Nießen Anna, Schimmack Simon, Weber Tim F, Mayer Philipp, Bergmann Frank, Hinz Ulf, Büchler Markus W, Strobel Oliver
Department of General, Visceral and Transplantation Surgery, Heidelberg University Hospital, Heidelberg, Germany.
Department of Diagnostic and Interventional Radiology, Heidelberg University Hospital, Heidelberg, Germany.
Pancreatology. 2021 Jan;21(1):224-235. doi: 10.1016/j.pan.2020.11.020. Epub 2020 Dec 3.
BACKGROUND/OBJECTIVES: Mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) of the pancreas and periampullary region are extremely rare and heterogeneous malignancies. Literature is sparse, clinical management is not standardized and little is known about survival outcomes. The aim of this study was to identify pathological and radiological features of MiNEN and assess the outcome of surgical management.
Patients undergoing surgery for pancreatic and periampullary MiNEN between 2001 and 2019 were retrospectively analysed based on a prospective database. Histological, radiological and clinical features were assessed. Survival was analysed in a nested case-control study and matched-pair analyses with pure neuroendocrine neoplasms (pNEN) and ductal adeno- or acinar cell carcinomas of the pancreas. A literature review with focus on survival after surgical resection was additionally performed.
Of 13 patients with MiNEN, 5 had acinar-MiNEN and 8 adeno-MiNEN. Two of 5 (40%) acinar-MiNEN and one adeno-MiNEN patients had liver metastases. All but one adeno-MiNEN (88%) showed preoperative radiological features of pancreatic adenocarcinoma, 3 of 5 (60%) acinar-MiNEN exhibited mainly neuroendocrine features. No surgical mortality was observed. The 5-year overall survival rate in all MiNEN was 40%. Five-year survival rate was 58% in adeno-MiNEN and comparable to that of matched ductal adenocarcinomas (36%) and pNEN (48%). Five-year overall survival rate was 20% in acinar-MiNEN, compared to 39% in acinar carcinoma patients and 59% in matched pNEN patients.
MiNEN are rare and difficult to distinguish from pure adenocarcinoma or neuroendocrine neoplasm preoperatively. Surgical resection would therefore be the treatment of choice in localized tumors.
背景/目的：胰腺和壶腹周围区域的混合性神经内分泌-非神经内分泌肿瘤（MiNEN）是极其罕见且异质性的恶性肿瘤。相关文献稀少，临床管理不规范，对生存结局了解甚少。本研究旨在确定MiNEN的病理和放射学特征，并评估手术治疗的结果。
基于前瞻性数据库，对2001年至2019年间接受胰腺和壶腹周围MiNEN手术的患者进行回顾性分析。评估组织学、放射学和临床特征。在一项巢式病例对照研究中分析生存情况，并与纯神经内分泌肿瘤（pNEN）以及胰腺导管腺癌或腺泡细胞癌进行配对分析。此外，还进行了一项聚焦于手术切除后生存情况的文献综述。
13例MiNEN患者中，5例为腺泡型MiNEN，8例为腺体型MiNEN。5例腺泡型MiNEN中有2例（40%）和1例腺体型MiNEN患者有肝转移。除1例腺体型MiNEN外，所有患者（88%）术前均表现出胰腺腺癌的放射学特征，5例腺泡型MiNEN中有3例（60%）主要表现为神经内分泌特征。未观察到手术死亡病例。所有MiNEN的5年总生存率为40%。腺体型MiNEN的5年生存率为58%，与配对的导管腺癌（36%）和pNEN（48%）相当。腺泡型MiNEN的5年总生存率为20%，相比之下，腺泡癌患者为39%，配对的pNEN患者为59%。
MiNEN罕见，术前难以与纯腺癌或神经内分泌肿瘤区分。因此，手术切除是局限性肿瘤的首选治疗方法。
