蝶鞍区生殖细胞瘤的鉴别诊断和治疗

总结北京协和医院神经外科1991年3月至2002年1月间11例经术后病理证实为蝶鞍区生殖细胞瘤患者的临床资料。其中,男性2例,女性9例。平均年龄16.8岁,平均病程2.6年。临床表现为尿崩症11例,视功能障碍6例,生长发育迟缓5例,垂体功能低下5例。肿瘤最大径≤3cm8例,>3cm3例。MRI显示肿瘤为T1呈低或等信号8例。术中肿瘤呈紫红色或暗灰色。术后病理均证实为生殖细胞瘤,术后均进行放射治疗。随诊3个月至6年,肿瘤消失,术后尿崩症状改善9例,视功能障碍改善4例,但垂体功能低下和生长发育迟缓改善不明显。研究结果显示当年轻人出现尿崩症、垂体功能低下,影像检查发现蝶鞍区实质肿瘤边界不清时,应考虑蝶鞍区生殖细胞瘤,高度怀疑时采用试验性放射治疗可协助诊断。

Diagnosis and treatment of sellar germinoma

That eleven patients with sellar germinomas were confirmed by pathological examinations were retrospectively reviewed. In the 11 patients, 2 were males and 9 were females. Mean age was 16.8 years old and mean history was 2.6 years. Among them, 11 patients had diabetes insipidus, 6 patients had visual disturbances, 5 patients had retarded growth, and 5 patients had hypopituitary function. The diameter of tumor was 1-3 cm in 8 cases and greater than 3 cm in 3 cases. MRI showed hypointensity or homogeneous on T1-weighted images in 8 cases. The tumors were fibrous, purple grey or reddish in color with rich blood supply. Germinomas were confirmed by postoperative pathological examinations. All patients received postoperative irradiation. Postoperative follow-up was from 3 months to 6 years. Diabetes insipidus was improved in 9 cases. Visual function were improved in 4 cases, but hypopituitarism and retarded growth were unchanged. In conclusion, when MRI shows sellar parenchyma tumor with unclear boundary in young patients with diabetes insipidus and hypopituitarism, sellar germinoma should be highly suspected. Otherwise, radiation therapy may profitably aid to confirm the diagnosis since irradiation is the main treatment of sellar germinomas.