Guía de práctica clínica sobre el manejo del schwannoma vestibular / Clinical practice guideline on the management of vestibular schwannoma

Introducción: El schwannoma vestibular (SV) es el tumor más frecuente del ángulo pontocerebeloso. La mayor accesibilidad a las pruebas radiológicas ha incrementado su diagnóstico. Teniendo en cuenta las características del tumor, la clínica y la edad del paciente se han propuesto tres estrategias terapéuticas, observación, cirugía o radioterapia. La elección de la más adecuada para cada paciente es un motivo de controversia frecuente. Material y métodos: El presente trabajo incluye una revisión exhaustiva sobre cuestiones relativas al SV que pueden servir de guía clínica en el manejo de pacientes con estas lesiones. La presentación se ha orientado en forma de preguntas que el clínico se hace habitualmente y las respuestas están redactadas y/o revisadas por un panel de expertos nacionales e internacionales consultados por la Comisión de Otología de la SEORL-CCC. Resultados: Se ha elaborado un listado con los 13 bloques temáticos más controvertidos sobre el manejo del SV en forma de 50 preguntas y se han buscado las respuestas a todas ellas mediante una revisión sistemática de la literatura (artículos publicados en PubMed y Cochrane Library entre 1992 y 2023 sobre cada bloque temático). Treinta y tres expertos, liderados por la Comisión de Otología de la SEORL-CCC, han analizado y discutido todas las respuestas. En el Anexo 1 pueden encontrarse 14 preguntas adicionales divididas en cuatro bloques temáticos. Conclusiones: Esta guía de práctica clínica sobre el manejo del SV ofrece respuestas consensuadas a las preguntas más habituales que se plantean sobre este tumor. La ausencia de suficientes estudios prospectivos hace que los niveles de evidencia sobre el tema sean en general medios o bajos. Este hecho incrementa el interés de este tipo de guías de práctica clínica elaboradas por expertos.(AU)

IntroductionVestibular schwannoma (VS) is the most common tumour of the cerebellopontine angle. The greater accessibility to radiological tests has increased its diagnosis. Taking into account the characteristics of the tumour, the symptoms and the age of the patient, three therapeutic strategies have been proposed: observation, surgery or radiotherapy. Choosing the most appropriate for each patient is a frequent source of controversy. Material and methods: This paper includes an exhaustive literature review of issues related to VS that can serve as a clinical guide in the management of patients with these lesions. The presentation has been oriented in the form of questions that the clinician usually asks himself and the answers have been written and/or reviewed by a panel of national and international experts consulted by the Otology Commission of the SEORL-CCC. Results: A list has been compiled containing the 13 most controversial thematic blocks on the management of VS in the form of 50 questions, and answers to all of them have been sought through a systematic literature review (articles published on PubMed and Cochrane Library between 1992 and 2023 related to each thematic area). Thirty-three experts, led by the Otology Committee of SEORL-CCC, have analyzed and discussed all the answers. In Annex 1, 14 additional questions divided into 4 thematic areas can be found. Conclusions: This clinical practice guideline on the management of VS offers agreed answers to the most common questions that are asked about this tumour. The absence of sufficient prospective studies means that the levels of evidence on the subject are generally medium or low. This fact increases the interest of this type of clinical practice guidelines prepared by experts.(AU)

Clinical practice guideline on the management of vestibular schwannoma.

INTRODUCTION: Vestibular schwannoma (VS) is the most common tumour of the cerebellopontine angle. The greater accessibility to radiological tests has increased its diagnosis. Taking into account the characteristics of the tumour, the symptoms and the age of the patient, three therapeutic strategies have been proposed: observation, surgery or radiotherapy. Choosing the most appropriate for each patient is a frequent source of controversy. MATERIAL AND METHODS: This paper includes an exhaustive literature review of issues related to VS that can serve as a clinical guide in the management of patients with these lesions. The presentation has been oriented in the form of questions that the clinician usually asks himself and the answers have been written and/or reviewed by a panel of national and international experts consulted by the Otology Commission of the SEORL-CCC. RESULTS: A list has been compiled containing the 13 most controversial thematic blocks on the management of VS in the form of 50 questions, and answers to all of them have been sought through a systematic literature review (articles published on PubMed and Cochrane Library between 1992 and 2023 related to each thematic area). Thirty-three experts, led by the Otology Committee of SEORL-CCC, have analyzed and discussed all the answers. In Annex 1, 14 additional questions divided into 4 thematic areas can be found. CONCLUSIONS: This clinical practice guideline on the management of VS offers agreed answers to the most common questions that are asked about this tumour. The absence of sufficient prospective studies means that the levels of evidence on the subject are generally medium or low. This fact increases the interest of this type of clinical practice guidelines prepared by experts.

