胰腺原发性神经内分泌肿瘤38例临床病理分析  

Primary pancreatic neuroendocrine neoplasms: a clinicopathological analysis of 38 cases

作　　者：汪锋 朱翔[2,3] 李怡婧 陈小曼[2,3] 陶明[4] 石雪迎[2,3] 

机构地区：[1]内蒙古自治区人民医院病理科,呼和浩特010017 [2]北京大学医学部病理学系 [3]北京大学第三医院病理科,北京100191 [4]北京大学第三医院普外科,北京100191

出　　处：《诊断病理学杂志》2014年第7期418-422,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的 探讨胰腺原发性神经内分泌肿瘤的临床病理特征及相关鉴别诊断.方法 收集38例原发性胰腺神经内分泌肿瘤的临床和病理资料,根据WHO（2010）消化系统神经内分泌肿瘤的分级标准进行形态评估,并结合文献讨论其临床特点、病理诊断、鉴别诊断以及治疗和预后.结果 38例中18例为女性,20例为男性;平均年龄49.9±14.9岁.其中功能型肿瘤17例,表现为相应的激素分泌症状;无功能型21例,多无临床症状或表现为急性胰腺炎.肿瘤最大直径0.5～17 cm,平均3.6±3.1 cm.肿瘤主要由小圆形细胞构成,细胞排列成团巢状、缎带状、腺样、实性;间质为丰富的薄壁血管,大多数情况下间质促纤维反应明显.免疫组化示CgA和Syn多呈弥漫（＋）,广谱CK和E-cadherin（＋）,vimentin、CD10和β-catenin（核）等标记则多为（-）.组织学分级：19例为G1,10例为G2,9例G3（NEC）.随访时间4～71个月;死亡5例,其中G3（NEC）4例,G21例.结论 胰腺原发性神经内分泌肿瘤较为少见,应和实性假乳头状肿瘤及腺泡细胞癌等其他少见胰腺原发性肿瘤相鉴别.较高级别的神经内分泌肿瘤预后相对较差.准确详尽的病理评估对指导治疗和提示预后有重要意义.Objective To investigate the clinicopathological features and differential diagnosis of pancreatic neuroendocrine neoplasms （PNEN）.Methods 38 cases of PNEN from Peking University Third Hospital were retrospectively studied.All the tumors were regraded according to WHO 2010 digestive system neuroendocrine tumor classification.The clinical features,pathologic diagnosis,differential diagnosis,treatment and prognosis were discussed according to the WHO 2010 digestive system neuroendocrine tumor classification standard.Results There were 18 females and 20 males,and age averaged 49.9 ± 14.9 years.Functional tumor cases （n =17） showed the corresponding hormone secretion symptoms.No functional cases （n =21）,showed no clinical symptoms or presented with acute pancreatitis.The maximum dimension of neoplasms were from 0.3 to 17.0 cm （average 3.6 ± 3.1 cm）.PNEN was composed of small round cells arranged in nest,ribbon,glandular and solid architecture,in most cases,with desmoplastic changes.Immunohistochemistry showed most of neoplasm cells diffusely expressed CgA,Syn,CK,and E-cadherin; but vimentin,CD10 and β-catenin （nuclear） were negative.Histological grading showed that 19 cases was G1,10 cases was G2,and 9 cases was G3.Follow up for 4-71 months showed 5 patients dead of disease,including 4 G3 cases and 1 G2 case.Conclusion Primary PNEN is rare.PNEN should be distinguished from solid-pseudopapillary neoplasm and acinar cell carcinoma.Patients with high grade PNEN have a relatively poor prognosis.Accurate and detailed pathological assessment is important to guide the treatment and prognosis.

关 键 词：胰腺神经内分泌肿瘤 组织学分级 生物学行为 鉴别诊断 

分 类 号：R736[医药卫生—肿瘤]