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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.

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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.

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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.

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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.

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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

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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.

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Introducción: Con el refinamiento de la técnica quirúrgica en el manejo de los tumores glómicos yugulotimpánicos la meta actual no sólo es conseguir la resección tumoral sino también la preservación funcional.Objetivos: Evaluar los resultados respecto a erradicación de la enfermedad y preservación de la función facial y auditiva en pacientes con tumores glómicos yugulo-timpánicos manejados mediante la técnica del muro del facial intactoTipo de estudio: Serie de casos (n=17)Resultados: La serie consta de 16 mujeres y un hombre con edad media de 48,6 años. El seguimiento mediofue de 17,5 meses (DE = 12,8). Todos los casos tuvieron función facial normal en el preoperatorio y elresultado al momento de la última valoración, escala House-Brackmann, mostró buena conservación funcional, HB I-II en 77 por ciento (10/13). También se logró buena conservación de la función auditiva, 88,9 por ciento (8/9). Los mayores porcentajes de neuropatía posoperatoria se encontraron con el X PC. No hubo casos de mortalidad y sólo uno de recidiva tumoral (8 por ciento). Se comparan los resultados con series ya publicadas, conotras técnicas quirúrgicas, observando, resección tumoral, preservación facial y auditivas comparablesConclusiones: La técnica descrita es una alternativa de manejo razonable para estos tumores, con buenosporcentajes de curación y de preservación funcional.

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Los tumores del glomus yugular o timpánicos son poco comunes. Aunque son considerados tumores benignos, de crecimiento lento y tratamiento quirúrgico, pueden llegar a adquirir grandes dimensiones. El pronóstico de estos tumores está relacionado estrechamente con su localización anatómica y el volumen de la lesión. Si la remoción quirúrgica no es posible la radioterapia constituye una alternativa de tratamiento válida con intenciones paliativas.

The glomus juglare tumors are very uncommon. They are considered as benign and slow growing tumors. The treatment of choice is surgery. The prognosis of these tumors is related to the anatomical location and size of the lesion. If surgery is not posible the next step would be radiotherapy.

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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

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