Cerebellopontine Angle Glioblastoma: Unlikely Presentation and Limited Treatment Options

Introduction: Glioblastoma represents 52% of primary brain malignancies and most commonly occurs in the cerebral white matter. Despite advances in multimodal oncologic therapies, mean overall survival (OS) is 12 to 18 months after maximal treatment. We present a rare case of glioblastoma arising within the cerebellopontine angle (CPA) and review the literature.

Objectives: This study was aimed to describe rare case presentation and incidence of CPA glioblastoma, discuss the rapidly progressive presentation of CPA syndrome associated with CPA glioblastoma, and delineate the current therapeutic limitations of glioblastoma within eloquent locations

Methods: Patient's electronic medical records were reviewed for clinical course and radiographic findings. Literature review using MEDLINE search of the English literature through April 2020 following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines was performed to identify all documented cases of glioblastoma located in the cerebellopontine angle.

Results:

Case report: a 58-year-old male presented with rapidly progressive headaches, disequilibrium, and gait disturbance. MRI revealed a heterogeneously enhancing right CPA mass with predominant extension into Meckel's cave ([Figs. 1]–[3]). Patient underwent craniotomy for planned gross total resection; however, unexpected adherence to eloquent structures and inconclusive frozen pathology necessitated obtaining biopsy only. Pathology confirmed IDH1 mutant glioblastoma. Repeat craniotomy achieved over 80% resection of the enhancing portion of the mass and was followed by initiation of the Stupp protocol. Postoperatively, patient developed cranial nerve V to VIII palsies which resolved with time. He remains alive with evidence of tumor progression at 4 months.

Literature Review: Search yielded 21 cases of CPA glioblastoma, including six pediatrics case and one spinal cord metastasis ([Fig. 4]). Approximately, half of the cases demonstrated OS of less than 6 months due to delayed diagnosis and treatment; further, only three patients had a comparable OS of 12 months after maximal treatment. All cases were treated with surgery and chemoradiation with no mention of laser interstitial thermal therapy, Optune, or alternative modalities.

Conclusion: Glioblastoma in the cerebellopontine angle is a rare, challenging entity, and should remain a differential diagnosis in presentations with rapidly progressing CPA syndrome. Accurate, early diagnosis is key in achieving a comparable overall survival to glioblastoma in common locations. Due to the depth of location and multiple adjacent eloquent structures, therapies remain highly limited beyond the current standard of care.

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No conflict of interest has been declared by the author(s).

Publication History

Article published online:
12 February 2021