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 multimodal en los schwanomas vestibulares grandes / Multimodal Treatment for Large Vestibular Schwannomas

Introducción: Los schwanomas vestibulares son lesiones clasificadas como grado I por la Organización Mundial de la Salud. Las opciones conductuales incluyen la observación, la radiocirugía o la resección microquirúrgica endoscópica. Objetivo: Describir el rol del tratamiento multimodal en los schwanomas vestibulares. Métodos: Se realizó una búsqueda sistemática de literatura médica para la identificación e inclusión de artículos en las siguientes bases de datos: PubMed/Medline, SciELO y Google Scholar, además de los servidores de preprints BioRvix y MedRvix. Se usaron los siguientes descriptores: vestibular schwanoma OR acoustic neuroma AND nerve centered approach OR combined approach AND partial resection OR subtotal resection OR near total resection. Se excluyeron editoriales, cartas al editor, libros, revisiones, metaanálisis y aquellos artículos con método deficiente de más de 20 años de publicación o sin mención al tema de interés. Fueron incluidos 16 artículos. Desarrollo: Los artículos incluyeron un total de 699 pacientes, de los cuales solo 228 (32,6 porciento) recibieron radioterapia en el período posoperatorio. El período de seguimiento promedió 49,6 meses. La preservación de la función facial se logró en el 88,3 porciento de los casos y el control tumoral en el 80,7 porciento. El estado de la preservación auditiva solo fue informado en el 50 porciento de los estudios. El uso de la radioterapia adyuvante mostró gran variabilidad. Conclusiones: El tratamiento multimodal para los schwanomas vestibulares grandes -compuesto por la resección subtotal, vaciamiento intratumoral, seguido de radiocirugía- se ha convertido en una opción plausible. Se necesita la publicación de mayor cantidad de reportes para ofrecer recomendaciones y estratificar la conducta(AU)

Introduction: Vestibular schwannomas are lesions classified as grade I by the World Health Organization. Behavioral options include observation, radiosurgery or endoscopic microsurgical resection. Objective: To describe the role of multimodal treatment for vestibular schwannomas. Methods: A systematic search of medical literature, for the identification and inclusion of articles, was carried out in the databases PubMed/Medline, SciELO and Google Scholar, as well as in the preprint servers BioRvix and MedRvix. The following descriptors were used: vestibular schwannoma OR acoustic neuroma AND nerve centered approach OR combined approach AND partial resection OR subtotal resection OR near total resection. Editorials, letters to the editor, books, reviews and metaanalyses were excluded, as well as articles with deficient method of more than 20 years of publication or not mentioning the topic of interest. Sixteen articles were included. Development: The articles included a total of 699 patients, of which only 228 (32.6 ) received radiotherapy in the postoperative period. The average follow-up period was 49.6 months. Preservation of facial function was achieved in 88.3 percent of cases; and tumor control, in 80.7 percent. Hearing preservation status was reported in only 50 percent of the studies. The usage of adjuvant radiotherapy showed great variability. Conclusions: Multimodal treatment for large vestibular schwannomas -composed of subtotal resection and intratumoral draining, followed by radiosurgery- has become a plausible option. The publication of a greater amount of reports is necessary to provide recommendations and stratify the behavior(AU)

Hearing loss in vestibular schwannoma

Introducción y objetivo: La hipoacusia es uno de los síntomas más frecuentes en el debut de un schwannoma vestibular (SV). El objetivo de este estudio es analizar la pérdida auditiva en pacientes con SV y determinar en qué medida se relaciona con el tamaño tumoral. Método: Se lleva a cabo un estudio retrospectivo observacional en 291 pacientes diagnosticados de SV entre 1995 y 2017 en el servicio de Otorrinolaringología y Cirugía Cervicofacial del Hospital Clínico Universitario de Salamanca analizándose diferentes datos pre y postoperatorios de los pacientes así como hallazgos radiológicos y quirúrgicos.Resultados: El síntoma más frecuente de consulta es la hipoacusia unilateral progresiva (31.6%). La asociación entre el tamaño del SV y la hipoacusia no es estadísticamente significativa (p=0.099).Conclusiones: La pérdida auditiva producida por un SV no es patrimonio de ningún estadio, aunque las pérdidas severas y profundas son más indicativas de neurinomas grados III-IV, y ante una pérdida auditiva leve o audición normal tendremos con más probabilidad un neurinoma grado I-II. (AU)

