小儿性腺外内胚窦瘤3例诊断与治疗分析

Diagnosis and treatment of primary endodermal sinus tumor out of gonad in children

目的总结小儿原发性腺外内胚窦瘤的诊断与治疗经验。方法回顾性分析我院2003年10月-2011年9月收治的3例性腺外内胚窦瘤患儿的病历资料。结果术前均行PEB或JEB方案新辅助化疗,根治性手术切除,术后病理均为完全缓解,术后继续化疗。随访3个月～8年,2例无病存活,1例原位复发,经术前化疗、再次手术切除及术后化疗后,现随访25个月,无病存活。结论甲胎蛋白水平对小儿性腺外内胚窦瘤诊断及术后复发监测灵敏度较高,化疗与手术结合治疗效果良好。

Objective To review the diagnosis and treatment of endodermal sinus tumor out of gonad in children. Methods Clinical data of 3 patients with endodermal sinus tumor out of gonad admitted in our hospital from October of 1994 to September of 2011 were reviewed retrospectively. One boy had the tumor in mesostenium and 2 girls had the tumors in cervix. They were diagnosed based on their clinical manifestations, imaging examinations, high levels of serum AFP and pathologic results. Results All patients received PEB or JEB neoadjuvant chemotherapy before operation. After radical excision, they continued to receive adjuvant chemotherapy. The pathological results showed completely remission. After 3-month-to-8-year followed-up, two cases remain disease-free survival. One got relapse in situ, and remain disease-free survival until now after the operation and chemotherapy. Conclusions The serum AFP level is sensitive to the diagnosis and relapse. The combination of surgery and chemotherapy can achieve a good therapeutic effect in children with endodermal sinus tumor out of gonad.