Angelico Roberta, Siragusa Leandro, Pathirannehalage Don Cristine Brooke, Sensi Bruno, Billeci Federica, Vattermoli Leonardo, Padial Belen, Palmieri Giampiero, Anselmo Alessandro, Coppola Alessandro, Tisone Giuseppe, Manzia Tommaso Maria
HPB and Transplant Unit, Department of Surgical Sciences, University of Rome Tor Vergata, 00133 Rome, Italy.
Department of Diagnostic Imaging and Interventional Radiology, Tor Vergata University of Rome, 00133 Rome, Italy.
J Clin Med. 2022 Aug 26;11(17):5021. doi: 10.3390/jcm11175021.
Mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) are a peculiar entity that can occur throughout the whole gastrointestinal trait, and pancreatic localization is rare. Their main characteristic is the presence of at least a neuroendocrine and an epithelial component, each accounting for at least 30% of the tumour mass. The presence of epithelial ductal component defines adeno-MiNEN. We report a case of a 59-year-old woman affected by pancreatic adeno-MiNEN with challenging diagnosis and successfully treated. A systematic literature review and pooled analysis was also performed, aiming to define the management and outcomes of pancreatic adeno-MiNEN. Out of 190 identified records, 15 studies including 28 patients affected by pancreatic-adeno-MiNEN were included in the analysis. Pancreatic adeno-MiNEN occurred mainly in males (82.8%) and at a mean age of 61.7 (range: 24-82) years. Pre-operative diagnosis was possible only in 14.2% of cases. At presentation, the majority had already advanced disease (TNM stage III (53.8%) and stage IV 19.3%). Adjuvant therapy was performed in 55% of patients, and the tumour recurrence rate was in 30% of cases. Median disease-free survival (DFS) was 12 months (range: 0-216 months) with a 5-year DFS of 16.6%, while the median overall survival (OS) was 12 months (range: 0-288 months) with a 5-year OS of 23.5%. Pancreatic adeno-MiNENs are rare; as they have very heterogenous behaviour, they are rarely diagnosed preoperatively and have poor prognosis. Treatment of localised MiNEN still relies on radical surgical resection, which seems essential to achieve a good oncological prognosis. International registry on MiNEN is necessary to improve the knowledge on this rare tumour and to improve its outcomes.
混合性神经内分泌-非神经内分泌肿瘤（MiNEN）是一种特殊的肿瘤类型，可发生于整个胃肠道，胰腺部位较为罕见。其主要特征是至少存在一个神经内分泌成分和一个上皮成分，且每个成分至少占肿瘤体积的30%。上皮导管成分的存在定义为腺性MiNEN。我们报告一例59岁女性胰腺腺性MiNEN患者，诊断具有挑战性但治疗成功。我们还进行了系统的文献综述和汇总分析，旨在明确胰腺腺性MiNEN的治疗及预后情况。在190条检索到的记录中，15项研究共纳入28例胰腺腺性MiNEN患者进行分析。胰腺腺性MiNEN主要发生于男性（82.8%），平均年龄61.7岁（范围：24 - 82岁）。术前仅14.2%的病例可做出诊断。初诊时，大多数患者已处于疾病进展期（TNM分期III期占53.8%，IV期占19.3%）。55%的患者接受了辅助治疗，肿瘤复发率为30%。无病生存期（DFS）中位数为12个月（范围：0 - 216个月），5年DFS率为16.6%；总生存期（OS）中位数为12个月（范围：0 - 288个月），5年OS率为23.5%。胰腺腺性MiNEN较为罕见；因其行为高度异质性难得术前诊断且预后较差。局限性MiNEN的治疗仍依赖根治性手术切除，这似乎是获得良好肿瘤学预后的关键。建立MiNEN国际登记系统对于增进对这种罕见肿瘤的认识并改善其预后很有必要。
