延伸至后颅窝的巨大囊性颅咽管瘤的临床特点和间质内放疗

Clinical features and interstitial radiotherapy of giant posterior fossa cystic craniopharyngiomas

目的 探讨向后颅窝伸展的巨大囊性颅咽管瘤（GPFCCPs）的临床特点和间质内放疗的疗效.方法 对海军总医院近13年收治的26例GPFCCPs进行回顾性分析并对有关文献报道进行综合复习.共有男14例,女12例,年龄3个月至14岁（平均7.2岁）.临床表现为身体发育迟缓6例,颅内高压症状21例;视力下降22例,其他脑神经受损19例和多饮多尿3例.肿瘤体积65～215 ml,平均137.3 ml.均接受囊液抽吸或引流后放射性同位素32P肿瘤内间质放疗和8～ 144个月（平均52.5个月）的随访.结果 共行27次手术操作,治疗33个囊肿.21例颅高压症状患者术后迅速缓解.肿瘤完全消失16例（19个肿瘤）,体积缩小＞75％ 10例（14个肿瘤）.22例视力下降者,完全恢复8例,改善10例,无变化2例,加重2例.其他25根脑神经受损中完全恢复22根,无变化3根.未发现与手术操作及间质内放疗有关的严重并发症.5例患者出现7个新生囊肿,重复治疗后肿瘤完全消失4个,明显缩小2个,进展1个.结论 GPFCCPs是一种少见的肿瘤类型,多见于儿童,无性别差异.以颅内压增高和脑神经受损为主要临床表现.尽管肿瘤体积巨大,放射性同位素间质内放疗仍可取得相对理想的肿瘤控制率和临床疗效和生存质量,且并发症发生率较低.

Objective To investigate the clinical features and efficacy of interstitial radiotherapy for giant posterior fossa cystic craniopharyngiomas （GPFCCPs）.Methods Twenty-six children with GPFCCP admitted to Navy General Hospital in nearly 13 years were analyzed retrospectively,and the related literature reports were reviewed comprehensively.There were 12 females and 14 males.Their age ranged from 3 months to 14 （mean 7.2） years old.The clinical manifestations were stunted body development （n =6）,increased intracranial pressure （n =21）,decreased visual acuity （n =22）,other brain nerves damage （n =19）,and polydipsia and polyuria （n =3）.The tumor volume ranged from 65 to 215 mL （mean 137.3 ml）.The children accepted radioisotope 32p interstitial radiotherapy and were followed-up for 8 to 144 months （mean 52.5 months） after cyst fluid aspiration or drainage.Results A total of 27 surgical procedures were performed and 33 cysts were treated.The intracranial hypertension symptoms of 21 children were relieved immediately after procedure.The tumors completely disappeared in 16 children （19 tumors）,the volume of 10 children （14 tumors） was reduced by 〉 75%.In.22 children with visual loss,8 were recovered completely,10 improved,2 unchanged,and 2 aggravated.In other 25 brain nerves damage,22 were recovered completely,and 3 unchanged.No serious complication associated with surgical operation and interstitial radiotherapy was found.Seven new cysts were found in 5 children.After repeated treatment,4 cysts were disappeared completely,2 reduced significantly,and 1 progressed.Conclusion GPFCCPs is a rare tumor type.It is more common in children without sex differences.The major clinical manifestations are increased intracranial pressure and brain nerves damage.Despite the huge volume of tumor,the interstitial radiotherapy of radioisotope still can achieve a relatively ideal tumor control rate,clinical efficacy and quality of life,and the incidence of complications is lower.