Sporadic NF2 Mosaic: Multiple spinal schwannomas presenting with severe, intractable pain following pregnancy.
Sporadic NF2 Mosaic: Multiple spinal schwannomas presenting with severe, intractable pain following pregnancy.
复制标题
DOI：
10.1016/j.inat.2017.09.006
复制
发表时间：
2017-12
期刊：
影响因子：
--
通讯作者：
Shin JH
中科院分区：
文献类型：
--
作者：
Zimering JH;Choi BD;Koch MJ;Dewitt JC;Stemmer-Rachamimov A;Shin JH
本文的目的是报告未确诊的散发性神经纤维瘤病2型表现为症状压迫脊柱肿瘤后怀孕。一名36岁的女性在妊娠中期出现进行性、严重的腰神经根痛，分娩后不久就变得顽固。磁共振成像显示圆锥周围有一个复杂的异质性低信号肿块。马尾有两个小的点状病灶，提示粘液乳头状室管膜瘤伴“点状转移”。患者接受了两个囊性压迫性圆锥肿块的手术切除。她的腰痛在手术后有所改善。肿块与囊性/细胞性神经鞘瘤一致。一个偶然的发现是一个小的，低级别的脊髓室管膜瘤，缺乏典型的组织学特征的粘液乳头状室管膜瘤。多发性、大的囊性神经鞘瘤在神经鞘瘤病中并不少见，然而，低级别室管膜瘤的同时发生强烈提示临床诊断为新发、散发性神经纤维瘤病2型。虽然颅神经鞘瘤（眼眶，听觉）已报告在怀孕期间扩大，据我们所知，这是第一次报告的多囊性/细胞神经鞘瘤造成严重的疼痛（由于圆锥压缩）在怀孕期间和怀孕后立即。
The aim of the present paper is to report undiagnosed sporadic neurofibromatosis type 2 presenting with symptomatic compressive spinal tumors following pregnancy. A 36-year-old woman experienced progressive, severe lumbar radicular pain in the second trimester of pregnancy which became intractable soon after delivery. Magnetic resonance imaging revealed a complex heterogeneous hypointense mass lesion around the conus. There were two small punctate lesions in the cauda equina suggestive of myxopapillary ependymoma with ‘drop metastases.’ The patient underwent surgical resection of two cystic compressive conus masses. Her low back pain improved after surgery. The masses were consistent with cystic/cellular schwannomas. An incidental finding was of a small, low-grade spinal ependymoma which lacked the characteristic histologic features of myxopapillary ependymoma. Multiple, large cystic schwannomas are not uncommon in schwannomatosis, however, the co-occurrence of low-grade ependymoma strongly suggests a clinical diagnosis of new, sporadic neurofibromatosis type 2. Although cranial nerve schwannomas (orbital, auditory) have been reported to enlarge during pregnancy, to our knowledge, this is the first report of multiple cystic/cellular schwannomas causing severe pain (due to conus compression) during and immediately after pregnancy.