Jugulotympanic Paragangliomas Research Articles

Postauricular Transcanal Posterior Tympanectomy (PT 2 ) Approach - A Modified Surgical Technique for Jugulotympanic Paragangliomas.

Introduction The surgical management of jugulotympanic paragangliomas has remained challenging. They are the second most common type of tumor of the temporal bone after acoustic neuroma. It has been noticed by the authors that the jugulotympanic paragangliomas may have extensions to the epitympanum and aditus in addition to the mesotympanum and hypotympanum. The modified technique could be an alternative to the conventional facial recess technique for complete removal of the tumors. Objective To highlight the modified surgical technique for the surgical treatment of jugulotympanic paragangliomas. Methods This is a retrospective review of 34 cases of jugulotympanic paragangliomas treated in a tertiary center with respect to clinical presentation, diagnosis, and surgical treatment. Tinnitus and hearing loss were predominant symptoms. A modified technique of postauricular transcanal posterior tympanectomy with extended hypotympanic access was performed in 29 patients. Only two cases were operated with a classical transcanal approach. A canal wall down the mastoidectomy was required in three patients. Results The patients operated on with the modified technique had complete excision evident by absence of any lesion in computed tomography and the disappearance of tinnitus. However, two patients had recurrence of symptoms and presence of tumor in the follow-up period. These two patients underwent revision surgery. None of the patients required postoperative radiotherapy or gamma knife therapy. Conclusions Jugulotympanic paragangliomas can be effectively managed with the modified technique to ensure complete removal of the lesions. This technique has not been reported earlier in the literature.

Contemporary Radiotherapy for Fisch-Classified Jugulotympanic Paragangliomas - Does Tumor Category Matter? Outcomes From a 13-Year Experience

<h3>Purpose/Objective(s)</h3> Jugulotympanic paragangliomas (JTPGs), recognized as slow-growing tumors, represent a treatment challenge because of their known vascular nature, tendency to be locally invasive, location near many vital structures, and their potential to cause very disabling symptoms. This study aimed to examine the outcomes of patients with Fisch-classified JTPGs treated with conventional radiotherapy including stereotactic radiosurgery. <h3>Materials/Methods</h3> Twenty-four consecutive patients with head and neck paragangliomas diagnosed between November 2003 and May 2017 were identified from the radiation oncology and radiosurgery databases and lists of patients presented for discussion at the weekly Department of Otolaryngology-Head and Neck Surgery multidisciplinary conference. After excluding individuals with carotid body glomus tumors or missing clinical and radiological follow-up information, 13 patients (with a mean age of 61.8 years; 6 glomus tympanicum and 7 glomus jugulare PGs; 10 women and 3 men;) composed the JTPG study participants. Non-invasive stereotactic radiosurgery or external beam radiotherapy was used to treat JTPGs in 11 patients and 2 patients, respectively. The investigation endpoints included subjective and objective responses to treatment (i.e., exhibited symptoms and signs of disease and the tumor) and observed toxicity. <h3>Results</h3> With regard to the risk designation of JTPGs, seven (54%) belonged to the Fisch A-B categories and six (46%) to C-D categories. During a median follow-up of 36 months (range 7-204) after-therapy, subjective responses consisted of eight patients (62%) who were symptom-free, and five individuals (38%) who did not improve. At a median radioimaging follow-up of 34 months (range 6-204), eight (62%) treated neoplasms were stable, two (15%) showed continued tumor growth, and three (23%) were smaller following radiation treatment. One participant (8%) experienced transient otalgia and tinnitus after external beam irradiation; no patient developed radiation oncogenesis. Long-term follow-up (from 75 to 204 months) was documented in six people (46%). Characterization and effects of treatment were not significantly different between the compared Fisch categorized groups of JTPGs. <h3>Conclusion</h3> The observed rates of tumor control (85%) and treatment-related toxicity (8%), and the long-term disease progression-free survival of some patients suggest that contemporary radiotherapy is efficacious and safe when applied for Fisch-classified JTPGs.

Intensity modulated conformal radiotherapy of a jugulo-tympanic paraganglioma

Jugulo-tympanic paragangliomas are rare benign tumors arising from the neuroectodermal cells of the tympanic cavity and the dome of the jugular vein. Surgical resection has long been the first-line treatment. However, when this is not possible, radiotherapy remains a therapeutic alternative allowing local control with less morbidity. We report the case of a patient with an unresectable tympano-jugular paraganglioma, the symptomalagy was dominted by chronic headaches and right sided hypoacousie, and the treatment consisted of external radiation therapy with a total dose of 50 Gy in 25 fractions, one fraction per day, 2 Gy per fraction using the IMRT technique. The evolution was good with disappearance of clinical symptomatology, significant local control with less toxicity.

