Dilated papilla with mucin extrusion is a potential predictor of acute pancreatitis associated with intraductal papillary mucinous neoplasms of pancreas

Background/objectivesAs intraductal papillary mucinous neoplasm (IPMN) of the pancreas is associated with acute pancreatitis (AP) in some cases, predicting the risk of pancreatitis is as important as predicting the risk of malignancy in IPMN cases. In this study, we attempted to clarify the characteristics of IPMN associated with AP, compared to those of IPMN not associated with AP.MethodsFrom January 2006 to March 2013, data from 88 patients who underwent surgery for IPMN were retrospectively investigated and analyzed. We evaluated clinical and pathological variables of each patient and compared patients with IPMN with AP to those without AP. Furthermore, we presented representative cases of mild and severe pancreatitis caused by IPMN.ResultsOverall, 12 of 88 patients with IPMN (13.6%) had AP. Seven of the 12 patients had a single episode of AP, whereas the remaining 5 patients were diagnosed with IPMN with repeated AP. Ten of 12 patients with AP were diagnosed with mild AP and the remaining 2 with severe AP. Regarding clinical findings, the proportion of dilated papilla with mucin extrusion was significantly higher in patients with IPMN with AP than in those without AP (p = 0.035). Histological findings indicated that the proportion of intestinal-subtype IPMN was significantly higher in patients with IPMN with AP (p = 0.013).ConclusionsAP caused by IPMN derives mostly from intestinal IPMN. Dilated papilla with mucin extrusion can be a potential predictor of AP.     

aPKCλ/ι is a beneficial prognostic marker for pancreatic neoplasms

Pancreatic cancer is a lethal disease. Overall survival is typically 6 months from diagnosis. Determination of prognostic factors in pancreatic cancer that would allow identification of patients who could potentially benefit from aggressive treatment is important. However, until date, there are no established reliable prognostic factors for pancreatic cancer patients. Herein, we propose a beneficial biomarker which is significantly correlated with the prognosis in pancreatic cancer patients. Atypical protein kinase C λ/ι (aPKCλ/ι) is overexpressed and has been implicated in the progression of several cancers. We tested the expression levels of aPKCλ/ι in two types of pancreatic neoplasm, pancreatic ductal adenocarcinoma (PDAC) and intraductal papillary mucinous neoplasms (IPMNs), by immunohistochemistry. Examination of the aPKCλ/ι expression levels in surgically resected specimens of PDCA (n = 115) demonstrated that the expression levels of aPKCλ/ιin PDAC had prognostic implications, independent of the Tumor-Node-Metastasis classification and World Health Organization tumor grade. In the case of IPMNs (n = 46) also, the expression levels of aPKCλ/ιin IPMN were found to be of prognostic importance, independent of the World Health Organization histological grade or morphological type. Interestingly, high expression levels of aPKCλ/ι were significantly correlated with a worse histological grade (p = 0.010) and advanced stage of the tumor (p = 0.0050) in IPMN patients. These findings suggest that high expression levels of aPKCλ/ι could be involved in the malignant transformation of IPMNs. Based on these observations, we propose the expression level of aPKCλ/ι as a prognostic marker common to different types of pancreatic neoplasms.     

胰腺囊性肿瘤的诊断与治疗

目的探讨胰腺囊性肿瘤的诊断和治疗。方法回顾性分析2008年1月-2012年7月间56例本院胰腺囊性肿瘤患者的临床资料,男性15例,女性41例,男女比例为1：2.73,其中浆液性囊腺瘤12例,黏液性囊腺瘤22例,黏液性囊腺瘤癌6例,导管内乳头状囊腺瘤3例和乳头状囊腺瘤癌1例,实性假乳头状瘤12例。结果所有患者均经手术治疗,胰十二指肠切除术13例,胰腺肿瘤节段切除术4例,1例因肿瘤侵及周围脏器及大血管行胃空肠及胆肠吻合术,1例行胰腺钩突部肿瘤切除、部分十二指肠空肠切除、十二指肠空肠吻合术,胰腺肿瘤局部切除术3例,胰体尾切除术18例,保留脾脏的腹腔镜胰体尾切除术8例,腹腔镜胰体尾加脾切除2例,腹腔镜胰腺肿瘤局部切除术6例。本组无围手术期死亡,腹腔感染2例,胃排空障碍3例,胰瘘4例,胆漏2例,均经保守治疗痊愈。结论 B超、CT和MRI是胰腺囊性肿瘤的主要诊断方法,具体手术方式应根据肿瘤所在部位、病理类型、与主胰管的关系以及患者全身情况综合考虑。     

Natural language processing for the development of a clinical registry: a validation study in intraductal papillary mucinous neoplasms

Background:

Medical natural language processing (NLP) systems have been developed to identify, extract and encode information within clinical narrative text. However, the role of NLP in clinical research and patient care remains limited. Pancreatic cysts are common. Some pancreatic cysts, such as intraductal papillary mucinous neoplasms (IPMNs), have malignant potential and require extended periods of surveillance. We seek to develop a novel NLP system that could be applied in our clinical network to develop a functional registry of IPMN patients.

