胰腺导管内乳头状黏液性肿瘤27例诊治分析

目的总结胰腺导管内乳头状黏液性肿瘤(IPMN)的诊治经验,寻找术前预测浸润性IPMN的指标.方法将2003年9月-2010年7月手术治疗的27例胰腺IPMN病例分为浸润性与非浸润性IPMN两组,分析比较两组间术前资料的差异.结果浸润性IPMN 15例,非浸润性IPMN12例.两组间术前梗阻性黄疸发病率、肿瘤最大直径、血...     

Intraductal papillary mucinous neoplasm in chronic calcifying pancreatitis： Egg or hen？

Intraductal papillary mucinous neoplasm (IPMN) is an increasingly reported entity. Extensive pancreatic calcification is generally thought to be a sign of chronic pancreatitis, but it may occur simultaneously with IPMN leading to diagnostic difficulties. We report a case of a patient initially diagnosed with chronic calcifying pancreatitis who was later shown to have a malignant IPMN. This case illustrates potential pitfalls in the diagnosis of IPMN in the case of extensive pancreatic calcification as well as clues that may lead the clinician to suspecting the diagnosis. The possible mechanisms of the relation between pancreatic calcification and IPMN are also reviewed.     

Recent advances in the diagnostic evaluation of pancreatic cystic lesions

Pancreatic cystic lesions (PCLs) are becoming more prevalent due to more frequent abdominal imaging and the increasing age of the general population. It has become crucial to identify these PCLs and subsequently risk stratify them to guide management. Given the high morbidity associated with pancreatic surgery, only those PCLs at high risk for malignancy should undergo such treatment. However, current diagnostic testing is suboptimal at accurately diagnosing and risk stratifying PCLs. Therefore, research has focused on developing new techniques for differentiating mucinous from non-mucinous PCLs and identifying high risk lesions for malignancy. Cross sectional imaging radiomics can potentially improve the predictive accuracy of primary risk stratification of PCLs at the time of detection to guide invasive testing. While cyst fluid glucose has reemerged as a potential biomarker, cyst fluid molecular markers have improved accuracy for identifying specific types of PCLs. Endoscopic ultrasound guided approaches such as confocal laser endomicroscopy and through the needle microforceps biopsy have shown a good correlation with histopathological findings and are evolving techniques for identifying and risk stratifying PCLs. While most of these recent diagnostics are only practiced at selective tertiary care centers, they hold a promise that management of PCLs will only get better in the future.     

Value of Near-Isovoxel Ultrasound for Evaluation of Ductal Communications with Pancreatic Cystic Lesions: Correlation with Magnetic Resonance Cholangiopancreatography

The aim of this study was to determine the value of near-isovoxel ultrasound (ISUS) using xMATRIX technology in assessment of ductal communications with pancreatic cystic lesions. Twenty patients with pancreatic cystic lesions (n = 21) on magnetic resonance cholangiopancreatography (MRCP), underwent 2-D ultrasound (US) and subsequent ISUS using a matrix probe. Two observers assessed the presence of ductal communications with pancreatic cystic lesions for all MRCP, 2-D US, and ISUS images with multi-planar reformation, using a 5-point confidence scale. Weighted-κ statistics and intra-class correlation coefficients were calculated. Inter-observer agreement for MRCP, 2-D US and ISUS was moderate, fair and moderate (0.475, 0.222 and 0.472), respectively. The intra-class correlation coefficients between ISUS and MRCP was higher than that between 2-D US and MRCP (0.8706 vs. 0.5353, observer 1; 0.7206 vs. 0.4818, observer 2, respectively). Correlation and inter-observer agreement were better with MRCP than with 2-D US. We conclude that ISUS may be useful in evaluating ductal communications with pancreatic cystic lesions.     

