垂体纤维肉瘤 Primary pituitary fibrosarcoma presenting with multiple metastases: a case report and literature review

 Neurol India. 2010 Mar-Apr;58(2):316-8.(第一作者）

Even though many cases of pituitary fibrosarcoma (PF) have been reported, the etiologic classification of these tumors, however, remains undefined. Moreover, owing to the paucity of available case studies, the clinical characteristics of primary pituitary fibrosarcoma (PPF) have not been fully described. We report a 26-year-old female with pathologically confirmed PPF, who presented with features of elevated intracranial pressure, oculomotor nerve palsy, field defects and panhypopituitarism. Despite the combination therapy, which included tumor removal, radiotherapy, and chemotherapy, magnetic resonance imaging demonstrated multiple intracranial and extracranial metastases at a seven-month follow-up, and the survival duration from diagnosis was only 11 months. Based on a review of the literature, we propose preliminary etiologic classification criteria for PF as well as a new therapeutic approach to reduce PPF recurrence and metastasis, including extended surgical resection and postoperative whole-brain radiotherapy.青岛大学医学院附属医院神经外科李照建

垂体纤维肉瘤是罕见的垂体恶性病变.我们遇到一例26岁女性病人,从开始出现症状到临床死亡仅仅11个月的时间,在治疗期间做过经鼻蝶肿瘤切除术,开颅小脑转移灶切除术,放疗及化疗.但病人最终因颅内外多发转移而死亡.我们对这例病人做了报道,并总结了世界上英文报道的相关病例文献,在国际上首次提出垂体纤维肉瘤的病因学分类: (1)鞍区病变(如垂体瘤/颅咽管瘤等)切除术后放疗继发纤维肉瘤样变; (2)泌乳素腺瘤可以自发性发生纤维肉瘤样恶变;(3)原发性垂体纤维肉瘤,一开始就是恶性,病情进展迅速,最容易发生颅内外广泛转移.后者在疾病早期往往就表现为全垂体功能低下,如果术前或者术中快速冰冻能证实诊断,我们提出: 最好做肿瘤连同全垂体切除(即垂体窝清扫术),术后药物内分泌替代治疗,否则很容易复发和转移.