Preoperative Endovascular Embolization of Glomus Jugulare Tumors: A Retrospective Case Series of 22 Embolizations in 20 Patients and Literature Review

Objective Glomus jugulare tumors, or tympanojugular paragangliomas, are rare, highly vascularized skull base tumors originated from paraganglion cells of the neural crest. With nonabsorbable embolic agents, embolization combined with surgery has become the norm. The authors assess the profile and outcomes of patients submitted to preoperative embolization in a Brazilian tertiary care hospital. Methods The present study is a single-center, retrospective analysis; between January 2008 and December 2019, 22 embolizations were performed in 20 patients in a preoperative character, and their medical records were analyzed for the present case series. Results Hearing loss was the most common symptom, present in 50% of the patients, while 40% had tinnitus, 30% had dysphagia, 25% had facial paralysis, 20% had hoarseness, and 10% had diplopia. In 7 out of 22 embolization procedures (31%) more than a single embolic agent was used; Gelfoam (Pfizer, New York, NY, USA) was used in 18 procedures (81%), in 12 of which as the single agent, followed by Embosphere (Merit Medical, South Jordan, UT, USA) (31%), Onyx (Medtronic, Minneapolis, MN, USA) (9%), and polyvynil alcohol (PVA) and Bead Block (Boston Scientific, Marlborough, MA, USA) in 4,5% each. The most common vessel involved was the ascending pharyngeal artery, involved in 90% of the patients, followed by the posterior auricular artery in 15%, the internal maxillary artery or the occipital artery in 10% each, and the superficial temporal or the lingual arteries, with 6% each. Only one patient had involvement of the internal carotid artery. No complications from embolization were recorded. Conclusions Preoperative embolization of glomus tumors is safe and reduces surgical time and complications, due to the decrease in size and bleeding.

Preservation of Cranial Nerves Function in Glomus Jugulare Surgery: 2-Dimensional Operative Video.

Owing to their invasive character, extreme vascularity, and critical location, glomus jugulare tumors present a formidable challenge. Techniques have been developed for safe and successful removal of even giant glomus paragangliomas.1-3 Preoperative evaluation including genetic, hormonal, and multiplicity workup4 has enhanced the safety of surgical management, as did modern preoperative embolization by eliminating excessive blood loss.5 Despite these advancements, surgical outcomes of glomus jugulare remain haunted by cranial nerve dysfunction such as facial nerve palsies and hearing loss, with lower cranial nerves dysfunction being the most morbid. These can be avoided by technical maneuvers to preserve the cranial nerves. The external ear canal is not closed to maintain conductive hearing. The facial nerve is not transpositioned and kept inside a bony protective canal. Cranial nerves IX, X, and XI are the most vulnerable because they pass through the jugular foramen ventral to the venous bulb wall. They are preserved by intrabulbar dissection that maintains a protective segment of the venous wall over the nerves. By mastering the anatomy of the upper neck, meticulous dissection is performed to preserve the course of IX, X, XI, and XII.6,7 Ligation of the jugular vein is delayed until the tumor is totally isolated to avoid diffuse bleeding.7 We present the case of a 60-yr-old woman with a glomus jugulare tumor with intradural, extradural, and cervical extension. The technical nuances of cranial nerves preservation are demonstrated. The patient consented to the procedure and publication of her images. Images at 2:12, 2:50, and 3:09 from Al-Mefty and Teixeira,6 with permission from JNSPG.

Glomus jugulare in a pediatric patient: case report and literature review.

PURPOSE: The jugular and tympanic glomus are rare neoplasms in the general population, being even more uncommon in the pediatric population. There is considerable morbidity associated with both disease and treatment. Treatment is essentially surgical, carried out in recent years in a multidisciplinary manner using preoperative embolization associated with microsurgery and eventually adjuvant radiotherapy. The outcome depends on the location of the lesion and its proximity to noble structures in addition to multidisciplinary monitoring in the postoperative period. METHODS: In this article, a literature review was carried out in the PubMed database, finding reports from 17 patients diagnosed with the disease. Only articles in English were considered. RESULTS: Moreover, we reported a case of a 14-year-old patient diagnosed with jugulotympanic glomus who underwent radical surgical treatment of the lesion. CONCLUSION: This is a rare case of jugulotympanic glomus in a pediatric patient, who underwent surgical treatment associated with multidisciplinary therapy, with a favorable postoperative outcome.

