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      Pediatric multicompartmental trigeminal schwannoma: illustrative case

      case-report

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          Abstract

          BACKGROUND

          Trigeminal schwannoma (TS) is an uncommon and histologically benign intracranial lesion that can involve any segment of the fifth cranial nerve. Given its often impressive size at diagnosis and frequent involvement of critical neurovascular structures of the skull base, it represents a challenging entity to treat. Pediatric TS is particularly rare and presents unique challenges. Similarly, tumors with extension into multiple compartments (e.g., middle cranial fossa, posterior cranial fossa, extracranial spaces) are notoriously difficult to treat surgically. Combined or staged surgical approaches are typically required to address them, with radiosurgical treatment as an adjunct.

          OBSERVATIONS

          The authors presented the unusual case of a 9-year-old boy with a large, recurrent multicompartmental TS involving Meckel’s cave, the cerebellopontine angle, and the infratemporal fossa. Near-total resection was achieved using a frontotemporal-orbitozygomatic craniotomy with a combined interdural and extradural approach.

          LESSONS

          The case report adds to the current literature on multicompartmental TSs in children and their management. The authors also provided a simplified classification of TS that can be generalized to other skull base tumors. Given a lack of precedent, the authors intended to add to the discussion regarding surgical management of these rare and challenging skull base lesions.

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          Most cited references32

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          Trigeminal neurinomas extending into multiple fossae: surgical methods and review of the literature.

          Since 1974, 27 patients with trigeminal neurinomas (TNs) have been treated at Keio University Hospital and ancillary institutes. In the present study the clinical features and developmental patterns of these 27 cases are analyzed, and the clinical features of 402 cases reported in the literature are reviewed. Based on the analysis of the developmental patterns of the TNs, the surgical strategy for a one-stage removal of TNs involving multiple fossae is described. Trigeminal neurinomas are classified into six types according to tumor location. Types M, P, and E are tumors involving a single compartment, that is, the middle fossa, posterior fossa, or extracranial space, respectively. Types MP (middle and posterior fossae), ME (middle fossa and extracranial space), or MPE (middle and posterior fossae and extracranial space) are tumors involving multiple compartments. Advances in neuroimaging technologies, such as magnetic resonance imaging, have revealed a high incidence of TNs extending into multiple fossae, namely 36.2% in cases reported since 1983 and 59% in the authors' series. All but one of the most recent 19 patients in this series underwent skull base surgery, whereas the remaining nine patients were surgically treated via the conventional subdural approach. The rate of total tumor removal and the clinical outcome were significantly better in those patients treated by skull base surgery than those treated by conventional surgery. The TNs extending into multiple fossae can be totally removed using the following single-stage surgical techniques: Type MP by the anterior transpetrosal approach; Type ME by the zygomatic or orbitozygomatic infratemporal approach; and Type MPE by the zygomatic transpetrosal approach. In 12 of 13 cases involving multiple fossae in this series, total tumor removal was achieved using single-stage skull base surgery.
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            Trigeminal schwannoma. Surgical series of 14 cases with review of the literature.

            A consecutive series of 14 patients with trigeminal schwannoma managed surgically at the Neurological Institute of New York since 1970 is reported. Nine women and five men (mean age 40 years) were diagnosed following a mean symptom duration of 33 months. Abnormalities of trigeminal nerve function were present in 11 patients on admission examination. Facial pain was a prominent feature in eight patients. Two patients, both with schwannomas arising from the trigeminal root, presented initially with typical trigeminal neuralgia. Additional cranial nerve palsies or cerebellar or pyramidal tract signs were noted in eight patients. The surgical approach to these tumors depends on their anatomical location. Four patients had tumors confined to the middle fossa, three patients had tumors limited to the posterior fossa, and seven patients had both supratentorial and infratentorial components of their tumors. Twenty operative procedures were performed on these patients, resulting in complete extirpation in six patients, nearly complete removal in seven patients, and partial removal in one patient. Adherence of the tumor to the lateral wall of the cavernous sinus or the brain stem precluded total removal. There was one postoperative death. In the immediate postoperative period, abnormalities of cranial nerves controlling the extraocular muscles were common. In general, these deficits were transient; however, some permanent loss of trigeminal nerve function occurred in nine patients. Two patients required tarsorrhaphy for neurotropic keratitis, and two patients underwent cerebrospinal fluid (CSF) shunting procedures for hydrocephalus or for a persistent CSF leak. The follow-up period ranged from 4 to 177 months (mean 47 months). The clinical features, anatomical considerations, and surgical approach to these rare tumors are discussed. A clinical review of 106 additional cases of trigeminal schwannoma, reported in the English literature since 1935, is also presented.
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              Surgical management of trigeminal schwannomas: defining the role for endoscopic endonasal approaches

