小肠异位胰腺 1例报告及文献复习

目的探讨异位胰腺的诊治，提高对该疾病的认识以减少误诊。方法回顾性分析 1例小肠异位胰腺病人的临床表现、诊断及治疗经过，结合文献分析其组织病理分型、临床表现和治疗经验。结果病人因“突发腹痛 1d”入院。入院后在全麻下急诊行开腹探查术，于术中见“小肠表面可及一肿物，肿物穿孔可见小肠内容物溢出”。术后病理证实肿物为异位胰腺。术后恢复满意，术后第 90天随访无并发症及影像学检查异常。结论异位胰腺属于一种先天性异常，缺乏特异性诊断方法，早期多无症状，因此可保守治疗；如有症状或术中意外发现为异位胰腺组织时，可手术切除。     

Early Diagnosis of a Pediatric Pancreatic Tumor by Abdominal Physical Examination: A Case Report

BackgroundDetecting a neoplasm in pediatric patients with acute abdominal pain is difficult, especially when there is no palpable mass. Ihara's maneuver, which allows the physician to apply manual pressure directly on the pancreatic body, is a useful palpation method of diagnosing acute pancreatitis in children. We report a case of solid pseudopapillary neoplasm of the pancreas (SPNP) detected by Ihara's maneuver.Case ReportAn otherwise healthy, 15-year-old male visited our emergency department with acute abdominal pain and nausea. His vital signs were appropriate for his age. A physical examination denied peritoneal signs, but Ihara's maneuver induced strong tenderness. His serum amylase and lipase were normal. A contrast-enhanced computed tomography scan revealed a well-defined, 2.2-cm, nonenhanced mass in the pancreatic tail. Laparoscopic distal pancreatectomy was performed, and the diagnosis of SPNP was confirmed. The patient was well postoperatively without any metastasis. SPNP is a rare neoplasm with low malignant potential. Although it typically occurs in young females, it has also been reported in children. The early diagnosis of SPNP is usually challenging because most patients do not have specific symptoms or laboratory findings. In the present case, the SPNP was difficult to detect by palpation because of its size, but Ihara's maneuver induced strong tenderness of the pancreas and led to a diagnosis.Why Should an Emergency Physician Be Aware of This?Ihara's maneuver has the potential to enable early diagnosis not only of pancreatitis but also of pancreatic tumors, such as SPNP.     

胰腺囊性肿瘤诊疗及进展

胰腺囊性肿瘤(PCNs)是少见肿瘤，发病原因尚不明确，不良生活习惯（吸烟、饮酒、重咖啡、高脂高蛋白饮食等）、慢性胰腺炎、环境污染因素及遗传因素等是潜在致病因素。PCNs分为浆液性囊性肿瘤（SCN）、黏液性囊性肿瘤（MCN）、胰腺导管内乳头状黏液肿瘤(IPMN)和实性假乳头状瘤（SPN）四种类型。发病症状常不典型，早期诊断难。PCNs具有典型影像特点，单个影像检查技术对PCNs的准确性和局限性不同，CT检查在胰腺病变中仍是最基本、最主要的检查方式。MRI对于小的囊性病灶比CT更有优势。超声内镜（EUS）充分结合了内镜和超声检查的优势，与CT、MRI检查相辅相成，同时还可进行细针穿刺取病理及囊液分析。尽管PCNs大部分为良性，但只要达到切除标准，均应推荐患者进行手术治疗，严格遵循PCNs诊治流程，制订个体化PCNs治疗策略，使患者利益最大化。     

Clinical presentation and prognosis of adenosquamous carcinoma of the pancreas – Matched-pair analysis with pancreatic ductal adenocarcinoma

IntroductionAdenosquamous carcinoma of the pancreas (ASCP) is a rare subtype of pancreatic adenocarcinoma. The aim of this study was to investigate the characteristics and outcomes of ASCP in comparison to pancreatic ductal adenocarcinoma (PDAC).Materials and methodsAll patients with ASCP treated between December 2001 and December 2017 were identified from a prospective database. Clinicopathological and follow-up data were analyzed. A nested case-control-study with matched-pair analysis was performed to compare overall survival of ASCP and PDAC.ResultsOf 4009 patients undergoing surgery for pancreatic adenocarcinoma 91 patients had ASCP. Compared to PDAC ASCP were larger (4.0 vs. 3.2 cm; p < 0.0001), more frequently involved lymph nodes (88% vs. 78%; p = 0.0216), more frequently showed poor differentiation (G3: 79% vs. 36%; p < 0.0001) and more frequently were located in the pancreatic tail (19% vs. 10%; p = 0.0179). Overall median post-resection-survival was shorter in ASCP (10.8 vs. 20.5 months in PDAC; p = 0.0085), but 5-year survival rates were comparable (18.2% vs. 17.5%). After matching for the unevenly distributed prognostic factors survival after resection of ASCP and PDAC was comparable (p = 0.8301). Localization in the head or several parts of the pancreas, high CA 19-9 levels, and M1 disease were independent predictors of survival in patients with ASCP.ConclusionASCP is more aggressive with poorer differentiation and higher rates of lymph node metastases compared to PDAC. In spite of a shorter median survival, 5-year survival rates after surgical resection of about 18% can be expected in ASCP and support resection as part of a multimodal therapy as the treatment of choice in this rare cancer.     