Extracranial temporal bone paragangliomas: Re-defining the role of otologic surgery within the scope of function-preserving multimodal concepts

IntroductionThe aim of this study was to present our concept in the management of extracranial temporal bone paragangliomas and demonstrate the outcome after primary surgical management of the middle ear component, with an individualized indication for adjuvant radiotherapy. Materials and methodsThe records of all patients treated for extracranial jugulotympanic paragangliomas by means of primary surgical management between 2010 and 2021 were studied retrospectively. ResultsTwenty-nine patients made up our study sample (mean age 58.8 years). 15 cases were managed solely by means of surgery. Out of the remaining 14 cases with reduction of the middle ear component, adjuvant irradiation was performed in 11 cases, whereas a wait-and-scan strategy was adopted at the patient's request in three cases. No further growth was detected in our study cases. ConclusionOur protocol seems to be associated with an acceptable quality of life and a satisfactory oncologic outcome.

Long term outcomes with linear accelerator stereotactic radiosurgery for treatment of jugulotympanic paragangliomas.

Data supporting linear accelerator (linac) stereotactic radiosurgery (SRS) for jugulotympanic paragangliomas (JTPs) come from small series with minimal follow-up. Herein, we report a large series of JTPs with extended follow-up after frameless linac-based SRS. JTPs treated with linac-based SRS from 2002 to 2019 with 1+ follow-up image were reviewed for treatment failure (radiographic or clinical progression, or persistent symptoms after SRS requiring intervention) and late toxicities (CTCAE v5.0). Forty JTPs were identified; 30 were treated with a multifraction regimen. Median clinical and radiographic follow-up was 79.7 (interquartile range [IQR] 31.7-156.9) and 54.4 months (IQR 17.9-105.1), respectively, with a median 4.5 follow-up scans (IQR 2-9). Seven-year progression-free survival (PFS) was 97.0% (95% confidence interval 91.1%-100.0%). PFS was similar between single- and multifraction regimens (log rank P = .99). Toxicity was seen in 7.7% (no grade III). With extended clinical and radiographic follow-up, frameless linac-based SRS provides excellent local control with mild toxicity <8%.

P14.67 Somatostatin receptor targeted radioligand therapyin head and neck paraganglioma

Abstract BACKGROUND Surgical resection is the therapy of choice in head and neck paraganglioma. However, surgery is associated with significant morbidity depending on tumor size and localization. In case of progressive residual or recurrent disease, new and less aggressive therapeutic options are warranted. Somatostatin receptor (SSTR) targeted therapies pose a promising alternative, as SSTRs are upregulated in paragangliomas. Only few studies provide evidence on efficacy of 177Lu-DOTATATE therapy in these patients. The aim of this study was to evaluate the efficacy of peptide receptor radiotherapy (PPRT) with 177Lu-DOTATATE for head and neck paragangliomas. METHODS A total of seven consecutive patients (mean age: 60 years, range: 14–84) with progressive head and neck paragangliomas (3 carotid body tumors, 4 jugulotympanic paragangliomas), treated with PRRT using 177Lu-DOTATATE, were included in this retrospective analysis. Therapy response was assessed by interim and post-therapy 68Ga-DOTATATE-PET-CT and MRI according to morphological and SSTR imaging parameters. Therapeutic uptake was classified on177Lu-DOTATATE scintigraphies 48h p.i. according to the Krenning scale. Longer-term clinical and imaging-based outcome were evaluated. RESULTS Patients received a median of 4 cycles (range: 3 to 5) of PRRT with 177Lu-DOTATATE (mean injected dose 7.3 GBq). Interim and post-therapy staging showed at least stable disease in all patients. Tumor uptake in 48h p.i. 177Lu-scintigraphies was moderate to high in all patients (Krenning scale; median: 3; range: 2–4). Furthermore, none of the patients showed progressive disease during the median follow-up time of 28 months (range: 6–37 months). CONCLUSION SSTR targeted therapy using 177Lu-DOTATATE shows promising efficacy and may lead to long lasting stable disease.

Jugulotympanic paragangliomas in southern Finland: a 40-year experience suggests individualized surgical management.