              Because multiple anatomical compartments are involved, the surgical management of trigeminal schwannomas requires a spectrum of cranial base approaches. The endoscopic endonasal approach to Meckel's cave provides a minimal access corridor for surgery, but few reports have assessed outcomes of the procedure or provided guidelines for case selection. A prospectively acquired database of 680 endoscopic endonasal cases was queried for trigeminal schwannoma cases. Clinical charts, radiographic images, and long-term outcomes were reviewed to determine outcome and success in removing tumor from each compartment traversed by the trigeminal nerve. Four patients had undergone endoscopic resection of trigeminal schwannomas via the transpterygoid approach (mean follow-up 37 months). All patients had disease within Meckel's cave, and 1 patient had extension into the posterior fossa. Gross-total resection was achieved in 3 patients whose tumors were purely extracranial. One patient with combined Meckel's cave and posterior fossa tumor had complete resection of the extracranial disease and 52% resection of the posterior fossa disease. One patient with posterior fossa disease experienced a sixth cranial nerve palsy in addition to a corneal keratopathy from worsened trigeminal neuropathy. There were no CSF leaks. Over the course of the study, 1 patient with subtotal resection required subsequent stereotactic radiosurgery for disease progression within the posterior fossa. Endoscopic endonasal approaches appear to be well suited for trigeminal schwannomas restricted to Meckel's cave and/or extracranial segments of the nerve. Lateral transcranial skull base approaches should be considered for patients with posterior fossa disease. Further multiinstitutional studies will be necessary for adequate power to help determine relative indications between endoscopic and transcranial skull base approaches.

                Author and article information

                Journal
                J Neurosurg Case Lessons
                J Neurosurg Case Lessons
                J Neurosurg Case Lessons
                Journal of Neurosurgery: Case Lessons
                American Association of Neurological Surgeons
                2694-1902
                17 May 2021
                17 May 2021
                : 1
                : 20
                : CASE2171
                Affiliations
                [1 ]Divison of Neurosurgery, University of Toronto, Toronto, Ontario, Canada
                [2 ]Division of Neurosurgery, Hospital for Sick Children, Toronto, Ontario, Canada; and
                [3 ]Division of Neurosurgery, St. Michael’s Hospital, Toronto, Ontario, Canada
                Author notes
                Correspondence Alexander P. Landry: University of Toronto, Toronto, ON, Canada. alex.landry@ 123456mail.utoronto.ca .

                INCLUDE WHEN CITING Published online May 17, 2021; DOI: 10.3171/CASE2171.

                Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

                Article
                103171/CASE2171
                10.3171/CASE2171
                9245852
                e792a8b1-4a60-4ed2-bcc2-04f233dbd419
                © 2021 The authors

                CC BY-NC-ND 4.0 ( http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 5 February 2021
                : 28 February 2021
                Page count
                Figures: 4, Tables: 1, References: 32, Pages: 5
                Categories
                Oncology, Oncology
                Pediatric, Pediatric
                Skull-Base, Skull Base
                Case Lesson

                trigeminal schwannoma,pediatric,skull base,anatomical classification,surgical approaches,mpe = middle fossa/posterior fossa/extracranial,mri = magnetic resonance imaging,ts = trigeminal schwannoma

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