MISME Syndrome:A Case Report Of Neurofibromatosis Type-2
Mnemonic MISME syndrome is a rare neurofibromatosis type-2 (NF2) for multiple inherited schwannomas,meningiomas,and ependymomas.It is an autosomal dominantly inherited disorder due to mutations of a tumor-suppressor gene on the chromosome 22q12.Clinically it is characterized by multiple benign tumors arising in both the central and the peripheral nervous system,particularly from the bilateral vestibulocochlear nerve in more than 90% of the patients withNF2 and more than two thirds of them develop spinal tumors,which are often devastating and difficult to manage.Simultaneous occurrence of triple tumors in an NF2 patient is rare.Furthermore,simultaneous occurrence of the triple tumors in a shared common location is even rarer.We reported here a young woman with MISME syndrome with triple tumors affecting the cervical spinal cord in whom staging microsurgical resection intervention produced a clinically favorable outcome.Histological reports with immunochemical staining showed the presence of schwannomas,meningiomas,and ependymomas.We claimed that surgical debulking on those symptomatic craniospinal tumors is the option of treatment of the choice.Staging microsurgical techniques may reduce adverse outcomes.
MISME syndrome,Neurofibromatosis type 2 craniospinal tumors staging operation,microsurgery,schwannomas,meningiomas,ependymomas
Jin Xin He Junhua Wang Dingkun Zhang Xinwen Wu Ye Liu Hanjiang Wu Zhangyi Jin Jun Luo
Neurosurgery Department,Zhejiang Provincial Tongde Hospital,Hangzhou,Zhejiang,China Department of Neurology,Temple University School of Medicine,Philadelphia,PA,USA
国内会议
北京
英文
79-87
2014-06-26（万方平台首次上网日期，不代表论文的发表时间）