不同病理分级的胰腺神经内分泌肿瘤的MRI征象对比分析及“主胰管绕道征”的诊断价值探讨

目的:对比分析G1、G2级胰腺神经内分泌肿瘤（pNEN）的MRI征象，探讨“主胰管绕道征”的诊断价值。方法:收集32例pNEN患者的临床资料，以术后病理学检查为金标准，定量分析G1、G2级临床指标及影像学指标的差异，并着重分析pNEN与主胰管的关系。结果:32例患者中，病理分级G1、G2、G3级的患者分别有12例、16例、4例，分别以胰尾、胰头、胰颈为主要发生部位。与G1级神经内分泌瘤（NET）相比，G2级NET肿瘤直径显著增大，不规则形态的病灶增多，边缘模糊，生物学行为出现明显恶性倾向，主胰管扩张、胰腺外侵犯及淋巴结/肝转移的发生率升高。G1级NET的MRI平扫信号均质程度高于G2级（P＜0.05），但二者在增强MRI扫描的各个期象信号强度均无明显差异（P＞0.05）。28例NET患者中，22例（78.6%）出现主胰管绕道征；神经内分泌癌（NEC）患者无一例出现此征象。术前，28例NET的诊断准确率为78.6%（22/28），存在6例误诊，其中，3例误诊为胰腺癌，2例误诊为囊腺瘤（癌），1例误诊为胰腺假性囊肿。在原MRI诊断依据的基础上纳入“主胰管绕道征”，诊断准确率提高至89.3%（25/28），但仍存在3例误诊，其中2例误诊为囊腺瘤（癌），1例误诊为胰腺假性囊肿，排除了胰腺癌的误诊。结论:MRI对鉴别诊断良恶性pNEN有较高的准确性，但对部分G1与G2级NET仍存在一定的误诊率，主胰管绕道征可帮助减少出现胰腺癌误诊。     

Fetal pancreatic Langerhans islets size in pregnancies with metabolic disorders

Objective: Metabolic disorders are a pandemic and increasing health problem. Women of childbearing age may also be affected, thus an abnormal metabolism may interfere with pregnancy short- and long-term outcomes, harming both mother and child. In the context of an abnormal maternal and intrauterine metabolic milieu the development of fetal organs, including pancreas, may be affected.

Aim: To investigate the effects of pregnancy metabolic disorders on the morphology of pancreatic Langerhans islets in human late-third trimester stillborn fetuses.

Methods: Samples from fetal pancreas underwent a quantitative histological evaluation to detect differences between pregnancy with (cases, n?=?9) or without (controls, n?=?6) abnormal metabolism.

Results: Results show that the islets size increases in fetuses from dysmetabolic pregnancies and that this increment is related to both beta-cell hyperplasia and hypertrophy. Moreover, according to pregnancy and fetal metabolic disorders, a threshold of abnormal size of the islets has been identified. Above this threshold the size of fetal pancreatic Langerhans islets should be considered excessively increased.

Conclusion: The study suggests that an accurate fetal pancreas analysis supplies an important tool in stillborn fetus, to discover metabolic disturbances that should be kept in mind and managed in future pregnancies.     

Subtyping of intraductal papillary mucinous neoplasms – pitfalls of MUC1 immunohistochemistry

Intraductal papillary mucinous neoplasms (IPMNs) are precursor lesions of pancreatic ductal adenocarcinoma (PDAC). Current edition of WHO Classification of Tumors of the Digestive System recognizes four different subtypes (gastric, intestinal, pancreatobiliary, and oncocytic) and recommends analysis of mucin expression (MUC1, MUC2, MUC5AC, MUC6) as well as evaluation of architectural and cell differentiation patterns for correct classification. However, there is no consensus on MUC1 expression of IPMN‐lesions in the literature. Current recommendations are based on studies where antibodies against the core MUC1 protein or sialylated MUC1 (tumor associated MUC1), not the fully glycosylated MUC1 were used. We have recently reported that MUC1 is strongly expressed in both gastric and intestinal types IPMN specimens from the cystic wall, obtained by endoscopic ultrasound guided microbiopsy procedure. We have used a commercial MUC1 antibody, validated and recommended for diagnostic use, which recognizes fully glycosylated MUC1. Based on the above, we propose a revision of the WHO Classification, specifying that antibodies against tumor associated MUC1 should be used for IPMN subtyping.