Objectives:

This study aims to validate the accuracy of our novel NLP system in the identification of surgical patients with pathologically confirmed IPMN in comparison with our pre-existing manually created surgical database (standard reference).

Methods:

The Regenstrief EXtraction Tool (REX) was used to extract pancreatic cyst patient data from medical text files from Indiana University Health. The system was assessed periodically by direct sampling and review of medical records. Results were compared with the standard reference.

Results:

Natural language processing detected 5694 unique patients with pancreas cysts, in 215 of whom surgical pathology had confirmed IPMN. The NLP software identified all but seven patients present in the surgical database and identified an additional 37 IPMN patients not previously included in the surgical database. Using the standard reference, the sensitivity of the NLP program was 97.5% (95% confidence interval [CI] 94.8–98.9%) and its positive predictive value was 95.5% (95% CI 92.3–97.5%).

Conclusions:

Natural language processing is a reliable and accurate method for identifying selected patient cohorts and may facilitate the identification and follow-up of patients with IPMN.     

胰管内乳头状黏液性肿瘤的术前良、恶性与浸润性预测因子

目的 分析术前预测胰管内乳头状黏液性肿瘤(IPMN)良、恶性与浸润性的因子,及不同病理类型IPMN的手术后生存率.方法 回顾性分析长海医院1993年1月至2009年9月间手术切除的78例IPMN病例资料,采用单因素与多因素分析的方法分析病史、临床表现、肝功能、CEA、CA19-9、影像学征象等在术前判断IPMN良、恶性与浸润性的价值,分析患者术后生存率.结果 单因素分析结果显示,黄疸、急性胰腺炎、血CA19-9>37 U/ml、AKP、肿块边界不清为恶性及浸润性预测因子;主胰管扩张、分支胰管直径>30 mm、出现壁节结等为恶性预测因子;CEA>6 ng/ml为浸润性预测因子.多因素分析显示,肿块边界不清为恶性及浸润性预测因子;急性胰腺炎为浸润性预测因子.良性IPMN患者的5年生存率为100%;恶性IPMN的2年生存率为78.9%、5年生存率为68.5%,其中浸润性IPMN的2年生存率为64.6%、5年生存率为43.1%.结论 肿块边界不清为IPMN恶性预测因子,急性胰腺炎、肿块边界不清是IPMN的浸润性预测因子.     

Parenchyma-sparing resections for pancreatic neoplasms

Background/purpose

In recent years there has been an increase in the indications for pancreatic resection of benign or low-grade malignant lesions, especially in young patients with long life expectancy. In this setting, patients may benefit from parenchyma-sparing resections in order to decrease the risk of development of exocrine/endocrine insufficiency.

Methods

A review of the literature and authors experience was undertaken.

Results

Parenchyma-sparing resections of the pancreas including enucleation, middle pancreatectomy (MP) and middle-preserving pancreatectomy are described. Short and long-term outcomes after surgery are analyzed with special regard to postoperative morbidity/mortality, and oncological and functional long-term results.

Conclusions

Parenchyma-sparing resections are safe and effective procedures for treatment of benign and low-grade malignant neoplasms. Despite a significant postoperative morbidity they are associated with good long-term functional and oncological results. Enucleation should preferentially be performed laparoscopically whenever possible.     

Pancreatic head resection with segmental duodenectomy for pancreatic neoplasms

Background/purpose

We have experienced 67 cases of pancreatic head resection with segmental duodenectomy (PHRSD) for benign or low-grade malignant tumor of the pancreatic head region. Here we introduce our operative technique for these 67 cases.

Methods

Pancreatic head resection is performed with segmental duodenectomy including minor and major papilla. By conserving the right gastric artery and the gastroduodenal artery, 5–7 cm of the first portion of the duodenum is preserved with good arterial circulation. In addition, by conserving the anterior inferior pancreatoduodenal artery, the third portion and anal side or the second portion of the duodenum are preserved with good arterial circulation. Cholecystectomy is performed. The procedure is completed by resection of the pancreatic head with 3–4 cm of segmental duodenectomy including minor and major papilla. Reconstruction of the alimentary tract is performed with pancreatogastrostomy, end-to-end duodenoduodenostomy and end-to-side choledochoduodenostomy.

