Honczarenko K, Grzelec H, Nowacki P, Fabian A
Department of Neurology, Medical School, Szczecin.
Folia Neuropathol. 1994;32(4):255-8.
A case of 32-year-old woman was described in which progressive bilateral hearing and visual loss, hypokinesia, epileptic focal seizures were present. The brain CT-scan after a few years of the disease onset, showed the presence of multiple extracerebral tumors. Multiple meningiomas were diagnosed, probably associated with von Recklinghausen's disease. On the post-mortem examination 48 tumors of the different size and location in subdural space were found. Histologically transitional meningiomas with predominance of fibroblastic component were diagnosed. It was very interesting that apart from intracranial location of meningiomas, the same type of tumor was found within thoracic spinal root. The authors discuss the mechanisms of the development of multiple meningiomas and its association with neurofibromatosis.
描述了一例32岁女性病例，该患者出现进行性双侧听力和视力丧失、运动功能减退、癫痫局灶性发作。疾病发作几年后的脑部CT扫描显示存在多个脑外肿瘤。诊断为多发性脑膜瘤，可能与冯·雷克林豪森病有关。尸检发现硬膜下间隙有48个大小和位置各异的肿瘤。组织学诊断为以成纤维细胞成分占主导的过渡型脑膜瘤。非常有趣的是，除了颅内有脑膜瘤外，在胸段脊神经根内也发现了相同类型的肿瘤。作者讨论了多发性脑膜瘤的发生机制及其与神经纤维瘤病的关联。
