Morimoto K, Yoshimine T, Saitoh Y, Yamada M, Mogami H, Yamagata S, Miyagawa J
Department of Neurosurgery, Osaka University Medical School, Japan.
No Shinkei Geka. 1987 Aug;15(8):877-82.
Werner's syndrome is rare and autosomal recessive condition with multiple progeroid features. There is an increased incidence of neoplasm such as meningioma or sarcoma in association with this syndrome. However, the pathogenesis of this subject is still controversial. To date, only two cases have been reported, dealing with surgical operation. Our present case of a 39-year-old woman who had a meningioma associated with Werner's syndrome is the third of such cases. In this rare case we did histopathological examinations including electron microscopic and immunohistochemical studies, which showed intense proliferation of the connective tissue in the tumor tissue. The present morphological study may imply a close relationship between proliferation of collagen fibers in the meningioma and the aberration of connective tissue metabolism in context of theory of the pathogenesis of Werner's syndrome.
沃纳综合征是一种罕见的常染色体隐性疾病，具有多种早老性特征。该综合征常伴有如脑膜瘤或肉瘤等肿瘤发病率的增加。然而，该疾病的发病机制仍存在争议。迄今为止，仅有两例关于手术治疗的报道。我们目前报道的这例39岁患有与沃纳综合征相关脑膜瘤的女性患者是此类病例中的第三例。在这个罕见病例中，我们进行了组织病理学检查，包括电子显微镜和免疫组织化学研究，结果显示肿瘤组织中的结缔组织有强烈增殖。目前的形态学研究可能意味着在沃纳综合征发病机制理论背景下，脑膜瘤中胶原纤维的增殖与结缔组织代谢异常之间存在密切关系。
