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.

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.

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.

[The arrest of bleeding from the sigmoid sinus and the upper segments of internal jugular vein].

The objective of the present work was to develop an efficacious non-injurious technique for the arrest of hemorrhage from the sigmoid sinus and the upper segments of the internal jugular vein. The authors proposed a relatively simple and highly effective method to be used to the blockade of the sigmoid sinus. It was employed to treat 8 patients none of whom suffered complications in the late postoperative period (the follow-up study lasting from 6 months to 2 years after the intervention).

[Diagnosis and treatment of chordoma in the jugular foramen area:three cases report].

OBJECTIVE: To discuss the early diagnosis and the treatment of chordoma in the jugular foramen area. METHODS: Three cases of chordoma in the jugular foramen area were diagnosis and reviewed retrospectively. The initial symptom was facial paralysis. Surgical removal was performed through the infratemporal fossa approach in 1 case, through petro-occipital approach in 2 cases. For repairing facial nerve defect, great auricular nerve was used in 1 case and facial-hypoglossal anastomosis was used in another 2 cases. RESULTS: The tumor were totally removed in all 3 cases. The patients were follow-up for 6 months to 1 year and no recurrence was found. The function of facial nerve was II grade (House-Brackmann) in one patient and IV grade in another 2 patients. No complications occurred in 3 cases. CONCLUSIONS: Although chordoma originating from the jugular foramen area were extremely rare, it was possible to make early diagnosis through finding clinical feature and imaging methods. The final diagnosis depended on pathology. Generally, the different surgical approaches were used according to the size and position of the tumor. The classic infratemporal fossa approach for tumor removal and facial nerve reconstruction should be considered in the patients with chordoma around jugular foramen.

Aporte de las imágenes en el diagnóstico de los paragangliiomas de cabeza y cuello / Radiologic support for the diagnosis of paragangliomas of the head and neck

Los paragangliomas de cabeza y cuello son tumores que derivan de las células neuroendócrinas de los paraganglios extrasuprarrenales y se extienden desde la base de cráneo hasta el cayado aórtico. El pronóstico es en general favorable, excepto los que se ubican en el hueso temporal. En el presente trabajo se describen las características en imágenes de las diferentes localizaciones y subtipos de este tumor, en conjunto con una revisión de la literatura hasta la actualidad. Los métodos por imágenes son muy útiles para poder arribar al diagnóstico. El diagnóstico diferencial es de vital importancia a la hora de evitar procedimientos invasivos riesgosos y contraindicados en estos pacientes, como la biopsia

Aporte de las imágenes en el diagnóstico de los paragangliiomas de cabeza y cuello / Radiologic support for the diagnosis of paragangliomas of the head and neck

Los paragangliomas de cabeza y cuello son tumores que derivan de las células neuroendócrinas de los paraganglios extrasuprarrenales y se extienden desde la base de cráneo hasta el cayado aórtico. El pronóstico es en general favorable, excepto los que se ubican en el hueso temporal. En el presente trabajo se describen las características en imágenes de las diferentes localizaciones y subtipos de este tumor, en conjunto con una revisión de la literatura hasta la actualidad. Los métodos por imágenes son muy útiles para poder arribar al diagnóstico. El diagnóstico diferencial es de vital importancia a la hora de evitar procedimientos invasivos riesgosos y contraindicados en estos pacientes, como la biopsia

Jugular foramen schwannomas: diagnosis, management, and outcomes.

OBJECTIVES/HYPOTHESIS: To describe the presentation, radiographic findings, and surgical management of seven patients who have been diagnosed and treated with jugular foramen schwannomas at the University of Utah. STUDY DESIGN: Retrospective chart review. METHODS: The charts of seven patients diagnosed with jugular foramen schwannomas were reviewed for presentation symptoms, radiographic findings, and physical examination findings. For the six who underwent surgical excision, the surgical procedure used, cranial nerve function results, audiometric results, perioperative complications, and other follow-up data are presented. RESULTS: Seven patients were identified from ages 24 to 69 years. Six of the seven underwent surgical excision. Primary presentation symptoms included dizziness, hearing loss, dysphagia, diplopia, tongue paresis, and hoarseness. The choice of surgical approach was based on the size and location of the tumor. All patients had complete excision of their tumors. The nerve of origin included the glossopharyngeal, vagus, and spinal accessory nerves. Preoperative cranial nerve dysfunction continued postoperatively for lower cranial nerves but resolved in patients who were noted to have preoperative dysfunction of cranial nerve V and VI. The rate of new lower cranial nerve injury was 15% and was only seen in the cranial nerves that were determined to be the nerve of origin. In two cases, a temporary feeding tube was required. No recurrences have been noted to date. CONCLUSIONS: Jugular foramen schwannomas can be successfully diagnosed preoperatively with computed tomography and magnetic resonance imaging. These tumors can be successfully managed with surgery and low morbidity.

Tumors of the jugular foramen: diagnosis and management.

