检索历史 清除

目的 探讨鞍区罕见肿瘤的临床特点及其显微手术治疗,以提高对该病的诊治水平.方法 收集经显微手术治疗的6例鞍区罕见肿瘤患者的临床资料进行回顾性分析,并结合术前肿瘤标记物甲胎蛋白(AFP)和绒毛膜促性腺激素(HCG)联合检测与术后免疫组化染色指导后续治疗.结果 本组6例患者术前全部误诊,术毕病变镜下全切2例,大部切除4例.病理诊断肿瘤5例,炎性假瘤1例,病理类型分别为平滑肌肉瘤、卵黄囊瘤、混合性生殖细胞肿瘤、胚胎癌和毛细胞性星形细胞瘤及霉菌性炎性假瘤.术前AFP或HCG在卵黄囊瘤、胚胎癌和混合性生殖细胞瘤中均有不同程度升高.随访1个月至3年,卵黄囊瘤、胚胎癌和平滑肌肉瘤患者分别在术后5、6、10个月死亡,炎性假瘤患者术后2个月出现蛛网膜下腔出血,病情危重,余生存良好.结论 鞍区罕见肿瘤以高度恶性生殖细胞肿瘤为主,病理类型复杂,术前误诊率高,临床预后差.术前AFP和HCG联合检测对指导治疗、判断预后有一定意义.

Objective To investigate the clinical characteristics and microsurgical managements of rare tumors in the sellar region. Methods Six rare cases of tumors in the sellar region treated by microsurgery from Jan 2000 to Jan 2010 were reviewed retrospectively.Subsequent treatments were according to the status of preoperative alpha fetal protein(AFP) and human chorionic gonadotropin(HCG) measurement as well as confirmed by histopathological examination in all six patients. Results Total resection of the tumor was achieved in 2 cases and subtotal resection in 4 cases.Postoperative histopathology confirmed that the lesions were tumors in 5 cases and fungal pseudotumor in 1 case.Moreover,variety of histological types were observed in the present series,including leiomyosarcoma,malignant yolk sac tumor,mixed germ cell tumor,embryonal carcinoma,pilocytic astrocytoma and fungal pseudotumor,respectively.The serum levels of AFP and HCG were elevated to some extent in the patients with malignant yolk sac tumor,mixed germ cell tumor or embryonal carcinoma.Follow-up was conducted in all patients for 1 month to 3 years.The patients with malignant yolk sac tumor and embryonal carcinoma as well as leiomyosarcoma died in 5,6,10 months after operation,respectively.Subarachnoid hemorrhage occurred in the case of fungal pseudotumor at 2 months sfter surgery.The other two patients were surviving well. Conclusions Rare nongerminomatous malignant germ cell tumors are predominantly susceptible to the sellar region.Furthermore,High misdiagnosis rate and poor prognosis are characteristic in the present study.Dynamic AFP and HCG detection may play an important role in the diagnosis of those non-germinomatous malignant germ cell tumors located in the sellar region.The importance of awareness of the presence of such rare lesions in the sellar region is emphasized.