Free Communications

Ovarian-type mucinous tumours occur very rarely in the retroperitoneum. We present a case of primary retroperitoneal mucinous tumour of borderline malignancy in a 58-year-old woman, detected as an incidental finding. The patient presented with acute renal failure, investigation for which revealed a complex pelvic mass initially thought to be in the right adnexa and consistent with an ovarian neoplasm. Surgical findings revealed a 130-mm, right-sided non-communicating retroperitoneal pelvic mass, posterior to the appendix, which was completely resected. Both ovaries were normal. Macroscopically, it was a multi-loculated cystic structure with a smooth external surface containing clear and mucinous fluid. Microscopic examination showed a mucinous tumour of borderline malignancy. The literature contains approximately nine other cases of primary mucinous retroperitoneal tumour of borderline malignancy. These cases have occurred in women aged 36–60 years. Most patients were asymptomatic and the mass was detected as an incidental finding. The patients have been followed up for up to 6 to 18 months and, to date, none have recurred. There are limitations to pre-operative radiological imaging. A definitive diagnosis can only be made after complete surgical excision and histological examination, having excluded retroperitoneal involvement by mucinous tumours from sites such the ovaries, bowel, appendix and pancreas.     

Hereditary progressive mucinous histiocytosis: 3 different phenotypes in 3 family members

We describe 3 cases of multiple histiocytic cutaneous tumors that began in childhood and affected 3 members from 2 generations of the same family: a mother, a daughter and a nephew. The lesions were mostly skin‐colored papules distributed symmetrically on the dorsum of the forearms and hands and on the face and thighs. There were no signs of spontaneous regression. The clinical and histopathological features were consistent with a diagnosis of hereditary progressive mucinous histiocytosis (HPMH), but phenotypic expression varied somewhat between the 3 patients. HPMH has only been described in 8 families to date, and just one of the reports included 3 well‐documented cases. Our cases confirm that HPMH can affect males and expands the clinical spectrum of skin lesions in this disease.     

Guanylyl cyclase C is a specific marker for differentiating primary and metastatic ovarian mucinous neoplasms

Aims:  The aim was to assess the value of GCC in distinguishing primary ovarian mucinous neoplasms from metastatic mucinous adenocarcinomas with ovarian involvement. Guanylyl cyclase C (GCC) is a brush border membrane receptor for the endogenous peptides guanylin and uroguanylin, and the homologous diarrhoeagenic bacterial heat-stable enterotoxins that is selectively expressed by epithelial cells from the duodenum to the rectum, but not by normal epithelia of the stomach or oesophagus, or normal extramucosal cells in humans.
Methods and results:  Fifty ovarian tumours: 27 primary ovarian mucinous neoplasms (seven cystadenomas, 10 borderline tumours and 10 cystadenocarcinomas) and 23 metastatic mucinous adenocarcinomas with ovarian involvement [13 colorectal adenocarcinomas, four gastric adenocarcinomas, six appendiceal mucinous tumours (four adenocarcinomas, one with neuroendocrine features, and two appendiceal mucinous cystadenomas)] were studied. For primary ovarian mucinous neoplasms, 25 of 27 were negative for GCC. Twelve of 13 cases of colorectal adenocarcinoma (except for one neuroendocrine adenocarcinoma) were positive for GCC. Three of four appendiceal mucinous adenocarcinomas were positive for GCC in both the primary and metastatic tumours (except for one neuroendocrine adenocarcinoma). Two of two appendiceal mucinous cystadenomas were positive for GCC. Of four cases of gastric adenocarcinoma with ovarian involvement, only one (primary tumour) exhibited focal GCC staining.
Conclusions:  GCC is a useful marker for differentiating between primary and secondary ovarian mucinous neoplasms.     