OBJECTIVE: Jugular foramen tumors are rare cranial base lesions that present diagnostic and management difficulties. With the availability of new diagnostic procedures these tumors have been more precisely studied and questions of whether, when, and how these lesions should be treated often arise. Data from 106 consecutive patients surgically treated in the past 17 years were retrospectively analyzed to identify surgical outcomes. METHODS: The tumors were approached by a multidisciplinary team composed of neurosurgeons, ear, nose, and throat surgeons, and neuroradiologists. Hypervascular lesions were embolized 3 to 5 days before surgery. The same surgical technique was used to resect all tumors. The surgical defect was covered with vascularized myofascial flaps. The internal carotid artery was infiltrated in two patients, and a saphenous graft bypass was carried out before removal of the lesions. The facial nerve was reconstructed with nerve grafts (great auricular nerve) or XII/VII anastomosis in five cases. Postoperative radiotherapy was carried out for malignant and invasive tumors. RESULTS: Paragangliomas were the most frequent lesions, followed by schwannomas and meningiomas. Complete excision was possible in 89% of benign tumors and 80% of paragangliomas. Lower cranial nerve deficit was the most frequent complication (10 patients, 9.4%), transient in 4 patients. Facial and cochlear nerve paralysis occurred in 8 patients (7.5%). The function of the facial nerve recovered spontaneously in 3 patients. Four patients (3.7%) developed postoperative cerebrospinal fluid leakage. Four patients (3.7%) died after surgery. CONCLUSION: Radical removal of benign jugular foramen tumors is the treatment of choice and may be curative. Large lesions can be radically excised in one surgical procedure with preservation of lower cranial nerves. Cranial base reconstruction with vascularized myofascial flaps reduces the incidence of postoperative cerebrospinal fluid leakage. Damage of the lower cranial nerves is the most serious surgical complication.

Anatomic landmarks of the glossopharyngeal nerve: a microsurgical anatomic study.

OBJECTIVE: Compared with other lower cranial nerves, the glossopharyngeal nerve (GPhN) is well hidden within the jugular foramen, at the infratemporal fossa, and in the deep layers of the neck. This study aims to disclose the course of the GPhN and point out landmarks to aid in its exposure. METHODS: The GPhN was studied in 10 cadaveric heads (20 sides) injected with colored latex for microsurgical dissection. The specimens were dissected under the surgical microscope. RESULTS: The GPhN can be divided into three portions: cisternal, jugular foramen, and extracranial. The rootlets of the GPhN emerge from the postolivary sulcus and course ventral to the flocculus and choroid plexus of the lateral recess of the fourth ventricle. The nerve then enters the jugular foramen through the uppermost porus (pars nervosa) and is separated from the vagus and accessory nerves by a fibrous crest. The cochlear aqueduct opens to the roof of this porus. On four sides in the cadaver specimens (20%), the GPhN traversed a separate bony canal within the jugular foramen; no separate canal was found in the other cadavers. In all specimens, the Jacobson's (tympanic) nerve emerged from the inferior ganglion of the GPhN, and the Arnold's (auricular branch of the vagus) nerve also consisted of branches from the GPhN. The GPhN exits from the jugular foramen posteromedial to the styloid process and the styloid muscles. The last four cranial nerves and the internal jugular vein pass through a narrow space between the transverse process of the atlas (C1) and the styloid process. The styloid muscles are a pyramid shape, the tip of which is formed by the attachment of the styloid muscles to the styloid process. The GPhN crosses to the anterior side of the stylopharyngeus muscle at the junction of the stylopharyngeus, middle constrictor, and hyoglossal muscles, which are at the base of the pyramid. The middle constrictor muscle forms a wall between the GPhN and the hypoglossal nerve in this region. Then, the GPhN gives off a lingual branch and deepens to innervate the pharyngeal mucosa. CONCLUSION: Two landmarks help to identify the GPhN in the subarachnoid space: the choroid plexus of the lateral recess of the fourth ventricle and the dural entrance porus of the jugular foramen. The opening of the cochlear aqueduct, the mastoid canaliculus, and the inferior tympanic canaliculus are three landmarks of the GPhN within the jugular foramen. Finally, the base of the styloid process, the base of the styloid pyramid, and the transverse process of the atlas serve as three landmarks of the GPhN at the extracranial region in the infratemporal fossa.

Paragangliomas yúgulotimpánicos. Presentación de 5 casos y revisión de la literatura / Jugulotympanic paragangliomas. Presentation of five cases and bibliographic review

Presentamos 5 casos clínicos de pacientes diagnosticados de Paraganglioma yúgulotimpánico por nuestro Servicio de ORL entre los años 1992 y 2000. Cuatro eran mujeres frente a un único varón, con una edad media de 56 años. Analizamos las manifestaciones clínicas de los pacientes, exploraciones complementarias solicitadas y tratamiento administrado. Finalmente, realizamos una revisión bibliográfica sobre esta patología (AU)

Efficiency of gamma knife radiosurgery in the treatment of glomus jugulare tumors.

This study was performed to assess the impact of gamma knife radiosurgery (RS) in the treatment of glomus jugulare tumors. Between February 1993 and February 1999, thirteen patients (9 women, 4 men; mean age 63.5 years, range 29 to 79 years) underwent stereotactic radiosurgery for glomus jugulare tumors with the Leksell Gamma Knife at the Neurosurgical Department of the University of Vienna. Four patients, mean age 74.5 years, range 67 to 79 years, underwent radiosurgery as the only treatment. Nine patients received radiosurgery as adjuvant therapy after previous treatment had failed: surgical resection in 9 patients and additional fractionated external beam radiation in two of these patients. Pretreatment evaluation included the staging of all tumors according to the Fisch Classification: De1 (7), De2 (1), Di1 (4) and Di2 (1). The mean follow-up period was 4.2 years, range 0.7 to 6.7 years. Ten patients, 77 %, were treated prior to 1997, the mean follow-up period being 5 years. Six patients showed no clinical changes, while six experienced an improvement of their clinical symptoms and neurological deficits. One patient was lost to follow-up. Radiation-induced transient cranial nerve neuropathies were observed in two patients. Serial MRI scans revealed tumor control in all patients, with unaltered tumor size in 10 and shrinkage in three patients. The results indicate that RS is an attractive treatment option for glomus jugulare tumors and will occupy an increasingly important role in the management of these tumors in selected patients.