Treatment of jugulotympanic paragangliomas (JTPGLs) remains challenging with no clear guidelines for management or follow-up. The aim of this retrospective case-note study was to assess long-term results of operatively and conservatively managed JTPGLs between years 1974-2013. A total of 36 patients with JTPGLs were identified. Clinical characteristics and management outcomes of patients were reviewed. Data were extracted on demographics, symptoms, timing of diagnosis, tumor location and size, embolization, and management, including pre- and post-operative imaging, analysis of operative techniques, and follow-up. Pulsatile tinnitus and hearing loss were the most common presenting symptoms. Thirty-four (94%) patients were treated with primary surgical therapy and two (6%) with radiotherapy. The surgical approaches included endaural approach for Fisch Class A tumors and a variety of approaches for Fisch Class B-D tumors with an increasing predilection for function-preserving surgery. Eight (24%) patients received subtotal resection. Five (15%) patients had a local recurrence within 10years after primary surgery. Two (6%) patients suffered a permanent cranial nerve (CN) deficit after primary surgery. We advocate radical surgery when tumor resection is possible without compromising CNs. Function-preserving surgery with at least a 10-year follow-up for Fisch Class B-D tumors should be considered if CNs are in danger.

Management of multicentric paragangliomas: Review of 24 patients with 60 tumors.

The purpose of this study was to reflect our experience in the management of multicentric head and neck paragangliomas, including genetic study and counseling, diagnostic tools, types of treatment, and the need for monitoring. A retrospective review of 24 patients with multicentric paraganglioma, including a total of 60 paragangliomas: 37 carotid body tumors, 13 jugulotympanic paragangliomas, and 10 vagal paragangliomas. A total of 26 surgical procedures were performed, including the resection of 36 paragangliomas. Four tumors were pending surgery at the time of this review. Radiotherapy was administered in 7 patients with 9 tumors. A "wait-and-scan" policy with periodic MRI imaging tests was instituted for 9 patients with 12 paragangliomas. The knowledge of the different modalities of management is especially relevant in patients with multicentric paragangliomas. In every case, benefits and potential risks of all treatment options may be taken into consideration for every individual patient.

68Ga-DOTANOC PET/CT for Baseline Evaluation of Patients with Head and Neck Paraganglioma

The purpose of this study was to evaluate the role of (68)Ga-labeled DOTANOC PET/CT for baseline evaluation of patients with head and neck paragangliomas (HNPs). The data for 26 patients (mean age ± SD, 34.3 ± 10.4 y; 50% men) with known or suspected HNPs who underwent (68)Ga-DOTANOC PET/CT for staging were retrospectively analyzed. PET/CT was performed after intravenous injection of 132-222 MBq of (68)Ga-DOTANOC. The images were evaluated by 2 experienced nuclear medicine physicians in consensus, both qualitatively and quantitatively. The PET/CT findings were grouped as HNPs, paraganglioma at other sites (non-HNPs), and metastatic disease. The size and maximum standardized uptake values (SUVmax) were measured for all lesions. All of the patients also underwent whole-body (131)I-metaiodobenzylgunanidine ((131)I-MIBG) scintigraphy and conventional imaging (CT/MR imaging) of the head and neck region. Their results were compared with those of (68)Ga-DOTANOC PET/CT. (68)Ga-DOTANOC PET/CT findings were positive in all 26 patients, and 78 lesions were detected. PET/CT imaging demonstrated 45 HNPS, 10 non-HNPs, and 23 metastatic sites. Fifteen patients (57.6%) had more than one site of disease on PET/CT. Among 45 HNPs, 26 were carotid body tumors (CBTs), 15 glomus jugulare, 3 glomus tympanicum, and 1 laryngeal paraganglioma. A positive correlation was seen between size and SUVmax of HNPs (ρ = 0.323; P = 0.030). The SUVmax of the CBTs was higher than that of jugulotympanic paragangliomas (P = 0.026). No correlation was seen between size and SUVmax (ρ = 0.069; P = 0.854) of non-HNPs. The size and SUVmax of non-HNPs were significantly less than those of HNPs (P = 0.029 and 0.047, respectively). (131)I-MIBG scintigraphy showed only 30 of the 78 lesions and was inferior to PET/CT (P < 0.0001). Conventional imaging (CT/MR imaging) was positive for 42 of 49 head and neck lesions and was inferior to PET/CT on direct comparison (P = 0.015). A combination of CT/MR imaging and (131)I-MIBG scintigraphy detected only 53 of 78 (67.9%) lesions and was also inferior to PET/CT (P < 0.0001). (68)Ga-DOTANOC PET/CT is useful for the baseline evaluation of patients with HNPs and can demonstrate synchronous paragangliomas at other sites and distant metastases. It is superior to (131)I-MIBG scintigraphy and conventional imaging (CT/MR imaging) for this purpose.