ENDOSCOPIC DIAGNOSIS OF INTRADUCTAL PAPILLARY‐MUCINOUS NEOPLASM OF THE PANCREAS BY MEANS OF PERORAL PANCREATOSCOPY USING A SMALL‐DIAMETER VIDEOSCOPE AND NARROW‐BAND IMAGING

Background: Intraductal papillary‐mucinous neoplasm (IPMN) is an intraductal tumor in which the mucin‐producing epithelium shows proliferated papillary and a wide variety of pathological changes ranging from hyperplasia to adenocarcinoma. Therefore, it is important to determine whether an IPMN is benign or malignant. In the present study of patients with IPMN, the protrusion was observed by a peroral pancreatoscopy (PPS) using a small‐diameter videoscope and narrow‐band imaging (NBI). We carried out the differential diagnosis of benign lesion to malignant lesion. Methods: Between April 2003 and May 2009, PPS using a small‐diameter videoscope by means of NBI was carried out on 21 hospitalized patients with IPMN (10 cases of adenocarcinoma, 11 cases of adenoma or hyperplasia; 14 males and seven females, with a mean age of 69.4 years). Results: Fifteen focal lesions of the 16 cases in the head of the pancreas (93.7%) and four focal lesions of the five cases in the pancreatic body (80%) were observable, whereas two lesions (adenocarcinoma in the pancreatic body, and adenoma in the uncus of pancreas) were not observable. Endoscopically, seven cases were classified as villous type and two cases as vegetative type, and nine cases were diagnosed as adenocarcinoma. Ten cases with sessile type or semipedunculated type were diagnosed as adenoma or hyperplasia. Vascular patterns and protrusions were detected more clearly in the NBI images than under white light observation. Conclusions: When combined with a videoscope and NBI, pancreatoscopy provided a clear image and was useful for evaluating whether the IPMN was benign or malignant.     

乳腺单纯性黏液腺癌的临床病理分析

目的研究乳腺单纯黏液腺癌的临床病理特征,比较它和浸润性导管癌的区别,并分析病理特征和预后的关系。方法回顾性分析了23例可手术乳腺单纯性黏液腺癌患者和同期863例对照的浸润性导管癌的临床病理资料。结果单纯性导管癌占全部同期乳腺癌的2.6%以下,患者中位年龄51岁(23岁~86岁),肿块中位大小2.5 cm;单纯性黏液腺癌患者中位年龄62岁(36~83岁),肿块中位大小3.0 cm。浸润性导管癌和单纯性黏液腺癌之间的患者年龄、肿块大小、腋淋巴结状态转移数、ER状态和HER-2状态存在显著的差异,后者的临床病理特征明显优于前者。而且ER PR 组和ER PR-组在单纯性黏液腺癌中的比例显著提高。中位随访17.6月(1~24个月)内,无一例单纯性黏液腺癌发生复发转移。对照的863例浸润性导管癌患者中,2例死亡,6例复发转移。结论单纯性黏液腺癌临床病理学特征显著优于浸润性导管癌,提示了其预后的优势,而且ER阳性比例高,年龄大,内分泌治疗可能更加行之有效。     

Mucinous adenocarcinoma of the tail of the pancreas presenting with a choroidal metastasis

AIM: To report a case of mucinous adenocarcinoma of the tail of the pancreas presenting with a solitary choroidal metastasis. ·METHODS: A 57 years old female patient presented with central metamorphosia in the right eye. Fundoscopy showed a solitary pale raised lesion in the central right macula about 6 disc diameters in length. Her past ocular and medical history was unremarkable. A B-scan confirmed a raised solid lesion in the posterior pole within the macula while fluorescein angiography revealed a central lesion with no areas of leakage. ·RESULTS: In view of the history and the clinical findings a FBC, ESR, LFT, Chest X-ray, and abdominal CT were requested .The chest X-ray revealed multiple opaque lesions in both lung fields suggestive of metastatic pulmonary nodules. The CT revealed multiple nodules in the liver and a 3.5cm x 2.6cmlesion in the tail of the pancreas. A CT guided liver biopsy was performed and it revealed moderately differentiated metastatic mucinous adenocarcinoma. Subsequent blood analysis revealed an elevated CA19-9. The primary tumour site was identified as the tail of the pancreas and was decided to instigate palliative treatment. ·CONCLUSION: There are few reports that demonstrate the significance of a solitary choroidal lesion as the initial clinical sign of cancer of the tail of the pancreas. This case highlights the importance of performing detailed abdominal imaging studies in cases where a solid choroidal lesion of unknown origin is identified.