Cerebellopontine angle epidermoids presenting with cranial nerve hyperactive dysfunction: pathogenesis and long-term surgical results in 30 patients.

OBJECTIVE: To provide the characteristics and long-term surgical results of patients who present with cerebellopontine angle epidermoids and trigeminal neuralgia (TN) or hemifacial spasm.

METHODS: A total of 30 patients (23 women, 7 men) who presented with cerebellopontine angle epidermoids and TN (28 patients) or hemifacial spasm (2 patients) between 1982 and 1995 were reviewed, with emphasis being placed on the clinical manifestations, the mechanisms of symptom development, the long-term follow-up results, and the anatomic relationship between the tumor and the surrounding neurovascular structures.

RESULTS: The average age of the patients was 37.8 years at symptom onset and 49.3 years at the time of the operation. The tumor-nerve relationships were classified into four types: complete encasement of the nerve by the tumor, compression and distortion of the nerve by the tumor, compression of the nerve by an artery located on the opposite side of the unilateral tumor, and compression of the nerve by an artery on the same side of the tumor. Total resection was achieved in 17 patients (56.7%). Microvascular decompression of the respective cranial nerve was achieved in nine cases of direct arterial compression in addition to tumor removal. The symptom was relieved completely in all cases. In an average follow-up period of 11.5 years, three patients developed recurrent symptoms: two experienced tumor regrowth, and one had arachnoid adhesion.

CONCLUSION: Hyperactive dysfunction of the cranial nerves, especially TN, may be the initial and only symptom that patients with cerebellopontine angle epidermoids experience. The occurrence of TN at a younger age was characteristic of TN patients with epidermoids, in contrast to patients with TN due to a vascular cause. The symptom is elicited by compression of the nerve by the tumor per se, by an artery that is displaced to the nerve, or by both. Careful resection of the tumor, whose capsule occasionally is strongly adherent to the neurovascular structures, is necessary, and microvascular decompression to straighten the neuraxis should be performed in some cases to achieve a complete, permanent cure of symptoms with a low rate of recurrence.