A Multicenter Experience of the Treatment of Type Di Tympanojugular Paragangliomas: Total vs. Partial Resection and Residue Management

Abstract

Introduction: Tympanojugular paragangliomas (TJ-PGs) showing intradural growth into the cerebellopontine angle (Fisch classification Di) represent a surgical challenge, with their proper surgical management still under debate. Methods: This is an international multicenter retrospective review of patients with Di TJ-PGs who underwent surgery in three high-volume skull base surgery centers. We aimed to establish practice patterns for treating Di TJ-PGs, namely the surgical approach, total versus partial resection, and whether a staged procedure was needed. We also examined the status of the facial and lower cranial nerves (LCNs), postoperative complications, and residue management after partial resection. Results: Thirty-two patients were included in this study with an average follow-up of 66 months. Preoperative angiography with selective embolization was performed in all patients, and a type A infratemporal fossa approach was the most common surgical technique. Total resection was achieved in 16 cases. A single-stage procedure was performed in 26 patients and a staged procedure in 6. CSF leakage in the neck was the main reported complication. Most patients had an HB I-II-grade facial nerve at the last follow-up, and three patients experienced worsened lower cranial neuropathies. In 16 patients residual disease was present after surgery and was managed with either radiotherapy or observation. Conclusions: Di TJ-PGs pose a complex treatment challenge for which clear-cut management recommendations have not been established. Surgical resection, when indicated, may be total, the preferred option in young healthy candidates, or partial, mainly employed in elderly or high-risk patients, always considering the tumor’s relationship to critical structures. When residual tumor is present, both radiological surveillance and adjuvant radiotherapy can be effective strategies.