Tratamiento quirúrgico del schwannoma vestibular. Revisión de 420 casos / Surgical treatment of vestibular schwannoma. Review of 420 cases

Introducción y objetivos: El schwannoma vestibular es el tumor más frecuente en el ángulo ponto-cerebeloso. El objetivo de nuestro estudio es reflejar nuestra experiencia en el tratamiento quirúrgico de este tumor. Material y métodos: Estudio retrospectivo de 420 schwannomas vestibulares intervenidos en nuestro centro entre 1994-2014. Se incluyen el tamaño tumoral, la audición preoperatoria, los abordajes quirúrgicos utilizados, el resultado definitivo de la función facial y auditiva y las complicaciones derivadas de la cirugía. Resultados: Un total de 417 pacientes con 420 tumores fueron analizados, siendo 209 mujeres (50,1%) y 208 varones (49,9%). La edad media fue de 49,8±13,2 años. La mayoría de los tumores se resecaron mediante abordaje translaberíntico (80,2%). La resección tumoral completa tuvo lugar en 411 tumores (98,3%), y la conservación de la integridad anatómica del nervio facial en 404 (96,2%). El resultado definitivo del facial fue grado I y II de House-Brackmann en el 69,9%, siendo significativamente mejor en los tumores de menos de 20mm. Entre las complicaciones se incluyen 3 casos de fístula (0,7%) y 16 acúmulos retroauriculares de líquido cefalorraquídeo (3,8%), 5 de meningitis (1,2%), 4 sangrados intracraneales (0,9%) y exitus en 3 pacientes (0,7%). Conclusiones: El tratamiento quirúrgico del schwannoma vestibular sigue siendo el de elección en la mayoría de los casos. En nuestra experiencia, la tasa de complicaciones es baja, siendo el tamaño tumoral el principal factor influyente en la función facial postoperatoria (AU)

Introduction and objectives: Vestibular schwannoma is the most frequent cerebellopontine angle tumor. The aim of our study is to reflect our experience in the surgical treatment of this tumor. Material and methods: Retrospective study of 420 vestibular schwannomas operated in our hospital between 1994-2014. We include tumor size, preoperative hearing, surgical approaches, definitive facial and hearing functional results, and complications due to surgery. Results: A total of 417 patients with 420 tumors were analyzed, 209 female (50.1%) and 208 male (49.9%). Mean age at diagnosis was 49.8±13.2 years. The majority of the tumors were resected through a translabyrinthine approach (80.2%). Total tumor removal was achieved in 411 tumors (98.3%), and anatomic preservation of facial nerve in 404 (96.2%). Definitive facial nerve outcome was House-Brackmann grade I and II in 69.9%, and was significantly better in tumors under 20mm. Surgical complications included cerebrospinal fluid leakage in 3 patients (0.7%) and retroauricular subcutaneous collection in 16 (3.8%), 5 cases of meningitis (1.2%), 4 patients with intracraneal bleeding (0.9%), and death in 3 patients (0.7%). Conclusions: Surgery is the treatment of choice for vestibular schwannoma in the majority of patients. In our experience, the complication rate is very low and tumor size is the main factor influencing postoperative facial nerve function (AU)

Surgical treatment of vestibular schwannoma. Review of 420 cases. / Tratamiento quirúrgico del schwannoma vestibular. Revisión de 420 casos.

