Objective: Isolated amyloidomas rarely manifest in nervous system tissues. To the authors' knowledge, there have been 52 documented cases of primary amyloid tumors of the central nervous system and closely associated structures. The authors present a case of a woman with a history of presumptive trigeminal neuralgia who was found to have an amyloidoma of the trigeminal ganglion.
Clinical presentation: A 32-year-old Caucasian patient presented with a chief complaint of severe numbness and pain throughout the right side of her face. Her symptoms had been progressive over the previous 3 years. Medical management of her presumptive diseases with Zoloft (Pfizer Inc., New York, NY) and Neurontin (Pfizer Inc.) failed to improve or halt her right facial numbness and pain. Brain magnetic resonance imaging was acquired, demonstrating abnormal contrast enhancement and enlargement of the right trigeminal ganglion. The lesion abutted and indented the right internal carotid artery and extended from Meckel's cave into the inferior cavernous sinus and distally to the foramen ovale.
Intervention: The patient underwent a right frontotemporal craniotomy for resection of the gasserian ganglion lesion. A delicate incision was made in the wall of the cavernous sinus, allowing confirmatory biopsy of the lesion. With the site of the tumor within the cavernous sinus verified by pathology, the remainder of the tumor was removed. A final pathological review of the resected tumor confirmed a diagnosis of amyloidoma of the trigeminal ganglion.
Conclusion: We present the case of a patient with a rare trigeminal ganglion amyloidoma that closely mimicked idiopathic trigeminal neuralgia. Even in the absence of systemic signs of amyloidosis, this benign protein deposition disease should be considered in the differential for atypical dysesthesias of the trigeminal dermatomes. Furthermore, central and peripheral nervous system amyloidomas respond well to surgical resection and rarely recur.