the size of pheochromocytoma and the level of metanephrines.

Objective. Pheochromocytomas (PHEO) and paraganglioma (PGLs) are rare neuroendocrine catecholamine-producing tumors that arise from the chromaffin cells of either the adrenal medulla or extra-adrenal paraganglionic tissues. Despite the recent advances in imaging technologies, biochemical evidence of excessive catecholamine production by the tumor is considered the most important test for the diagnosis of these tumors. The aim of the present study is to investigate the role of the catecholamine metabolites (normetanephrine and metanephrine) levels in the diagnosis of PHEO/PGLs and to evaluate if their levels correlate with the size of these tumors. Patients and Methods. Twenty-five patients were included in the study during the time period of 10 years. Their data were compared with another set of 25 patients to obtain the sensitivity and specificity of metanephrine and normetanephrine in the diagnosis of PHEO/PGLs. The tumor size was reviewed in every patient to obtain the correlation coefficient between the tumor sizes and the plasma/24-hour urinary metanephrine levels. Results. The sensitivity and specificity rates for plasma metanephrine were 80-92% and 92-96%, respectively; while for 24-hour urinary metanephrine were 80-90% and 95-100%, respectively. We found a strong positive relationship between the tumor size and the plasma levels of normetanephrine (r=0.518, p<0.01), and metanephrine (r=0.577, p<0.01). While the relation with the 24-hour urinary concentrations of normetanephrine (r=0.384, p=0.01) and 24-h urinary meta-nephrine (r=0.138, p<0.01) was low. Conclusion. The determination of plasma and 24-hour urinary levels of metanephrines is a reliable test for the diagnosis of PHEO, as they are continuously produced by the tumor cells in contrast to catecholamines.

Postoperative objective and subjective voice analysis in patients who underwent carotid endarterectomy and carotid body tumor resection

Aim: This study aimed to compare voice changes after Carotid endarterectomy (CEA) and Glomus body tumor resection with objective instrumental and acoustic assessments. Material and Methods: A total of 14 patients underwent CEA or glomus carotid tumor resection at our institution between 2009 and 2013. Among the 14 patients, two patients were excluded due to vocal cord paralysis. This study was applied to 12 patients. Patients were called retrospectively, and objective and subjective voice parameters were measured and compared. Voice handicap index was used to measure subjective voice parameters. Objective voice analysis was performed for patients by using Multi-dimensional voice programming (MDVP), a computer-aided program. Results: A total of 12 patients were included in the study. Five patients underwent carotid endarterectomy. Glomus tumor resection was performed on seven patients. Of the patients who underwent carotid endarterectomy, four were male and one was female. Three of the patients who underwent glomus tumor resection were male and four were female. Voice handicap index (VHI) showed that two patients who underwent glomus tumor resection experienced moderate problems and the patients who underwent CEA had no obvious problems. Patients who underwent glomus carotid tumor resection obtained higher values in all MDVP parameters than those who underwent CEA. Conclusion: Despite the small number of patients, it can be said that glomus tumor resection in neck surgery, that is, tumor surgery, causes more voice changes than carotid endarterectomy operations. The elevated objective sound parameters found in this study necessitate that patients should be informed regarding the potential of developing postoperative dysphonia, especially those who rely on their voice as their profession (e.g. sound artists, teachers, politicians).

Multidisciplinary management of carotid body tumors: a single-institution case series of 22 patients.

Carotid body tumors (CBTs) are rare, slow-growing neoplasms derived from the parasympathetic paraganglia of the carotid bodies. Although inherently vascular lesions, the role of preoperative embolization prior to resection remains controversial. In this report, the authors describe an institutional series of patients with CBT successfully treated via resection following preoperative embolization and compare the results in this series to previously reported outcomes in the treatment of CBT. All CBTs resected between 2013 and 2019 at a single institution were retrospectively identified. All patients had undergone preoperative embolization performed by interventional neuroradiologists, and all had been operated on by a combined team of cerebrovascular neurosurgeons and otolaryngology-head and neck surgeons. The clinical, radiographic, endovascular, and perioperative data were collected. All procedural complications were recorded. Among 22 patients with CBT, 63.6% were female and the median age was 55.5 years at the time of surgery. The most common presenting symptoms included a palpable neck mass (59.1%) and voice changes (22.7%). The average tumor volume was 15.01 ± 14.41 cm3. Most of the CBTs were Shamblin group 2 (95.5%). Blood was predominantly supplied from branches of the ascending pharyngeal artery, with an average of 2 vascular pedicles (range 1-4). Fifty percent of the tumors were embolized with more than one material: polyvinyl alcohol, 95.5%; Onyx, 50.0%; and N-butyl cyanoacrylate glue, 9.1%. The average reduction in tumor blush following embolization was 83% (range 40%-95%). No embolization procedural complications occurred. All resections were performed within 30 hours of embolization. The average operative time was 173.9 minutes, average estimated blood loss was 151.8 ml, and median length of hospital stay was 4 days. The rate of permanent postoperative complications was 0%; 2 patients experienced transient hoarseness, and 1 patient had medical complications related to alcohol withdrawal. This series reveals that endovascular embolization of CBT is a safe and effective technique for tumor devascularization, making preoperative angiography and embolization an important consideration in the management of CBT. Moreover, the successful management of CBT at the authors' institution rests on a multidisciplinary approach whereby endovascular surgeons, neurosurgeons, and ear, nose, and throat-head and neck surgeons work together to optimally manage each patient with CBT.

