Neurofibromatosis Type 2: Long-Term Treatment Outcome

ABSTRACT

OBJECTIVE: The objective is to clarify the long-term functional outcome of NF-2 and to elucidate optimal treatment strategy. METHODS: The authors retrospectively analyzed clinical records and radiological imaging of 32 patients of NF-2 treated at from 1979 to 2000. Age at diagnosis was 30(14-54). Male to female ratio was 1418. Mean follow-up(F/U) periods were 61(6-240) months. Four patients were lost during F/U periods. Fifty-one tumors of 29 patients were surgically treated including radiosurgery, and three patients rejected any treatment. Eleven tumors of 10 patients with non-schwannomas were managed by craniotomy, and one of them was managed by biopsy only. Among 21 tumors of 19 patients with schwannomas, 16 tumors of 14 patients were vestibular schwannomas(VS), one trigeminal schwannoma, and four spinal schwannomas. Fourteen tumors with 13 patients were managed by radiosurgery. RESULTS: Presenting symptoms were hearing problem(44%, 14/32 patients), motor or sensory change (25%, 8/32 patients), and visual symptoms (15%, 5/32 patients). Long-term functional outcome was poor (KPS; median 46.6). Six patients died during follow periods and the cause of death was aspiration pneumonia related to lower cranial palsy or high cervical cord lesion(except 1 case; suicide). In 17 patients, 7 patients of initial hearing had preserved after any treatment modalities, another 10 patients had deteriorated hearing function. In facial nerve function, 12 patients except one patient deteriorated after surgical resection. Even though facial-hypoglossal anastomosis was performed in two patients, there was no improvement of facial nerve function. CONCLUSION: Long-term results of NF-2 patients were unfavorable. The early detection of the tumor, regular F/U of patients and individually refined management are important for the optimal treatment of NF-2 patients.