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Background: The expanded transpromontorial transcanal approach (ExpTTA) represents a recent addition to the surgical approaches available for the treatment of vestibular schwannoma. An initial purely endoscopic version has been complemented by the use of the microscope and it is now one of the possible surgical options for small to medium-sized vestibular schwannomas with a predominantly intracanalar development., Methods: This is a series of 54 patients who underwent microsurgical resection of sporadic, unilateral vestibular schwannoma, mainly Koos I-II with non-serviceable hearing, between January 2016 and January 2023 using the expanded transcanal transpromontorial approach. We describe the surgical technique, focusing on anatomical landmarks, and analyzing its advantages and shortcomings. Retrospective analysis of clinical outcomes is presented, including early and late complications. The mean follow-up was 46.7 months., Results: We achieved gross total resection of the lesion in all cases, confirmed on the first follow-up MRI at least 6 months after each procedure. We did not record any intraoperative complication nor disease recurrence. We recorded two postoperative severe facial nerve palsies, one of which was permanent. No cases of disabling vertigo or imbalance were reported, and all patients reported full recovery of autonomy in daily activities. Three cases of otoliquorrhea were managed conservatively successfully., Conclusions: The transcanal transpromontorial approach combines the advantages of endoscopy with the possibilities provided by microsurgery. Our experience confirms its safety in terms of surgical complications and facial nerve outcome. This approach is amongst the treatment options for small-medium schwannomas in patients with impaired hearing, especially in young patients, ensuring radical resection, disease control, and minimal morbidity., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

According to the fifth edition of the World Health Organization (WHO) classification of tumors of the central nervous system (CNS), diffuse midline glioma H3 K27-altered is a grade 4 infiltrative glioma that arises from midline anatomical structures and is characterized by the loss of H3 K27me3 and co-occurring H3 K27M mutation or EZHIP overexpression. However, the H3 K27M mutation has also been observed in circumscribed gliomas and glioneuronal tumors arising in midline anatomical structures, which may result in diagnostic pitfalls.Rosette-forming glioneuronal tumor (RGNT) is a CNS WHO grade 1 neoplasm that histologically features neurocytic and glial components and originates in midline anatomical structures.This study aimed to assess whether RGNTs, similar to other midline tumors, may exhibit immunohistochemical loss of H3 K27me3 and harbor the H3 K27M mutation.All seven analyzed RGNTs displayed immunohistochemical loss of H3 K27me3 in all tumor cells or H3 K27me3 mosaic immunostaining. In one case, H3 K27me3 loss was associated with the H3 K27M mutation, whereas the other six cases did not exhibit any H3 mutations or EZHIP overexpression. During a follow-up period of 23 months, the H3 K27M-mutant case remained unchanged in size despite partial resection, indicating that the H3 mutation may not confer higher biological aggressiveness to RGNT.The immunohistochemical loss of H3 K27me3 co-occurring with the H3 K27M mutation may result in the potential misdiagnosis of RGNT, especially in cases of small biopsy specimens consisting of only the glial component., (© 2024. The Author(s).)

Main Goals: To analyze how and when the endoscope is used in vestibular schwannoma surgery and identify the benefits of using endoscopy in this type of surgery., Background: It is currently unclear if there is any benefit from using an endoscope in vestibular schwannoma surgery so this retrospective analysis set out to study this., Methods: All the patients who underwent vestibular schwannoma surgery at our clinic were included for all the vestibular schwannoma approaches taken. We studied when endoscopy was used during surgery and the goal of using endoscopy. Several pre- and postoperative factors were assessed such as complications, facial function, and hearing function in the case of techniques that allow hearing preservation., Results: From January 2015 to September 2018, 280 patients underwent lateral skull base surgery. Of these, 112 were included in this study. The endoscope was used in all 112 patients, and in eight cases it was possible to identify residual disease using the endoscope to check the surgical field, and then to remove the disease under endoscopic view. Moreover, in two other cases, the endoscope was used to resolve a vasculoneural conflict between the anterior inferior cerebellar artery (AICA) loop and facial nerve in one case, and for deafferentation of the superior and inferior vestibular nerves in the second case. No major intraoperative complications occurred in our series. There was no statistically significant difference in postoperative facial nerve function between patients in whom the endoscope was used as a diagnostic tool and patients in whom it was used as an operative tool (p = 0.3152)., Conclusions: The endoscope may be useful, especially in surgical techniques where there is poor control of the internal auditory canal (IAC). An endoscopic support technique is strongly recommended to avoid residual disease, particularly in retrosigmoid and retrolabyrinthine approaches. Moreover, the recent introduction of the transcanal transpromontorial approach allows the endoscope to be used during all the procedures in patients affected by a vestibular schwannoma limited to the IAC or to support surgical procedures during an enlarged microscopic approach.