Jugulotympanic Paraganglioma, Mimicking Chronic Suppura tive Otitis Media

Paraganglioma or glomus tumour are named according to their origin. Jugulotympanic paragangliomas (JTP) originates in the middle ear. There are several diagnostic pitfalls of this tumour. We now report a rare case of JTP in a 47 years old female. Pre-operative diagnosis of this case was chronic suppurative otitis media, Per-operatively it revealed an irregular somewhat polypoid mass. On histologic examination the mass composed of nests of round to oval cells surrounded by delicate vascular septae and the diagnosis was JTP. Because JTPs are rare tumour and have variable clinical presentation &amp; different histologic findings they are easy to misdiagnose. However this case report may help to generate awareness and to avoid misinterpretation of JTPs. DOI: http://dx.doi.org/10.3329/akmmcj.v3i2.11693 AKMMC J 2012: 3(2): 37-38

Jugulotympanic paragangliomas: 75 years of evolution in understanding

Jugulotympanic paragangliomas were first described approximately 75 years ago. Since that time, there has been considerable evolution in knowledge of tumor biology, methods of classification, and appropriate management strategies. This paper attempts to summarize these gains in information.

Middle Ear Adenomas Stain for Two Cell Populations and Lack Myoepithelial Cell Differentiation

Middle ear adenomas (MEAs) are benign neoplasms along a spectrum with neuroendocrine neoplasms (carcinoid tumors). Immunohistochemical (IHC) staining for myoepithelial markers has not been reported in these tumors. The archives of the Cleveland Clinic, University of Virginia and Armed Forces Institute of Pathology were retrospectively searched for tumors arising within the middle ear with material available for IHC staining. Twelve cases of MEAs, four cases of jugulotympanic paragangliomas (JPGs), 10 cases of ceruminous adenomas (CAs) and four cases of ceruminous adenocarcinomas (CACs) were obtained. IHC staining was performed for smooth muscle actin (SMA), p63, S-100 protein, cytokeratin 5/6 (CK5/6), and cytokeratin 7 (CK7). The MEAs were positive for: CK7 (92%, luminal), CK5/6 (92%, abluminal), p63 (83%, abluminal), and negative for SMA and S-100 protein. The JPGs were negative for CK7, CK5/6, p63 and SMA; S-100 protein highlighted sustentacular cells. The CAs were positive for: CK7 (100%, luminal), CK5/6 (100%, abluminal), S-100 protein (80%, abluminal), p63 (100%, abluminal), and SMA (90%, abluminal). CACs demonstrated two patterns, (1) adenoid cystic carcinoma-type: positive for CK7 (100%, luminal), CK5/6, S-100 protein, p63, and SMA (all 100%, abluminal); and (2) conventional-type: CK7 (50% luminal), and no CK5/6, SMA, S-100 protein, or p63 expression. The IHC profile of MEAs suggests that these tumors harbor at least two cell populations, including luminal and basal cells. However, unlike ceruminous adenomas, MEAs lack true myoepithelial differentiation given the absence of S-100 protein and SMA staining in all cases.

Function‐preserving therapy for jugulotympanic paragangliomas: A retrospective analysis from 2000 to 2010

Treatment for jugulotympanic paragangliomas (JTPs) is shifting from radical toward function-preserving surgery. Alternative primary treatments are available, such as stereotactic radiotherapy (SRT) and radiosurgery. The aim of this study was to analyze the results after primary function-preserving surgery with or without adjuvant radiotherapy, or primary SRT, in the treatment of JTPs. Retrospective analysis. From 2000 to 2010, 45 patients (mean age, 62.5 years) received function-preserving treatment for JTPs-four type A paragangliomas (8.9%), 19 type B (42.2%), 13 type C (28.9%), and nine type D (20%) in the Fisch classification. The patients were retrospectively assigned to three groups: primary function-preserving surgery, primary stereotactic radiotherapy, and close follow-up. The end points for analysis were long-term tumor control, cranial nerve function, and hearing. A total of 64.4% of the patients (n = 29) received primary surgery, with complete resection in 18. Eleven patients had surgical tumor reduction, with fractionated postoperative radiotherapy (mean 53.5 Gy; range, 50-56 Gy) in five cases and radiosurgery (18 Gy) in one case; five patients received close follow-up. Twelve patients (26.7%) underwent primary radiotherapy (11 with SRT [mean, 50 Gy]; one with radiosurgery [16 Gy]). Four patients (8.9%) received a wait-and-scan strategy. The mean follow-up was 46.8 months. The overall tumor control rate was 97.2%. With these high tumor control rates, consistent with the literature, function-preserving therapy for JTPs currently appears to offer the greatest benefit for patients.