INTRODUCTION AND OBJECTIVES: Vestibular schwannoma is the most frequent cerebellopontine angle tumor. The aim of our study is to reflect our experience in the surgical treatment of this tumor MATERIAL AND METHODS: Retrospective study of 420 vestibular schwannomas operated in our hospital between 1994-2014. We include tumor size, preoperative hearing, surgical approaches, definitive facial and hearing functional results, and complications due to surgery. RESULTS: A total of 417 patients with 420 tumors were analyzed, 209 female (50.1%) and 208 male (49.9%). Mean age at diagnosis was 49.8±13.2 years. The majority of the tumors were resected through a translabyrinthine approach (80.2%). Total tumor removal was achieved in 411 tumors (98.3%), and anatomic preservation of facial nerve in 404 (96.2%). Definitive facial nerve outcome was House-Brackmann grade I and II in 69.9%, and was significantly better in tumors under 20mm. Surgical complications included cerebrospinal fluid leakage in 3 patients (0.7%) and retroauricular subcutaneous collection in 16 (3.8%), 5 cases of meningitis (1.2%), 4 patients with intracraneal bleeding (0.9%), and death in 3 patients (0.7%). CONCLUSIONS: Surgery is the treatment of choice for vestibular schwannoma in the majority of patients. In our experience, the complication rate is very low and tumor size is the main factor influencing postoperative facial nerve function.

Paragangliomas de cabeza y cuello: experiencia en 126 pacientes con 162 tumores / Head and neck paragangliomas: Experience in 126 patients with 162 tumours

Introducción: Los paragangliomas de cabeza y cuello son tumores infrecuentes de naturaleza neuroendocrina. El objetivo de nuestro estudio es mostrar nuestra experiencia en el manejo de estos tumores. Métodos: Estudio retrospectivo de paragangliomas cervicocefálicos diagnosticados entre 1978-2014. En total fueron 126 pacientes con 162 tumores. Entre los tumores analizados se incluyeron 88 paragangliomas yugulotimpánicos (54,3%), 53 carotídeos (32,7%) y 21 vagales (12,9%). Resultados: La edad media al diagnóstico fue de 53,1 años; 87 pacientes eran mujeres (69,1%), y 39 varones (30,9%). La multicentricidad estuvo presente en 24 pacientes (19,1%). Se encontraron mutaciones genéticas germinales en el 50% de los pacientes analizados, siendo SDHD y SDHB las más frecuentes. Se intervinieron quirúrgicamente 72 paragangliomas aislados: 9 carotídeos, 21 timpánicos, 37 yugulares y 5 vagales; 25 tumores aislados fueron observados periódicamente: 7 carotídeos, 3 timpánicos, 9 yugulares y 6 vagales; 5 tumores yugulares fueron radiados. Los paragangliomas multicéntricos fueron tratados individualmente, con un total de 26 procedimientos quirúrgicos y 36 tumores resecados, 9 tumores sometidos a radioterapia y 12 controlados periódicamente. La complicación derivada de la resección de paragangliomas carotídeos aislados fue baja (15%) comparativamente con los paragangliomas yugulares (45,5%; p = 0,04). La complicación nerviosa fue mayor en tumores con extensión intradural (100%) con respecto a tumores extradurales (37,5%; p = 0,007). Conclusiones: El manejo de paragangliomas de cabeza y cuello incluye diferentes estrategias, entre las que están la cirugía, la radioterapia y la observación periódica. La combinación de todas ellas es especialmente importante en los pacientes con paragangliomas multicéntricos (AU)