Anaplastic Hemangiopericytoma of the Jugular Foramen: Case Report and Systematic Review.

BACKGROUND: Hemangiopericytomas (HPCs) and solitary fibrous tumors (SFTs) are rare tumors of mesenchymal origin. Here, the authors present a rare case of anaplastic HPC in the jugular foramen (JF). The authors also conduct a systematic review of the literature to examine the current fund of knowledge on JF HPC/SFTs. METHODS: A systematic MEDLINE search was conducted using key words "hemangiopericytoma" OR "solitary fibrous tumor" AND "jugular foramen" OR "extracranial" OR "skull base." Clinicopathologic characteristics and outcomes of the present case were reviewed and compared with those in the literature. RESULTS: A 41-year-old male, who had undergone stereotactic radiation therapy 6 years ago for a presumed glomus jugulare tumor, presented to our institution with worsening dysphagia, hoarseness, persistent tongue weakness, and radiographic evidence of tumor progression. The patient underwent uncomplicated gross total resection with sacrifice of the infiltrated hypoglossal nerve. Histopathologic evaluation revealed anaplastic HPC/SFT (World Health Organization grade III). Review of the literature yielded 9 additional cases of JF HPC/SFT in 5 males (56%) and 4 females (44%), with a mean age of 49.6 years old. Patients commonly presented with pain (37.5%) and lower cranial nerve deficits (100%). Preoperative diagnoses included glomus jugulare (n = 2) or JF schwannomas (n = 3). All patients underwent microsurgical resection of the lesion, except for 1 who refused all treatment after diagnostic biopsy. CONCLUSION: The authors present the only reported case of anaplastic HPC of the JF. The illustrative case and those found on systematic review of the literature highlight the importance of tissue diagnosis and appropriate management.

Incidentally Detected 18F-PSMA Uptake in Glomus Jugulare.

PSMA (prostate-specific membrane antigen) PET/CT scan is the imaging modality of choice for staging and restaging of carcinoma prostate. Although initially thought to be a tracer with high specificity for prostatic tissue, with its extensive clinical use, there has been a rise in published literature citing its uptake in nonprostatic conditions. We present a case where false-positive PSMA uptake was noted in glomus jugulare during staging workup for carcinoma prostate.

Tumor de glomus yugulotimpánico, a propósito de un caso / Jugulotympanic glomus tumor, case presentation

Resumen Los tumores de glomus yugulo-timpánico o paragangliomas son originados de células de la cresta neural y representan un número bajo de los tumores de cabeza y cuello (0,5 %). Su crecimiento lento y síntomas progresivos provocan que se retarde el diagnóstico. Conocer la clínica que frecuentemente cursa con pérdida auditiva unilateral, tinnitus pulsátil y afectación de algún par craneal, puede ayudar a sospechar el diagnóstico de glomus. Se mencionan diferentes tipos de métodos diagnósticos, así como nuevas opciones de tratamiento. Dada la localización y su importante vascularización, hay casos en los que la cirugía abierta es muy riesgosa y se buscan procedimientos terapéuticos que disminuyan las complicaciones y morbilidades en estos pacientes, tanto como procedimientos endovasculares. En el artículo se discute el caso de una femenina de 31 años con clínica de inicio insidioso con hipoacusia izquierda progresiva, tinnitus pulsátil izquierdo y disfonía. En 2013, se realiza resonancia magnética nuclear donde se observa proceso infiltrativo tumoral sugestivo de paraganglioma. Se hace embolización endovascular con onyx de glomus yugulotimpánico en cuatro ocasiones, con seguimiento cada 6 meses por no ser candidata a otro tipo de tratamiento, debido al tamaño y localización de la masa y su condición no resecable.

