Multiple inherited Schwannomas, meningiomas, and ependymomas (MISME): A case report on rare case of neurofibromatosis type 2 tumors

• Main Authors: Ahmad Faried, MD, PhD, Guata Naibaho, MD, Rully Hanafi Dahlan, MD, MSc, Roland Sidabutar, MD, MSc, Sevline Estethia Ompusunggu, MD, MSc, Muhammad Zafrullah Arifin, MD, PhD
• Format: Article
• Language: English
• Published: Elsevier 2017-12-01
• Series: Interdisciplinary Neurosurgery
• Online Access: http://www.sciencedirect.com/science/article/pii/S2214751917301123

Neurofibromatosis type 2 (NF-2) is an autosomal dominant inheritance, chromosome 22q12 mutations in peripher and central nervous systems. Several literatures have discussed rare cases of triple NF-2 tumor, known as multiple inherited Schwannomas, meningiomas and ependymomas or MISME. Incidence of MISME reported from many country, but never been reported from Indonesia. Hopefully the MISME presence can be more revealed in our country and adding the evidence in world literature. A 15-year-old boy presented to our center with a progressive bilateral hearing loss for 2 years and started to experience weakness of both lower extremities since 1.5 years. Patient underwent both craniotomy intracranial and spinal tumor removal. Histopathological result showed Schwannomas, meningiomas and ependymoma. Diagnosis of MISME was made and had been treated with excellent clinical outcome. We are reporting a rare case of MISME syndrome, which is an extremely exceptional case in our center due to rare occurrence of MISME and the need to confirm NF-2 diagnosis using molecular investigations. Keywords: Neurofibromatosis type 2, MISME syndrome, Rare case report