Ahn Jung Yong, Kwon Seong Oh, Shin Moon Soo, Shim Jeong Yun, Kim Ok Joon
Department of Neurosurgery, Pundang CHA Hospital, Pochon CHA University, Sungnam, 463-070, Korea.
Yonsei Med J. 2002 Feb;43(1):109-13. doi: 10.3349/ymj.2002.43.1.109.
In most cases, while schwannoma is sporadically manifested as a single benign neoplasm, the presence of multiple schwannomas in one patient is usually indicative of neurofibromatosis 2. However, several recent reports have suggested that schwannomatosis itself may also be a distinct clinical entity. This study examines an extremely rare case of probable schwannomatosis associated with intracranial, intraspinal and peripheral involvements. A 63-year-old woman presented with a seven-year history of palpable lumps on both sides of the supraclavicular area and hearing impairment in both ears. On physical examination, no skin manifestations were evident. Facial sensory change, deafness in the left ear and decreased gag reflex were revealed by neurological examination. Magnetic resonance imaging revealed multiple lesions of the trigeminal nerves, acoustic nerves, lower cranial nerves, spinal accessory nerve, brachial plexuses, and spinal nerves. Pathological examination of tumors from the bilateral brachial plexuses, the spinal nerve in the T8 spinal position and the neck mass revealed benign schwannomas. Following is this patient case report of multiple schwannomas presenting with no skin manifestations of neurofibromatosis.
在大多数情况下，虽然神经鞘瘤通常以单发良性肿瘤的形式散发性出现，但一名患者出现多个神经鞘瘤通常提示为神经纤维瘤病2型。然而，最近的几份报告表明，神经鞘瘤病本身也可能是一种独特的临床实体。本研究报告了一例极为罕见的可能与颅内、脊柱内及周围受累相关的神经鞘瘤病病例。一名63岁女性，有锁骨上区双侧可触及肿块7年病史及双耳听力障碍。体格检查未发现明显皮肤表现。神经科检查发现面部感觉改变、左耳失聪及咽反射减弱。磁共振成像显示三叉神经、听神经、低位颅神经、副神经、臂丛神经和脊神经有多处病变。对双侧臂丛神经、T8脊髓位置的脊神经及颈部肿块的肿瘤进行病理检查，结果显示为良性神经鞘瘤。以下是该例无神经纤维瘤病皮肤表现的多发性神经鞘瘤患者的病例报告。