Results

In 67 cases with diseases of the pancreatic head region, chiefly intraductal papillary mucinous neoplasms, this procedure was successfully performed without operative or hospital death. Postoperative quality of life was quite satisfactory.

Conclusion

Total resection of the pancreatic head can be performed safely and effectively by this procedure.     

Endoscopic diagnosis of intraductal papillary mucinous neoplasm of the bile duct

Background/Purpose

Intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) is considered an uncommon tumor. The purpose of this study was to evaluate the diagnostic utility of endoscopic cholangiography (ERC) with subsequent peroral cholangioscopy (POCS) and/or intraductal ultrasonography (IDUS) for this tumor.

Methods

From December 1991 to November 2006, a retrospective analysis was made of eight patients with IPMN-B. Their clinical features and the endoscopic diagnostic strategy for POCS and IDUS were reviewed.

Results

In all the patients, ERC failed to show papillary tumors, due to coexisting mucin or biliary sludge. POCS was carried out after ERC and it showed the presence and locations of papillary tumors in all patients, except for one with a tumor in the peripheral intrahepatic bile duct (B3). IDUS was performed in seven of the eight patients; in five of these patients, intraductal protruding tumors were clearly visualized, whereas flat tumors were not identified in the remaining two patients. In one of the eight patients, endoscopic nasobiliary drainage did not remove the huge amount of mucin. Hence, this patient required subsequent percutaneous biliary drainage. Six of the eight patients underwent surgical treatment; five patients underwent a hepatic resection with or without extrahepatic bile duct resection and one underwent a pancreaticoduodenectomy. Five of the six operated patients are still alive; one patient died of gastric cancer 90 months after the operation (mean follow-up period, 45.3 months). The two remaining patients, who were considered inoperable due to major medical comorbidities, died of liver failure and cholangitis 3 and 6 months, respectively, after stent placement.

Conclusion

ERC failed to delineate intraductal papillary tumors, due to coexisting mucin. The presence and location of papillary tumors were correctly diagnosed by both POCS and IDUS, but POCS may be better than IDUS to diagnose the extent of the tumor.     

Reappraisal of synchronous and multifocal mucinous lesions of the female genital tract: a close association with gastric metaplasia

Aims:  To describe the gastric phenotype of synchronous mucinous metaplasia and neoplasms of the female genital tract (SMMN–FGT).
Methods and results:  Six patients (mean age 57 years, range 39–83 years) were diagnosed with SMMN–FGT. All six patients had mucinous metaplasia of endometrium, which showed features of lobular endocervical glandular hyperplasia (LEGH)/pyloric gland metaplasia (PGM) in five and was associated with mucinous adenocarcinoma in three. Five patients had mucinous metaplasia of the fallopian tubes, of which three showed borderline features. Two patients had mucinous borderline tumour of the ovary. Five patients had cervical lesions including LEGH/PGM associated with either adenocarcinoma in situ or minimal deviation adenocarcinoma of the cervix. All mucinous lesions were positive for HIK1083 and/or MUC6. One patient with minimal deviation adenocarcinoma involving the vagina died of her disease, whereas five patients, including three with microinvasion and three with positive peritoneal cytology or mucinous ascites, were alive without recurrence after a mean follow-up of 46 months (range 13–102 months).
Conclusions:  There is a close association between SMMN–FGT and LEGH/PGM. Microinvasion and positive peritoneal cytology may not have an influence on outcome.     

Mucinous breast carcinoma with myoepithelial‐like spindle cells

Appearance of spindle cells has been believed as a benign index of breast cytology. But, we have frequently observed the spindle cells in smears from mucinous carcinoma of the breast. Here, we characterized the biochemical nature of the spindle cells, so as to clarify their identity in cytology. Nineteen cases of breast mucinous carcinoma were used for cytological examination. The spindle cells were located at edges of tumor cell nests and in the backgrounds of cytological specimens. Immunohistological examination revealed that the spindle cells exhibited both immunoreactivity against carcinoembryonic antigen (CEA) and epithelial membrane antigen (EMA). Immunoreactivity against vimentin, cytokeratin, or alpha‐smooth muscle actin was, however, not observed. The mode of distribution of biochemical markers suggests that the positive cells for anti‐CEA antibody and anti‐EMA antibody are tumor cells compressed by mucin, while the vimentin‐positive cells are fibroblasts. We assert that the presence of spindle cells can be a characteristic feature of mucinous carcinoma of the breast. Discrimination of the spindle cells in mucinous carcinoma from myoepithelial cells and naked bipolar nuclei in benign lesions was established here. It should facilitate precise diagnosis of breast cancer. Diagn. Cytopathol. 2009. © 2009 Wiley‐Liss, Inc.