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Bilateral Spontaneous Regression Of Vestibular Schwannoma In Neurofibromatosis Type 2: A Case Report.

Sebök et al., 2018Brain tumor

Sebök, M., van Niftrik, C. H. B., & Bozinov, O. (2018). Bilateral spontaneous Regression of Vestibular Schwannoma in Neurofibromatosis Type 2. World neurosurgery, 113, 195–197. https://doi.org/10.1016/j.wneu.2018.02.075

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Abstract

BACKGROUND: Patients with neurofibromatosis type 2 and bilateral vestibular schwannoma (VS) are frequently treated surgically for any tumor progression, and often repeated surgery or radiation treatment is even considered. Some VS progression occurs without the development of new clinical symptoms, or it does not progress in size over many years, even in the absence of any specific treatment. CASE DESCRIPTION: A 61-year-old male patient with neurofibromatosis type 2 presented with bilateral VS. In a long-term follow-up, both had increased in size but also showed bilateral spontaneous regression during an 11-year follow-up period with a "watch-and-wait" strategy. CONCLUSIONS: We emphasize conservative treatment ("watch and wait") in older patients even with long-term tumor progression without significant compression-related clinical symptoms.

Case Details

Disease Location

Bilateral vestibular schwannomas

Personal Characteristics

A 61-year-old patient with nf2 and bilateral vs, an intraventricular meningioma on the right side, three falx meningiomas, and thoracal and lumbal neurinomas (t2, t5 and l3). Nf2 confirmed via genetic testing and family history.

Clinical Characteristics

Patient initially daignosed in early 1980s with hearing loss. Consulted neurosurgery in 2006. MRI did not show any mass effect, so conservative wait and watch strategy adopted. Long term follow up showed worsening hearing loss with progression of right side tumor. Treatment discussed with patient and multidisciplinary conference - patient declined treatment. Follow up in 2015 showed stable size and complete regression in 2017. With regard to his other intracranial mass lesions, the intraventricular meningioma and one of the falx meningioma were discretely progressive in size. The rest of the tumors was stable over the whole period. None showed signs of growth regression in any of the mr imaging.

Remission Characteristics

Sought treatment in 2006, diligent observation -> follow up in 2015 showed stable size and complete regression in 2017. With regard to his other intracranial mass lesions, the intraventricular meningioma and one of the falx meningioma were discretely progressive in size. The rest of the tumors was stable over the whole period. None showed signs of growth regression in any of the mr imaging.

Treatment & Mechanisms

Proposed Remission Mechanisms

Authors hypothesize spontaneous intratumoral vascular thrombosis and subsequent ischemic necrosis following fibrosis

Non-Clinical Treatment

Authors confirm patient was not on any pharmaceutical or herbal medications