肾上腺原始神经外胚叶肿瘤1例报告并文献复习

Diagnosis and treatment of primitive neuroectodermal tumor of the adrenal gland:a case report and review of the literature

目的探讨肾上腺原始神经外胚叶肿瘤的临床表现、病理特点、治疗方法及预后。方法分析1例右侧肾上腺原始神经外胚叶肿瘤患者的病例资料并复习相关文献。结果手术根治切除肾上腺肿瘤,病理诊断为肾上腺原始神经外胚叶肿瘤,术后采取辅助大剂量放疗,半年后右侧腹壁肿瘤转移,再次手术切除转移灶,术后2月复查B超见右肾上腺区肿瘤复发,患者1年后死亡。结论肾上腺原始神经外胚叶肿瘤临床罕见,病理及免疫组化是原始神经外胚叶肿瘤确诊的金标准。手术是早期患者首选的治疗方法,晚期患者可以辅助联合化疗,对预后尚没有评估依据。

Objective To investigate the clinical and pathological characteristics,treatment and prognosis of primitive neuroectodermal tumor（PNET） of the adrenal gland.Methods The clinical data and pathological characteristics of a PNET case diagnosed and treated in Beijing Friendship Hospital was analyzed and relevant literature was reviewed.Results After the diagnosis was confirmed by pathological and immunohistochemical examinations,the patient received radical adrenalectomy followed by radiotherapy.Six months later,metastasis was found on the right abdominal wall and the abdominal tumor was removed.Two months later,metastasis was found in the adrenal region by B-ultrasound examination.The patient died of metastases one year later.Conclusions Pathological and immunohistochemical examinations are necessary for the diagnosis of PNET.Surgery is the best choice for tumors in early stage,and for late-stage tumors,chemotherapy and radiotherapy should be followed after surgery.PNET of the adrenal gland is a rare disease and there are not enough evidences for its prognosis.