Abstract Jugulo-tympanic glomus tumors or paragangliomas originate from neural crest cells and represent a low number of head and neck tumors (0.5%). Its slow growth and progressive symptoms cause the diagnosis to be delayed. The knowledge of the clinic that frequently involves unilateral hearing loss, pulsatile tinnitus and cranial nerve involvement can help to suspect the diagnosis. Different types of diagnostic methods are helpfun, as well as new treatment options have been proposed. Given the location and its important vascularization, there are cases in which open surgery is highly risky and alternative therapeutic procedures trying to reduce complications and morbidities in these patients need to be considered, as well as endovascular procedures. This article discusses the case of a 31-year-old female with an insidious onset of progressive left hearing loss, left pulsatile tinnitus and dysphonia. In 2013, nuclear magnetic resonance imaging was performed and a tumor infiltrative process suggesting paraganglioma was observed. Endovascular embolization was performed with onyx of jugulotympanic glomus on four occasions, with follow-up every 6 months, due to the size and location of the mass and its unresectable condition the tumor was considered non surgically resectable.

Magnetic Resonance Imaging Features of Collet-Sicard Syndrome Associated With Glomus Jugulare Paraganglioma.

Collet-Sicard syndrome is an unusual disorder. The authors here demonstrated the Magnetic resonance (MR) imaging findings of the Collet-Sicard syndrome associated with glomus jugulare tumor. Neoplastic or non-neoplastic lesion of skull base can cause Collet-Sicard syndrome. MR imaging can be used successfully to demonstrate the etiology of this syndrome.

Cirurgia híbrida na exérese de tumor glômico Shamblin II / Hybrid surgery in excision of a Shamblin II glomus tumor

O tumor glômico é uma neoplasia benigna rara originada de células paraganglionares da crista neural que se desenvolve na camada adventícia do vaso. São tumores não encapsulados e altamente vascularizados. Paciente feminina, 64 anos, foi diagnosticada com tumor glômico hipervascularizado com 5 cm posteriormente à bifurcação carotídea esquerda e oclusão de carótida contralateral. Optou-se por realizar embolização através de acesso endovascular seguida de punção percutânea direta, guiada por angiografia, para preenchimento da área remanescente. Após embolização, realizou-se a exérese cirúrgica do tumor com menor sangramento e maior facilidade para encontrar o plano de clivagem das estruturas adjacentes. Em acompanhamento tardio, a paciente apresenta-se sem recidiva tumoral. O tumor foi classificado como pertencente ao grupo Shamblin II, o qual inclui tumores apresentando de 4 a 6 cm com inserção arterial moderada. Através dessa dupla abordagem, foi possível notar uma redução relativa do sangramento intraoperatório e facilitação de identificação do plano de clivagem, colaborando para sua exérese e evitando o pinçamento cirúrgico

Glomus tumors are rare benign neoplasms originating from paraganglionic cells of the neural crest developing in the adventitious layer of the vessel. They are nonencapsulated and highly vascularized. A 64-year-old female patient was identified with a hypervascularized glomus tumor measuring 5 cm, posterior to the left carotid bifurcation and contralateral carotid occlusion. We performed preoperative embolization via endovascular access followed by direct percutaneous puncture, guided by angiography, to fill the remaining area. After embolization, surgical excision of the tumor was performed with reduced bleeding and it was easier to find the cleavage planes to adjacent structures. At late follow-up, the patient is free from tumor recurrence. The tumor was classified as Shamblin II, measuring 4 to 6 cm with moderate arterial insertion. Through this double approach we observed a relative reduction in intraoperative bleeding and improved identification of the cleavage plane, facilitating excision and avoiding surgical clamping

Serie de casos: Tumores del Golfo de la yugular / Case series: Glomus jugulare tumors

