Trigeminal Ganglioneuroma: A Rare Case of Trigeminal Neuralgia Caused by Cerebellopontine Angle Tumor

Abstract

Background: Intracranial ganglioneuromas are very rare benign tumors of neural crest origin and generally arise from the peripheral nervous system or adrenal glands. Very few cases of intracranial ganglioneuroma arising from the trigeminal nerve have been reported in the literature, all in East Asia.

Case description: A 52-year-old male presented to his primary care physician for evaluation of right facial and periorbital pain for over 18 months. He also reported a two-week history of diplopia with right lateral gaze, which resolved spontaneously. An MRI brain with and without contrast revealed a 3.0 × 2.6 × 2.4 cm enhancing extra-axial mass involving right Meckel's cave with a posterior 1.0 × 1.4 cm lobular component protruding into the prepontine cistern. The patient underwent a right middle fossa craniotomy for a subtemporal surgical resection of the tumor. Histologic section of the tumor demonstrated mature ganglion cells with surrounding satellite cells in a schwannian rich stroma, consistent with mature type ganglioneuroma.

Conclusions: To the best of our knowledge, this is the sixth case of trigeminal ganglioneuroma; however, it is the first case reported in the United States.

Keywords: Ganglioneuroma; Neuralgia; Trigeminal.