The most common reason for consultation in patients suffering from Vestibular Schwannoma (VS) is progressive hearing loss. The main objective of this study is analyzing the hearing loss in patients with VS and determining the extent to which the tumor grade and the hearing loss are related. Methods: An observational retrospective study was conducted with a sample of 291 patients diag-nosed with VS between 1995 and 2017 in the Department of Otorhinolaryngology and Head and Neck Surgery of the Tertiary Care Primary Center of Salamanca. We analyzed preoperative and postoperative data from patients with VS as well as radiological and surgical findings. Results: The most common reason for consultation at diagnosis was progressive unilateral hearing loss (31.6%). The relationship between the size of the VS and hearing loss in the patients who reported progressive unilateral hearing loss as a reason for consultation was not statistically significant (p=0.099). Conclusion: The most common reason for consultation in VS is progressive unilateral hearing loss. The hearing loss caused by VS does not have a statistically significant association with any tumor grade, although severe and profound hearing loss is more commonly associated with grade III-IV neurinomas, whereas mild hearing loss or normal hearing are more likely in grade I-II tumors. (AU)

Resección microquirúrgica de schwannoma vestibular grado IVa con preservación de la función facial / Microsurgical resection of vestibular schwannoma grade IVa with preservation of facial function

Introducción: La cirugía de los schwannoma vestibulares constituye un desafío para los neurocirujanos. Debido a que se trata de un tumor benigno la resección completa de la lesión implica la curación del paciente. Sin embargo, este objetivo no siempre es fácil de lograr preservando la función de los nervios facial y acústico, especialmente en tumores de gran tamaño. Objetivos: Presentar detalles técnicos de la cirugía de resección de un schwannoma vestibular de gran tamaño (IVa) en el que se pudo preservar la función facial. Materiales y métodos: Se presenta el caso de una paciente femenina de 36 años que consultó por hipoacusia izquierda. En la RM preoperatoria se evidenciaba una lesión ocupante de espacio del ángulo pontocerebeloso izquierdo compatible con schwannoma vestibular con compresión del tronco encefálico y sin efecto de masa sobre el IV ventrículo (grado IVa). Mediante un abordaje suboccipital retromastoideo en posición de decúbito lateral se realizó la resección de la lesión en forma completa asistida por monitoreo del nervio facial. En todo momento se pudo preservar el plano aracnoideo que separaba el tumor de los nervios adyacentes. Resultados: Se logró una resección macroscópicamente completa con preservación de la función del nervio facial. La paciente permaneció internada por 96 hs en el postoperatorio sin complicaciones derivadas del procedimiento. Conclusión: La preservación del plano aracnoideo es un detalle técnico de mucha importancia para disminuir las posibilidades de lesión de los nervios facial y auditivo en la cirugía de resección de los schwannoma vestibulares.

Introduction: The surgery of vestibular schwannomas is a challenge for neurosurgeons.Because it is a benign tumor, complete resection of the lesion involves healing the patient. However, this objective is not always easy to achieve, preserving the function of both the facial and acoustic nerves, especially when dealing with large tumors. Objective: The objective of the video is to present some technical details of a large vestibular schwannoma (IVa) surgery in which the facial function could be preserved. Materials and methods: We present the case of a 36-year-old female patient who consulted for left hearing loss. The preoperative MRI showed a space- occupying lesion of the left pontocerebellar angle, which was compatible with vestibular schwannoma, with compression of the brainstem but with no mass effect on the IV ventricle (grade IVa). By means of a retromastoid suboccipital approach in the lateral prone position, the lesion was completely resected assisted by neurophysiological monitoring of the facial nerve. At all times, the arachnoid plane separating the tumor from the adjacent nerves was preserved. Results: A macroscopically complete resection was achieved preserving the facial nerve function. The patient stayed hospitalized for 96 hours during the postoperative period without any complication from the procedure. Conclusion: Preserving the arachnoid plane is a very important technical detail to reduce the possibilities of injury of the facial and auditory nerves in the vestibular schwannoma resection surgery.

Change in tinnitus after acoustic neuroma removal using a translabyrinthine approach. A Prospective study. / Estudio prospectivo de la evolución del acúfeno tras exéresis por vía translaberíntica del neurinoma del acústico.