Introducción: Los tumores del foramen yugular son lesiones de la base del cráneo poco frecuentes, que presentan dificultades tanto para el diagnóstico como para el manejo. El objetivo del presente reporte es presentar una serie de casos y el manejo que se dio a estos pacientes. Métodos: El presente estudio descriptivo incluye pacientes con tumores del foramen yugular manejados quirúrgicamente desde el 2002 hasta 2006 en el Hospital Oncológico "Solón Espinosa Ayala", donde se revisó la técnica quirúrgica y el pronóstico. Los tumores fueron abordados por un mismo equipo multidisciplinario, neurocirujano, cirujano otorrinolaringólogo y radio-oncólogo. Resultados: Se presentan 10 casos. El nervio facial fue reconstruido con injertos del nervio auricular mayor o con anastomosis en cinco casos. Los paragangliomas fueron las lesiones más frecuentes seguidas por Schwannomas y meningiomas. La escisión completa fue posible en 8 casos de tumores benignos y en 4 paragangliomas. Los nervios craneales bajos fueron los más frecuentemente comprometidos con lesiones transitorias en cuatro pacientes. Y la función del nervio facial se recobró espontáneamente en 3 casos. Dos casos desarrollaron fístula de líquido céfalo raquídeo. Un paciente falleció después de la cirugía. Conclusión: Los paragangliomas con extensión intracraneal deben ser radicalmente resecados a través de abordajes multidisciplinarios con preservación de los nervios. La embolización preoperatoria debería considerarse. La extubación precoz, traqueotomía en casos seleccionados y temprana rehabilitación son muy importantes en el manejo postoperatorio.

Introduction: Jugular foramen tumors are rare lesions of the base of the skull, which present difficulties for both diagnosis and management. The objective of this report is to present a series of cases and the management that was given to these patients. Methods: The present descriptive study includes patients with jugular foramen tumors managed surgically from 2002 to 2006 in the "Solón Espinosa Ayala" Oncological Hospital, where the surgical technique and prognosis were reviewed. The tumors were treated by the same multidisciplinary team, neurosurgeon, otolaryngologist and radiation oncologist. Results: 10 cases are presented. The facial nerve was reconstructed with grafts of the greater auricular nerve or with anastomosis in five cases. Paragangliomas were the most frequent lesions followed by Schwannomas and meningiomas. Complete excision was possible in 8 cases of benign tumors and in 4 paragangliomas. Low cranial nerves were the most frequently compromised with transient lesions in four patients. And the function of the facial nerve recovered spontaneously in 3 cases. Two cases developed cerebrospinal fluid fistula. One patient died after surgery. Conclusion: Paragangliomas with intracranial extension must be radically resected through multidisciplinary approaches with preservation of the nerves. Preoperative embolization should be considered. Early extubation, tracheotomy in selected cases and early rehabilitation are very important in postoperative management.

Sorpresa diagnóstica en síndrome menièriforme: Bulbo yugular dehiscente / Diagnostic surprise in a Menière-like syndrome: Jugular bulb diverticulum

No disponible

Evaluation of osteologic parameters of jugular foramen and its significance with respect to expanded endoscopic endonasal approach

The jugular foramen (JF) is a large irregular hiatus, lies at the posterior end of the petro-occipital suture, posterior to the opening of carotid canal (CC) and it transmits major neurovascular structures. Tumors are the most common pathology involving structures present in JF. In the current scenario, lesions of structures present in JF progressing towards midline are suitably removed by expanded endoscopic endonasal approach (EEEA). In lieu of EEEA, we studied new parameters in relation to JF. The study was done on 50 human dry skulls with the help of sliding Vernier caliper and statistical analysis was done using SPSS software. Morphological study on the presence of dome, septa and relation of CC to JF was done. Morphometric parameters of JF and its distance from vomer, medial pterygoid plate (MPP), lateral pterygoid plate (LPP) and CC were studied. We also determined the distance from lateral and medial end of CC to the midsagittal plane (MSP). The dome of the jugular fossa (JFo) was seen in 42% skulls, each bilaterally and unilaterally. Complete and incomplete septa in JF were seen in 4% & 16% bilaterally and 8% & 18% unilaterally respectively. In the majority of the skulls, position of CC was anterior to JF and anteromedial was the next common position seen. Length & width of JF, depth of JFo, distance of JF from vomer, MPP, LPP & CC and the distance of CC to the MSP were more on the right side. This study may help neurosurgeons & ENT surgeons while approaching lesions around JF by EEEA

No disponible

Natural history of glomus jugulare: a review of 16 tumors managed with primary observation.