Bilateral Carotid Paraganglioma: Case Report; Successful Surgical Excision, Radiological Image and Review of the Literature

Introduction: Carotid body tumors are rare neoplasms that generally present as asymptomatic slow-growing masses in the neck, commonly called cervical paragangliomas, highly vascularized whose cells originate from the embryonic neural crest, carotid glomus tumor is uncommon, representing 1 out of every 30,000 head and neck tumors, they are located in the adventitia of the vessels, they usually grow surrounding the arteries of the carotid bifurcation and the nerves that surround it, in Ecuador the prevalence is at 55 years of age with greater frequency in women [1]. Clinical Case: A 33-year-old female patient with no significant clinical history, presented with a painful hard mass in the right cervical region that progressively increased in size, for the past 6 months. On physical examination, a tumor in the right cervical region of approximately 5 centimeters in diameter was found, with a hard consistency attached to deep planes, for which Eco Doppler and AngioTac of the neck were performed, which reported bilateral carotid paraganglioma. Excision of the glomus was necessary due to its size right carotid artery, surgery using a right cervico-lateral approach, identifying the vascular tumor mass of 4.8 x 2.9 x 2.8 cm in diameter, which was located in the carotid bifurcation. Removal of Shamblin II right carotid body paraganglioma by temporary clamping of the common carotid artery and the digital dissection method, there was no intraoperative massive bleeding or cranial nerve deficits in the postoperative period with favorable evolution, surgical wound in good condition during follow-up in outpatient consultation. Conclusion: Glomus carotid tumors are generally benign and diagnosed late. Imaging studies are essential for its diagnosis and staging.

Preoperative Balloon Occlusion Test of the Carotid Artery and Preoperative Tumor Embolization on Paraganglioma of Carotid Body: 20-Year Experience in a Single Institute and Literature Review

Background and Objectives Carotid body paraganglioma is the common type of carotid body tumor for which angiography, carotid artery balloon occlusion test (BOT) and tumor embolization could be considered before the surgery. We analyzed cases in a single institute and reviewed related literature to investigate the necessity of these preoperative examinations.Subjects and Method Medical records of patients who were diagnosed with paraganglioma were retrospectively analyzed from 2000 to 2019.Results Sixteen patients were identified. Of the total, 14 patients underwent surgery at this institute, and 13 underwent angiography. Of the 13 patients who underwent angiography, 6 patients underwent carotid artery BOT, and 12 patients underwent tumor embolization. The average tumor size of 6 patients who underwent carotid artery BOT was 28.7 mm, and 8 patients who did not undergo carotid artery BOT was 30.1 mm. The average tumor size of 12 patients who underwent tumor embolization was 29.4 mm. Two patients did not undergo tumor embolization, and their average tumor size was 30 mm. In 1 patient, both preoperative angiography and carotid artery BOT were performed, but tumor embolization was not performed due to spasm of tumor vessels.Conclusion Preoperative carotid artery BOT can be performed to reduce side effects in patients with the potential for carotid resection. In addition, tumor embolization is performed regardless of tumor size. By reducing the amount of bleeding during surgery and reducing the size of the tumor, it is possible to secure an appropriate surgical field of view to facilitate operation during surgery; however, its effectiveness needs to be clearly identified.