Introduction: Head and neck paragangliomas are rare tumours of a neuroendocrine nature. The aim of this study was to show our experience in the management of these kind of tumours. Methods: This was a retrospective study of head and neck paragangliomas diagnosed between 1978 and 2014. A total of 126 patients with 162 tumours were analysed. The paragangliomas included 88 jugulotympanic tumours, 53 carotid tumours and 21 vagal paragangliomas. Results: Mean age at diagnosis was 53.1 years; 87 patients were female (69.1%) and 39, male (30.9%). Multifocality was present in 24 patients (19.1%). Germline mutations were found in 20 patients analysed; SDHD and SDHB were the most frequent. Surgery was performed on 72 isolated paragangliomas: these were 9 carotid, 21 tympanic, 37 jugular and 5 vagal paragangliomas. There were 25 isolated tumours that were observed periodically: 7 carotid, 3 tympanic, 9 jugular and 6 vagal paragangliomas; 5 jugular tumours were irradiated. Multifocal paragangliomas were individually treated, with a total of 26 surgical procedures and 36 tumours resected, 9 irradiated and 12 tumours periodically observed. Postoperative cranial nerve deficits in isolated carotid paragangliomas were lower (15%) compared with jugular tumours (45.5%,P=.04). Nerve deficit was found more frequently in tumours with intradural extension (100%) than in extradural tumours (37.5%, P=.007). Conclusions: Management of head and neck paragangliomas include surgery, radiotherapy and wait and scan policies. A combination of all of them is usually needed in patients with multifocal paragangliomas (AU)

Head and neck paragangliomas: Experience in 126 patients with 162 tumours.

INTRODUCTION: Head and neck paragangliomas are rare tumours of a neuroendocrine nature. The aim of this study was to show our experience in the management of these kind of tumours. METHODS: This was a retrospective study of head and neck paragangliomas diagnosed between 1978 and 2014. A total of 126 patients with 162 tumours were analysed. The paragangliomas included 88 jugulotympanic tumours, 53 carotid tumours and 21 vagal paragangliomas. RESULTS: Mean age at diagnosis was 53.1 years; 87 patients were female (69.1%) and 39, male (30.9%). Multifocality was present in 24 patients (19.1%). Germline mutations were found in 20 patients analysed; SDHD and SDHB were the most frequent. Surgery was performed on 72 isolated paragangliomas: these were 9 carotid, 21 tympanic, 37 jugular and 5 vagal paragangliomas. There were 25 isolated tumours that were observed periodically: 7 carotid, 3 tympanic, 9 jugular and 6 vagal paragangliomas; 5 jugular tumours were irradiated. Multifocal paragangliomas were individually treated, with a total of 26 surgical procedures and 36 tumours resected, 9 irradiated and 12 tumours periodically observed. Postoperative cranial nerve deficits in isolated carotid paragangliomas were lower (15%) compared with jugular tumours (45.5%, P=.04). Nerve deficit was found more frequently in tumours with intradural extension (100%) than in extradural tumours (37.5%, P=.007). CONCLUSIONS: Management of head and neck paragangliomas include surgery, radiotherapy and wait and scan policies. A combination of all of them is usually needed in patients with multifocal paragangliomas.

Management of vagal paragangliomas: review of 17 patients.

Vagal paragangliomas are very rare benign vascular tumors of neuroendocrine nature, and are much less frequent than carotid and jugulo-tympanic tumors. The goal of this retrospective study is to review the clinical and genetic findings, surgical treatment, and complications of vagal paragangliomas, as well as to discuss the management options. During the period 1990-2013, 17 patients with vagal paragangliomas were referred to our institution. There were ten patients with isolated tumors, and seven with multicentric paragangliomas. There were nine women and eight men. Mean age of patients was 51.4 years. Five cases had a positive family history of paraganglioma (29.4 %). Germline mutations of SDH genes were found in six of our patients (35.3 %). Many options were considered in the management of vagal paragangliomas. Surgical treatment was performed in 11 young patients (64.7 %) using different approaches: in 4 patients the tumor was resected through a transcervical approach; in 3 through a transcervical-transmandibular approach; in 1 it was resected using a transcervical-transmastoid approach, and in 3 a type A infratemporal fossa approach was performed. In all operated cases, the removal of the tumor led to sacrificing of the vagus nerve. Postoperative hypoglossal nerve deficit was reported in 4 cases (36.3 %). In six elderly patients (35.3 %), we decided to "wait-and-scan" in order to avoid creating greater morbidity than that of the tumor itself. Many factors should be considered in the treatment of vagal paragangliomas: the age and general condition of the patient, the biological behavior of the tumor, tumor size, genetic results, bilaterality, multicentricity, lower cranial nerve function, and of course the potential morbidity of the surgical treatment itself. Rehabilitation and, possibly surgery, are necessary to treat postoperative lower cranial nerve deficits.