OBJECTIVE: To characterize clinical disease progression and radiologic growth in a series of observed, previously untreated, glomus jugulare tumors (GJT). STUDY DESIGN: Retrospective review. SETTING: Tertiary neurotologic referral center. SUBJECTS AND METHODS: All patients with primary GJTs that were observed without intervention for a minimum of 2 years. Primary outcome measures included progression of cranial neuropathy and/or radiologic growth. RESULTS: A total of 15 patients (80% female; median age, 69.6 years) with 16 GJTs met inclusion criteria. The most common indications for observation included advanced age (11; 73%) and patient preference (11; 73%). Cranial nerve function remained stable in most subjects over a median clinical follow-up period of 86.4 months. Among the 12 with serial imaging, 5 (42%) GJTs demonstrated radiologic growth, while 7 (58%) remained stable. The median growth rate of the 5 enlarging tumors using the maximum linear dimension was 0.8 mm/y (range, 0.6-1.6 mm/y) or 0.4 cm(3)/y (0.1-0.9 cm(3)/y) using volumetric analysis. There were no deaths attributable to tumor progression or treatment. CONCLUSION: In an older subset of patients, we found that a significant number of GJTs do not grow after time of diagnosis and symptoms frequently remain stable for many years. Even with disease progression, most GJTs exhibit indolent growth with slowly progressive cranial neuropathy, affording satisfactory physiologic compensation in most patients. In the absence of brainstem compression or concern for malignancy, observation of GJTs is a viable initial management option for elderly patients.

Modulation of BK channel activities by calcium-sensing receptor in rat bronchopulmonary sensory neurons.

This study was carried out to investigate the expression of large-conductance Ca(2+)-activated potassium (BK) channels and to explore the possible modulation of BK channel activities by calcium-sensing receptors (CaSR) in rat bronchopulmonary sensory neurons. The expression of BK channels was demonstrated by immunohistochemistry and RT-PCR. Results from whole-cell patch-clamp recordings demonstrated that activation of CaSR with its agonist spermine or NPS R-568 showed a dual regulating effect on BK channel activities: it potentiated BK currents in cells exhibiting low baseline BK activity while slightly inhibited BK currents in cells with high baseline BK activity. Blocking CaSR with its antagonist NPS 2143 significantly inhibited BK currents. Our results further showed that the modulation of BK currents by CaSR activation or blockade was completely abolished when the intracellular Ca(2+) was chelated by BAPTA-AM. In summary, our data suggest that CaSR plays an integrative role in bronchopulmonary afferent signaling, at least partially through the regulation of BK channel activities.

Radiotherapy for glomus jugulare paraganglioma.

Surgery has been long considered to be the treatment of choice for glomus jugulare paragangliomas, as it is the only modality able to totally eradicate the tumour. However, despite considerable progress in interventional radiology and nerve monitoring, surgery is associated with an unacceptably high complication rate for a benign tumour, explaining the growing place of radiotherapy in the management of these tumours. This review of the literature confirms the efficacy of conformal radiotherapy with or without intensity modulation and stereotactic radiotherapy, which both achieve tumour control rates ranging from 90% to almost 100% of cases, but for different tumour volumes, almost constant stabilization or even improvement of symptoms, and a considerably lower rate of adverse effects than with surgery. However, radiotherapy remains contraindicated in the presence of intracranial invasion or extensive osteomyelitis. In the light of these results, together with the improved quality of life and a better knowledge of the natural history of this disease, many authors propose radiotherapy as first-line treatment for all glomus jugulare paragangliomas regardless of their size, particularly in patients with no preoperative deficits.

Successful Onyx embolization of a giant glomus jugulare: case report and review of nonsurgical treatment options.