Immunohistochemical Profile of Neurotrophins and MIB-1 in Jugulotympanic Paragangliomas: Prognostic Value and Review of the Literature

Jugulo-tympanic paragangliomas are the most common primary neoplasm of the middle ear, but little is still known about the histological features differentiating the benign and malignant forms. We investigated, with an immunohistochemical procedure, the expression of neurotrophins with their receptors, in fifteen samples of paragangliomas, and MIB-1 in order to consider them as prognostic factors of malignancy. We observed a general positivity for NGF - TrKA - NT4 - TrKC in the cytoplasm, and a strong expression for BDNF in the extracellular space. MIB-1 was moderate in the nucleus of neoplastic cells, weak in the cytoplasm and totally absent in the extracellular space. The comparison between the clinical recurrences and the rate of cytoplasmatic neurotrophins showed strong immunoreactivity in recurrent patients. It should be emphasized that 2 of the 3 recurrences had a wider distribution of the neutrophins, leading to hypothesize the involvement of these substances in the cell proliferation of glomus tumors. Malignant forms of these rare glomus tumors cannot be clearly identified using MIB-1 as a prognostic marker, although we can affirm that neurotrophins and their receptors can be considered as a panel of potential diagnostic markers to monitor the development of such malignancies. Although the small number of patients does not allow definitive conclusions to be made, our findings showed a possible trend towards significance which requires a more powerful study to evaluate this further.

A jugulotympanic paraganglioma with craniocervical extension

Jugulotympanic paragangliomas (JTPs) are rarely encountered benign diseases derived embryonically from the autonomic nervous system. Although JTPs may originate extracranially or intracranially, little has been reported in the literature about large JTPs involving several anatomical structures in the craniofacial regions. Management of large JTPs is controversial. The authors describe a large JTP invading the cranial bones and extending through the neck to the left clavicle. Doppler sonography, computed tomography and magnetic resonance imaging were performed preoperatively to confirm the diagnosis. Combined therapeutic approaches were undertaken with preoperative selective embolization followed by craniofacial resection in cooperation with neurosurgeons.

Temporal Paragangliomas. A 12-Year Experience

ObjectiveOur objective was to perform a retrospective analysis of patients with jugulotympanic paragangliomas. We present the results according to the surgical approach applied in each case. Materials and methodsThis retrospective study presents the findings in 21 patients with jugulotympanic paragangliomas who were observed and treated in our department over a 12-year period (1999–2011). We performed a general otolaryngology exam, systemic evaluation, and radiological exam. Surgical treatment was performed in 20 cases out of 21. In 1 case, treatment with stereotactic radiosurgery was carried out. ResultsThe surgical approaches were: endaural, retroauricular transcanal, radical or modified mastoidectomy through facial recess, and infratemporal fossa approach. Preoperative embolisation was used in 12 cases. In all cases the diagnosis of paraganglioma was confirmed. The most frequent postoperative complications found were transitory palsy of the facial nerve, sensorineural hearing loss, imbalance, paralysis of the cranial nerves IX and XI, and salivary fistula. No recurrences were found after 12 years of follow-up. One case of persistence was found in the case treated with radiosurgery. ConclusionsIn our series surgery was found to be the elective therapy for patients with paraganglioma, with no recurrences after 12 years of follow-up. Preoperative embolisation decreases surgery time and intraoperative bleeding. Stereotactic radiotherapy cannot eliminate the tumour.

