286例功能性胰腺神经内分泌肿瘤的临床特点及治疗:一项单中心回顾性研究

Clinical characteristics and management of 286 patients with functional pancreatic neuroendocrine neoplasms:a single institution retrospective study

目的总结了较大样本功能性胰腺神经内分泌肿瘤(PanNENs)的临床特点及诊疗经过。方法回顾性分析2000年1月至2020年7月中国人民解放军总医院第一医学中心经病理诊断确诊的功能性PanNENs患者的临床资料、相关激素检查、影像学检查及病理结果。结果本研究共纳入286例患者(男性103例,女性183例),平均年龄为(45.55±15.23)岁,平均病程为24(12,60)个月。286例患者中,最常见罹患的是胰岛素瘤(266/286),其次是胰高血糖素瘤(10/286)、生长抑素瘤(3/286)、异位分泌促肾上腺皮质激素(ACTH)的肿瘤(3/286)、胃泌素瘤(2/286)和血管活性肠肽(VIP)瘤(2/286)。胰岛素瘤患者中,9例为多发性内分泌腺瘤病1型(MEN1)患者。肿瘤定位明确且局限的280例患者接受手术治疗。5例远处转移或者术后复发者同时接受介入治疗或药物化疗,经治疗后激素增多的症状得到明显缓解。术后肿瘤病理分级,神经内分泌肿瘤(NET)G1占41.95%、NET G2占54.90%、NET G3占3.15%、神经内分泌癌(NEC)占0%。结论多学科协作并进行系统的内分泌评估和精确定位肿瘤,精准施治,对改善功能性PanNENs患者的预后十分重要。

Objective To summarize the clinical characteristics and management of functional pancreatic neuroendocrine neoplasms(PanNENs)in a large Chinese cohort.Methods Retrospective review was performed in patients with a definite diagnosis of functional PanNENs administrated in the First Clinical Center of Chinese PLA Hospital between January 2000 and July 2020.The clinical data,related hormone examinations,imaging examinations and pathological findings were extracted from their medical records and analyzed.Results A total of 286 patients(male 103,female 183)aged(45.55±15.23)years were diagnosed to have definite functional PanNENs,with a duration of 24(12,60)months.The most frequent functional PanNENs was insulinoma(266/286),followed by glucagonoma(10/286),somatostatinoma(3/286),adrenocorticotropic hormone(ACTH)producing-tumor(3/286),gastrinomas(2/286)and vasoactive intestinal polypeptide(VIP)tumor(2/286).Nine patients were diagnosed to have multiple endocrine neoplasia type 1(MEN1)in which all the associated functional PanNENs were insulinomas.Two hundred and eighty patients with tumor localized in pancreas or with limited metastasis underwent surgery.The symptoms associated with hormonal over-secretion relieved significantly after surgery.Five patients with unresectable or recurrent metastases after surgery underwent systemic chemotherapy or other targeted therapies.With these various therapies,the symptoms also partially relieved.According to findings in pathological and immunochemical examination,all the functional PanNENs were categorized to neuroendocrine tumors(NET)G1(41.95%),NET G2(54.90%),NET G3(3.15%),and neuroendocrine carcinoma(NEC)(0%).Conclusion Multidisciplinary collaboration is essential for precise diagnosis and tumor localization,as well as successful surgery or other targeted therapies,to improve the prognosis of patients suffered from these disorders.