BACKGROUND: Otorrhagia is an uncommon but severe symptom of patients with large glomus jugulare tumors that erode through the tympanic membrane. In this case report we describe the use of transarterial embolization for long-term palliative management of otorrhagia in a patient with an unresectable glomus jugulare tumor. CASE DESCRIPTION: A 53-year-old woman presented with intermittent otorrhagia 10 years after subtotal resection of a glomus jugulare tumor. Follow-up magnetic resonance imaging showed progressive enlargement of the tumor with significant extension into the posterior fossa. Resection was thought to be impractical; therefore, transarterial embolization was offered as a palliative measure to help reduce the frequency and severity of bleeding episodes. RESULTS: Long-term control of otorrhagia was achieved after three rounds of intra-arterial embolization. In round one, the tumor was embolized from multiple external carotid artery feeding branches by the use of polyvinyl alcohol particles. In round two, the internal carotid artery was sacrificed by embolizing the cavernous and petrous segments with coils. In round three, persistent feeders from the cervical internal carotid artery were embolized with Onyx. In 6 years of clinical follow-up, the patient has had no otorrhagia or new neurological deficits. Serial magnetic resonance imaging revealed there has been no significant interval tumor growth. CONCLUSION: Long-term control of otorrhagia from glomus jugulare tumors can be safely achieved by intravascular embolization with Onyx.

Clinical Features and Surgical Outcomes of Glomus Tumors in the Temporal Bone and Skull Base / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Glomus tumors are benign, slow-growing tumors which cause various symptoms such as pulsatile tinnitus and hearing loss. The aim of this study was to analyze the clinical manifestation and surgical outcomes of the glomus tumors in the temporal bone and skull base. SUBJECTS AND METHOD: Medical records of 17 patients who were surgically managed for glomus tumors of the temporal bone and skull base from 1997 to 2013 were retrospectively reviewed. The location and the size of the tumor were analyzed by CT and MRI, and the Fisch classification method was used to classify the tumor. Pre- and postoperative facial function measured by House-Brackmann (H-B) grade and lower cranial nerve (LCN) function were collected. RESULTS: The mean age of 17 patients was 50.6 (18-83) years, and male to female ratio was 4:13. The most common symptom of glomus tumors was pulsatile tinnitus, and this symptom disappeared or changed naturally postoperatively. The majority of patients with glomus jugulare showed various degrees of immediate postoperative facial nerve palsy, and 8 out of 9 patients showed improvement of facial nerve function to H-B grade I in 3 months. LCN palsy occurred in 5 out of 9 patients immediately following the operation and remained in 3 patients with Fisch type C2 tumor. CONCLUSION: Glomus tumors of the temporal bone and skull base were successfully removed by surgery. The surgical outcome of glomus jugulare shows that facial nerve function is acceptable and the incidence of lower cranial nerve palsy may be related to the classification of tumor.

Glomus jugulare: high index of clinical suspicion is important for optimal management.

Glomus tumour is a type of extra-adrenal chemodectoma or paraganglioma, originating within the wall of jugular bulb. We report a case of a 60-year-old woman who presented with hearing loss and pulsatile tinnitus. High index of suspicion and appropriate use of imaging led to early diagnosis. However, the patient did not give consent for surgery and was managed satisfactorily with radiotherapy.

[Complications and pitfalls in surgery of the ear/lateral skull base]. / Fehler und Gefahren: Ohrchirurgie und Chirurgie der lateralen Schädelbasis.

Surgery of the ear and the lateral skull base is a fascinating, yet challenging field in otorhinolaryngology. A thorough knowledge of the associated complications and pitfalls is indispensable for the surgeon, not only to provide the best possible care to his patients, but also to further improve his surgical skills.Following a summary about general aspects in pre-, intra- and postoperative care of patients with disorders of the ear/lateral skull base, this article covers the most common pitfalls and complications in stapes surgery, cochlear implantation, surgery of vestibular schwannomas, and jugulotympanal paragangliomas. Based on these exemplary procedures, basic "do's and don'ts" of skull base surgery are explained, which the reader can easily transfer to other disorders. Special emphasis is laid on functional aspects, such as hearing, balance and facial nerve function. Furthermore, the topics of infection, bleeding, skull base defects, quality of life and indication for revision surgery are discussed.An open communication about complications and pitfalls in ear/lateral skull base surgery among surgeons is a prerequisite for the further advancement of this fascinating field in ENT surgery. This article is meant to be a contribution to this process.