4.6 Article

Spontaneous regression of vestibular schwannomas after resection of contralateral tumor in neurofibromatosis Type 2 Report of 2 cases

Journal

JOURNAL OF NEUROSURGERY
Volume 112, Issue 1, Pages 158-162

Publisher

AMER ASSOC NEUROLOGICAL SURGEONS
DOI: 10.3171/2009.5.JNS09240

Keywords

neurofibromatosis Type 2; tumor regression; vestibular schwannoma; natural history

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The authors report on 2 patients with bilateral vestibular schwannomas (VSs) who underwent unilateral surgical tumor removal. One patient was followed up for 4 years, the other for 9; in both cases, the contralateral VS regressed markedly without any additional treatment during the follow-up period. Serial MR imaging was performed to monitor the untreated tumor, which in both cases involved the only hearing ear. The tumors were assessed volumetrically. The contralateral tumors appeared to enlarge mildly at initial follow-up and then, with no treatment, regressed (to 23% of the original maximum volume in Case 1 and to 15% of the original maximum in Case 2). The largest posterior fossa diameter decreased from 30.1 mm to 18.6 mm in Case 1 over 4 years and from 27 mm to 16 mm over 8 years in Case 2. Hearing declined only mildly during follow-up in both patients. These cases demonstrate the first well-documented, long-term, spontaneous VS regressions in patients with neurofibromatosis Type 2. They underline the importance of careful observation of VS involving the only hearing ear in the management of bilateral VS to determine the natural growth pattern of the tumors. The mechanism of the dramatic spontaneous tumor regression is uncertain. (DOI: 10.3171/2009.5.JNS09240)

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