Manejo conservador del schwannoma vestibular / Conservative management of vestibular schwannoma

Introducción: El schwannoma vestibular (SV) es un tumor benigno de lento crecimiento originado en el VIII par craneal, en cuyo tratamiento entran a formar parte la microcirugía, la radioterapia estereoatáxica, y el manejo conservador de los tumores con controles radiológicos periódicos. Material y métodos: Estudio retrospectivo de pacientes con SV siguiendo un manejo conservador en un hospital de tercer nivel entre los años 1993-2013.Un total de 73 pacientes fueron incorporados a nuestro protocolo de seguimiento de SV. La edad media al diagnóstico fue de 59,7 años. El tamaño medio de 11,9 mm (4-27 mm), siendo el 58,9% intracanaliculares y el 41,1% extracanaliculares. El periodo de seguimiento medio fue de 35,75 meses. Resultados: En el 87,7% no hubo evidencia de crecimiento tumoral. Un total de 9 (12,3%) tumores incrementaron sus dimensiones. La velocidad media de crecimiento fue de 0,62 mm/año. El porcentaje de tumores extracanal que crecieron (20%) fue mayor que el de los tumores intracanal (7%). Siete pacientes experimentaron cambios significativos en su sintomatología (9,5%) y 6 de estos una pérdida de la audición útil (8,2%). Seis pacientes salieron del seguimiento y fueron intervenidos quirúrgicamente (8,2%). Conclusión: El seguimiento del SV con controles periódicos de resonancia magnética nuclear representa una opción válida de manejo, dado que la mayoría de los tumores de pequeño tamaño experimentan poco o nulo crecimiento a lo largo del tiempo (AU)

Introduction: Vestibular schwannoma (VS) is a benign, slow-growing tumour originating in the 8 th cranial nerve. The treatment includes microsurgery, stereotactic radiotherapy and conservative management of tumours with periodic radiological tests. Methods: This was a retrospective study of patients with VS following conservative management in a tertiary hospital between 1993 and 2013. A total of 73 patients were enrolled in our protocol. The mean age at diagnosis was 59.7 years. The average size was 11.9 mm (4-27 mm); 58.9% of the tumours were intracanalicular and 41.1%, extracanalicular. The mean follow-up period was 35.75 months. Results: In 87.7% of patients there was no evidence of tumour growth. A total of 9 tumours (12.3%) increased in size. The average growth rate was 0.62 mm/year. The percentage of extracanalicular tumours that grew (20%) was higher than that of intracanalicular tumours (7%). Seven patients (9.5%) experienced significant changes in their symptoms and 6 of these (8.2%) experienced a loss of useful hearing. Six patients (8.2%) left follow-up and underwent surgery. Conclusions: Periodic monitoring of vestibular schwannomas with magnetic resonance imaging represents an option for management, because most small tumours experience little or no growth over time (AU)

Conservative management of vestibular schwannoma.

INTRODUCTION: Vestibular schwannoma (VS) is a benign, slow-growing tumour originating in the 8th cranial nerve. The treatment includes microsurgery, stereotactic radiotherapy and conservative management of tumours with periodic radiological tests. METHODS: This was a retrospective study of patients with VS following conservative management in a tertiary hospital between 1993 and 2013. A total of 73 patients were enrolled in our protocol. The mean age at diagnosis was 59.7 years. The average size was 11.9mm (4-27mm); 58.9% of the tumours were intracanalicular and 41.1%, extracanalicular. The mean follow-up period was 35.75 months. RESULTS: In 87.7% of patients there was no evidence of tumour growth. A total of 9 tumours (12.3%) increased in size. The average growth rate was 0.62mm/year. The percentage of extracanalicular tumours that grew (20%) was higher than that of intracanalicular tumours (7%). Seven patients (9.5%) experienced significant changes in their symptoms and 6 of these (8.2%) experienced a loss of useful hearing. Six patients (8.2%) left follow-up and underwent surgery. CONCLUSIONS: Periodic monitoring of vestibular schwannomas with magnetic resonance imaging represents an option for management, because most small tumours experience little or no growth over time.