Paragangliomas temporales. Experiencia de 12 años

Objective Our objective was to perform a retrospective analysis of patients with jugulotympanic paragangliomas. We present the results according to the surgical approach applied in each case. Materials and methods This retrospective study presents the findings in 21 patients with jugulotympanic paragangliomas who were observed and treated in our department over a 12-year period (1999 to 2011). We performed a general otolaryngology exam, systemic evaluation and radiological exam. Surgical treatment was performed in 20 cases out of 21. In 1 case, treatment with stereotactic radiosurgery was carried out. Results The surgical approaches were: endaural, retroauricular transcanal, radical or modified mastoidectomy through facial recess and infratemporal fossa approach. Preoperative embolization was used in 12 cases. In all cases the diagnosis of paraganglioma was confirmed. The most frequent postoperative complications found were transitory palsy of the facial nerve, sensorineural hearing loss, imbalance, paralysis of the cranial nerves IX and XI and salivary fistula. No recurrences were found after 12 years of follow-up. One case of persistence was found in the case treated with radiosurgery. Conclusions In our series surgery was found to be the elective therapy for patients with paraganglioma, with no recurrences after 12 years of follow-up. Preoperative embolization decreases surgery time and intraoperative bleeding. Stereotactic Radiotherapy cannot eliminate the tumour..

Bilateral neck paragangliomas

Paragangliomas of the head and neck are rare neoplasms presented as cervical mass, generally bilateral, that arise from chemoreceptors located at the carotid bifurcation (carotid body tumors), along the vagus nerve (vagal paragangliomas), and in the jugular fossa and tympanic cavity (jugulotympanic paragangliomas). They are typically asymptomatic at the beginning, highly vascular, slow-growing and compressing the surrounding anatomic structures. Only radical surgery is the curative treatment for paragangliomas. We present a case of a 62- year-old woman with a diagnosis of bilateral neck paragangliomas where surgical removal was judged burdened by excessive risk because of the size of the tumor.

Management of Paragangliomas in Otolaryngology Practice

Paragangliomas of the head and neck are highly vascular lesions originating from paraganglionic tissue located at the carotid bifurcation (carotid body tumors), along the vagus nerve (vagal paragangliomas), and in the jugular fossa and tympanic cavity (jugulotympanic paragangliomas) and should be considered in the evaluation of all lateral neck masses. The aim of this study is to review an institutional experience in the management of these tumors. Twenty-six patients with 27 paragangliomas were treated in our institution during a period of 7 years (2000-2007). There were 15 women (57.6%) and 11 men (42.4%) with a mean age of 33.5 years. A painless lateral neck mass was the main finding in 16 patients (61.5%). There was no evidence of a functional tumor. Carotid angiography was performed on all of our patients (100%) to define the vascular anatomy of the lesion. Twenty-two paragangliomas (of the 25 operated paragangliomas; 88%) underwent selective embolization of the major feeding arteries. We performed surgery on 24 (92.3%) patients. Two patients were treated with radiotherapy. Most lesions were paragangliomas of the carotid bifurcation (n = 14 [51.8%]), whereas 6 patients were diagnosed with jugular (22.2%), 1 with a vagal (3.7%), 1 with a tympanic paraganglioma (3.7%), 2 with jugulotympanic paraganglioma (7.4%), and 1 with laryngeal paraganglioma (3.7%). In 1 patient (3.8%), bilateral paragangliomas in the carotid bifurcation were detected. There was an evidence of malignancy in all cases (3.8%). Preoperative embolization has proven successful in reducing tumor vascularity in approximately 22 (of 25 who accepted surgery; 88%) paraganglioma patients. The common preoperative complication was vascular injury, which occurred in 6 (23%) of 26 patients; the main postoperative complication was transient cranial nerve deficit in 4 (15.3%) of 26 patients; and a permanent Horner syndrome was documented in 2 patients (7.6%). Cerebrospinal fluid leak occurred in 1 patient (3.7%). Postoperatively, stroke was occurred in 1 patient (3.7%). Two patients with jugular paraganglioma were treated with irradiation because of skull base extension with significant symptomatic relief. The primary therapeutic option for paragangliomas is complete excision of tumor with preservation of vital neurovascular structures. Combined therapeutic approach with preoperative selective embolization followed by surgical resection is the safe and the effective method for complete excision of the tumors with a reduced morbidity rate.

Current assessment and management of glomus tumors

To provide an overview on the recent advances in the diagnosis and treatment of glomus tumors (paragangliomas). The review focuses on the genetics of paragangliomas and discusses the role of newer therapeutic modalities in the management of jugulotympanic paragangliomas. Recent advances in the field of genetics allowed the identification of three main loci involved in hereditary paragangliomas. Genetic screening of patients with family history identifies affected individuals. The management of glomus tumors continues to be a challenging task. Surgery remains the mainstay of treatment for young healthy patients with functional cranial nerve deficits. Gamma knife radiosurgery is offered as an adjunctive to limited surgery and as the primary treatment modality in poor surgical candidates or patients with bilateral disease.