Technique modification proposal for carotid body tumors resection: trans-bulb dissection technique (TBD)

Technique modification proposal for carotid body tumors resection: trans-bulb dissection technique (TBD)

Perioperative blood pressure and heart rate alterations after carotid body tumor excision: a retrospective study of 108 cases

BackgroundArising from chemoreceptor cells, carotid body tumors (CBTs) are rare neoplasms associated with hemodynamics. Perioperative changes in blood pressure (BP) and heart rate (HR) are not completely understood.MethodsThis retrospective, observational, controlled study included all CBT patients from 2013 to 2018 in Peking Union Medical College Hospital. Perioperative changes in BP/HR within or between unilateral/bilateral/control groups were investigated. Perioperative details across Shamblin types were also assessed.ResultsThis study included 108 patients (116 excised CBTs). The postoperative systolic BP and HR increased in both unilateral (mean difference of systolic BP = 5.9mmHg, 95% CI 3.1 ~ 8.6; mean difference of HR = 3.7 bpm, 95% CI 2.6 ~ 4.9) and bilateral (mean difference of systolic BP = 10.3mmHg, 95% CI 0.6 ~ 19.9; mean difference of HR = 8.4 bpm, 95% CI 0.5 ~ 16.2) CBT patients compared with the preoperative measures. Compared with control group, the postoperative systolic BP increased (difference in the alteration = 6.3mmHg, 95% CI 3.5 ~ 9.0) in unilateral CBT patients; both systolic BP (difference in the alteration = 9.2mmHg, 95% CI 1.1 ~ 17.3) and HR (difference in the alteration = 5.3 bpm, 95% CI 1.0 ~ 9.6) increased in bilateral CBT patients. More CBT patients required extra antihypertensive therapy after surgery than controls (OR = 2.5, 95% CI 1.14 ~ 5.5). Maximum tumor diameter, intraoperative vascular injury, continuous vasoactive agent requirement, total fluid volume, transfusion, estimated blood loss, operation duration, postoperative pathology, overall complications, and intensive care unit/hospital lengths of stay significantly varied among Shamblin types.ConclusionCBT excision may be associated with subtle perioperative hemodynamic changes. Perioperative management of CBT patients necessitates careful assessment, full preparation and close postoperative monitoring.

Step-by-Step Stereotactic Radiotherapy Planning of Glomus Jugulare: A Guide to Radiation Oncologists—Dr Kanhu’s ROSE (Radiation Oncology from Simulation to Execution)

Background Glomus jugulare is a rare, slow-growing tumor that arise within the jugular foramen of the temporal bone. In the past, surgery was the primary modality of treatment for glomus Jugulare, but it leads to many complications and increased mortality. Radiotherapy was indicated in adjuvant setting in post-operative residual disease. But, with the advent of highly conformal radiation planning, stereotactic radiosurgery (SRS), is now one of the main modalities of radiation treatment in glomus jugulare. Objective To describe the procedural steps for radiation planning of SRS of glomus jugulare. Methods The step-by-step procedure for stereotactic planning of glomus jugulare has been described using a clinical scenario of glomus jugulare. Results The stereotactic radiation planning of glomus jugulare starts with the basic history and relevant clinical evaluation, that is, visual testing. Computed tomography (CT) scan and magnetic resonance imaging (MRI) of the brain is the imaging modality of choice. The radiation planning of glomus jugulare starts with CT simulation. MRI of brain should be done in the prescribed format to achieve uniformity in radiation planning. After CT and MRI image fusion, contouring of target, organs at risk (OAR) and radiation planning should be done. The plan evaluation includes target and OAR coverage index, conformity, homogeneity and gradient index, and beam arrangement. After radiation plan evaluation, treatment is delivered after quality assurance and dry run. Conclusion The paper highlights the sequential process of radiation planning for SRS in glomus jugulare—starting from simulation, planning, evaluation of plan, and treatment.

Glomus jugulare tumors treatment by gamma knife radiosurgery: A single center study.

Glomus jugulare tumor are benign vascular tumors and surgical resection is almost impossible. We have treated these tumors by Gamma knife radiosurgery and share our experience. This study was conducted at the Neurospinal and Cancer Care Institute, Karachi from January 2010 to May 2020. Thirty-four patients with glomus jugulare tumors treated with gamma knife radiosurgery were included in the study. The comprehensive clinical and demographic characteristics of all patients were collected through a manually designed questionnaire. Computed tomography, digital subtraction angiography and magnetic resonance imaging were used to make the diagnosis. Data was incorporated and analyzed by SPSS version 26. A total of 34 patients were included in the study of which 16(47%) were males and 18(53%) were females with first follow up after 6-month up to two year clinical and radiological follow-up. The mean age of the patients was 42.5±13.5 with a minimum age of 20 years and maximum age of 65 years. The KPS scale was 2.09±0.45 and the volume of the tumor was 33.8±22.5 cm3. The improvement was shown in 27 patients of which 14 were males and 13 were females showing insignificance post radiation change. Of all 34 patients, the outcome was recorded as 3(9%) for excellent, 22(64%) for good, 6(17%) for fair and 3(9%) were poor results. Gamma Knife radiosurgery is a safe and effective primary therapy and salvage therapy for residual and recurrent cases of glomus jugulare and tympanicum tumors.