INTRODUCTION: Tinnitus is one of the primary symptoms of vestibular schwannoma (VS) and the effect of surgery is unpredictable. MATERIALS AND METHODS: We conducted a prospective study of the patients who underwent a translabyrinthine approach for the treatment of their VS (2009-2013) at our Hospital. Patients answered the Tinnitus Handicap Inventory (THI) questionnaire pre- and postoperatively. The clinical charts provided data such as age, gender, tumour size, preoperative audiometry and postoperative facial function. RESULTS: The study included 39 patients. Of these, 71.8% suffered from tinnitus: 50% grade I, 17.9% grade II, 10.7% grade III, 21.4% grade IV and 0% grade V. We found no statistical association between tinnitus and the different variables measured preoperatively. Postoperatively, 48.7% of the patients suffered from tinnitus: 31.6% grade I, 36.8% grade II, 10.5% grade III, 15.8% grade IV and 5.3% grade V. The difference between mean pre- and postoperative THI was statistically significant (P=.011); this difference was greater in younger patients. We have found a significant negative correlation (r=-0.335; P=.037) between preoperative audiometry and postoperative THI. CONCLUSIONS: We did not find any significant association between tinnitus and age, gender, tumour size and postoperative facial function. Translabyrinthine surgical removal of VS in these patients led to better THI results, with the younger patients having better outcomes. The patients with poorest preoperative audition were the ones that had the best results in the postoperative THI questionnaire.

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.

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.

Radiosurgery as treatment for acoustic neuroma. Ten years' experience.

INTRODUCTION AND OBJECTIVE: The acoustic neuroma is a benign tumour that usually affects the vestibular portion of the vestibulocochlear nerve. It represents 8% of all intracranial tumours and 80% of those arising at the cerebellopontine angle. There are 3 treatment options: microsurgery (the technique of choice), radiosurgery and observation. The objective of the study was to evaluate the results and side effects obtained using radiosurgery as treatment for acoustic neuroma. MATERIAL AND METHODS: We performed a review of all patients treated with radiosurgery (Gamma Knife and linear accelerator) at doses of 1200-1300 cGy for unilateral acoustic neuroma in our hospital from January 1999 until January 2010. In all patients we evaluated the overall state, tumour growth control rate (tumour smaller or remaining the same size), the involvement of v and vii cranial nerves and central nervous system disorders. We also assessed follow-up time and changes in hearing thresholds after radiosurgery. RESULTS: From a total of 35 patients studied, with a mean age of 58.29 years and lacking statistically significant differences in gender, the tumour growth control rate was over 90%. The main reason for visit (65.71%) was unilateral and progressive hearing loss. After treatment, 34.28% of patients had hearing loss. The involvement of the cranial nerves (v-vii) was transitory in 100% of cases. Gamma Knife radiosurgery was administered in 82.85% of patients. CONCLUSION: Although microsurgery is the treatment of choice for acoustic neuroma, we consider radiosurgery as a valid alternative in selected patients (elderly, comorbidity, small tumour size and sensorineural hearing loss, among others).

[Clinical and radiological evolution of a group of untreated acoustic neuromas]. / Evolución clínica y radiológica de un grupo de neurinomas del acústico no tratados.

INTRODUCTION: The acoustic neuroma is a benign tumour that originates in the vestibular branch of the eighth cranial nerve. The main treatment is surgery, but many authors suggest that with elderly patients or in small neuromas we can opt for watchful waiting. METHODS: This was a retrospective study from 2007 to 2013 that included 27 patients diagnosed of acoustic neuroma that had not been treated due to the size of the tumour, age and comorbidities, or by patient choice. We evaluated overall condition, hearing thresholds, degree of canal paresis and central disorders. RESULTS: After 6 years of follow up, clinical manifestations of 18 patients remained unchanged, 5 patients underwent hearing loss and developed tinnitus, 2 cases had more intense tinnitus and 2 cases had dizziness. The radiological controls by magnetic resonance imaging showed that the initial maximum diameters (5-16mm) increased by 1.7mm on average, with annual growth rates below 0.5mm. CONCLUSION: In selected cases, such as for small neuromas and in elderly patients, the conservative option of close monitoring with magnetic resonance imaging is an important alternative given that, in our cases, clinical features and radiological image did not suffer major changes. If there were any such changes, therapeutic options could be proposed.