Navigating the Complexities of Skull Base Paragangliomas: Case Report and Overview with Emphasis on Multi-Disciplinary Approach to Diagnosis and Management

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Authors: Jayesh Gupta, Prashant Vempati, Sepideh Amin‐Hanjani, Adel Azghadi, Grayson G. Cole, Claire M. E. Gleadhill, Madelyn N. Stevens, Ann K. Jay
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Abstract

Background: Paragangliomas are rare neuroendocrine neoplasms derived from neural crest cells. 1 Skull base paragangliomas, or glomus tumors, are less common than other skull base masses such as meningiomas, however, they are the most prevalent tumor of the jugular foramen. 2 Their location adjacent to critical neurovascular structures, high vascularity, and locally aggressive behavior requires collaboration between an experienced multidisciplinary team. 3 Purpose: We present the imaging findings of a rare case of glomus jugulotympanicum with extensive vascular invasion into the internal jugular vein, which has to date, only been described in a few case reports. 4 The purpose of this exhibit is to highlight the diagnostic and management considerations of glomus tumors from a multidisciplinary team. Methods: With Institutional Review Board approval, a retrospective search of our institution's database was performed for skull base paragangliomas from 2021-2025. Imaging studies, including CT, MRI and DOTATATE-PET, as well as radiation planning datasets and pathologic specimens, were reviewed. Clinical records, operative notes, and intraoperative images were also examined. Results: CT, MR, and DOTATATE-PET findings demonstrated a rare case of glomus jugulotympanicum with extensive invasion into the internal jugular vein. Representative imaging, along with pathologic specimens, is used to illustrate typical appearances of glomus tumors and to distinguish them from common mimics. Clinical considerations from otolaryngology, neurosurgery, and radiation oncology perspectives are discussed. Conclusion: Skull base paragangliomas pose diagnostic and therapeutic challenges due to their vascularity, locally aggressive nature and involvement of critical neurovascular structures. Optimal outcomes require a multidisciplinary approach that integrates neuroradiology, pathology, otolaryngology, neurosurgery and radiation oncology expertise to ensure accurate diagnosis and effective management. Figs. 1 and 2 Contrast enhanced sagittal CT image and corresponding coronal GA-68 DOTATATE PET-CT a glomus jugulotypanicum extending into and expanding the internal jugular vein. Fig. 3 Coronal CT in bone window shows a destructive mass in the left skull base extending into the middle ear cavity. Even though glomus paragangliomas are benign lesions, they cause aggressive bony erosion due to their vascular nature. Publication History Article published online: 27 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

Fields of Study & Subjects

Adrenal and Paraganglionic Tumors Meningioma and schwannoma management Head and Neck Surgical Oncology

Publication details

Journal / source
Journal of Neurological Surgery Part B Skull Base
Volume
87
Issue
S 01
Pages
S1–S548
Publication date
27 February 2026
Language
EN