Chronic asymptomatic pseudocyst with sludge aggregates masquerading as mucinous cystic neoplasm of the pancreas

Pseudocyst of the pancreas is sometimes difficult to distinguish from mucinous cystic neoplasm of the pancreas. A 37-year-old asymptomatic Japanese man was diagnosed with hypertension. He had a 20-years history of habitual drinking of alcohol, but no history of pancreatitis or abdominal trauma. During examinations to ascertain the cause of hypertension, ultrasonography and computed tomography incidentally demonstrated a huge cyst in the head of the pancreas. Laboratory data were within normal limits, including serum levels of amylase, carcinoembryonic antigen, and carbohydrate antigen 19-9. Imaging studies showed a huge unilocular cyst, measuring 7 cm, in the head-to-body of the pancreas, and two small unilocular cysts, measuring 1.4 and 1.5 cm, in the tail and head of the pancreas, respectively. A mural nodule was suspected in the largest cyst. Endoscopic retrograde cholangiopancreatography demonstrated communication of the main pancreatic duct with the two small cysts in the head and tail of the pancreas but not with the huge cyst. There were no ductal changes suggesting chronic pancreatitis. Laparotomy was performed under the tentative diagnosis of potentially malignant mucinous cystic neoplasms of the pancreas. However, inflammatory adhesion was dense around the pancreas and the mural nodule suspected preoperatively was found to be sludge aggregates in a pseudocyst. The diagnosis of an intraoperative frozen section of the cyst wall was pseudocyst of the pancreas. Cystojejunostomy was performed. We report this case because the pre-operative diagnosis was mucinous cystic neoplasm of the pancreas, but the diagnosis changed with careful intraoperative examinations, to pseudocyst of the pancreas. We discuss the differential diagnosis of the two conditions. (Received Oct. 1, 1997; accepted Jan. 23, 1998)     

The "Duct-Ectatic" Variant of Mucinous Cystic Neoplasm of the Pancreas: Clinical and Radiologic Studies of Seven Cases

Seven cases of the "duct-ectatic" variant of mucinous cystic neoplasm of the pancreas are presented. This new entity, the duct-ectatic variant of mucinous cystic neoplasm of the pancreas, probably masquerades as pancreatic pseudocyst clinically and radiologically. However, it has the characteristic pathologic findings of the classic "megacystic" type of mucinous cystic neoplasm. If there are cysts visualized on pancreaticogram which are in a diffusely duct-ectatic configuration and are in communication with the main pancreatic duct, the duct-ectatic variant of mucinous cystic neoplasm should be strongly suspected rather than the pancreatic pseudocyst.     

The Pathogenesis of Pancreatic Pseudocysts—A Canine Experimental Model

In summary, an experimental model that mimics the pseudocyst formation in man was produced in dogs. The following conditions were met: encapsulated cyst containing pancreatic juice; an actively secreting pancreas with hyperamylasemia and pancreatitis; a ductal communication; and more pronounced effects with partial pancreatic ductal obstruction.     

Mucinous Cystadenoma: Pitfalls of Differential Diagnosis

Cystic neoplasms of the pancreas often are difficult to differentiate from pseudocysts. It has been proposed that a history of clinical pancreatitis, elevated serum pancreatic enzymes, elevated cyst fluid amylase, and a communication with the pancreatic duct suggest the diagnosis of a pseudocyst. We report the case of a young woman who presented with a cystic mass in the pancreas and was thought to have a pseudocyst because of the above; at surgery, a mucinous cystadenoma was documented. The pitfalls of differentiating neoplastic cysts of the pancreas from pseudocysts are discussed.     

Endoscopic therapy for chronic pancreatitis: An evidence-based review

In the setting of chronic pancreatitis, pancreatic ductal obstruction, and ductal leak, pseudocyst formation and biliary obstruction present many challenges for endoscopists. Although chronic pancreatitis has a variety of clinical manifestations, most commonly patients present with intermittent or chronic abdominal pain. Recent studies suggest stenting of pancreatic ductal strictures has a significant impact on reducing chronic pain. The removal of ductal calculi, presumably from relieving obstruction, also improves abdominal pain. When the site of leak is bypassed, ductal leaks may be cured by endoscopic stenting. Multiple plastic bile duct stents to treat chronic pancreatitis-associated bile duct stricture can delay the need for surgery. Although these endoscopic techniques have been beneficial for many patients, further study is warranted to better define their role in chronic pancreatitis compared with well-established surgical techniques.     

Lymphoepithelial cyst of the pancreas. No evidence for Epstein-Barr virus-related pathogenesis.

Compared to pseudocyst formation after prior pancreatitis, true cysts of the pancreas are rare. Pancreatic cysts with irregular wall components or a mucinous content raise the suspicion for the presence of a cystic neoplasm, and surgical resection is recommended. A case of a patient with a history of prostate cancer is described in whom a cyst of the pancreatic tail was discovered incidentally. Based on the radiographic features, which did not support the presence of a serous cystadenoma, a spleen-preserving distal pancreatectomy was performed. Histologic features were characteristic for a lymphoepithelial cyst (LEC) of the pancreas, lined with thinned squamous epithelium surrounded by benign lymphoid tissue. Since LECs of the parotid gland, which are associated with acquired human immunodeficiency, are frequently related to Epstein-Barr virus (EBV) infection, EBV in situ hybridization was performed and did not reveal evidence for EBV. Twenty-eight instances of pancreatic LECs have been reported, primarily affecting adult males, without evidence of increased numbers of EBV-positive cells. The pathogenesis, differential diagnosis, and clinical implications of lymphoepithelial pancreatic cysts are discussed.     

Multiple 'cysts' in the entire pancreas in a case of cystic adenocarcinoma of the pancreas

Abstract A case of multiple 'cysts' coexisting with cystic adenocarcinoma in the pancreas is presented. The cysts were lined by epithelium consisting of either cuboidal and flat cells or mucinous cells, which formed a papillary pattern; the former cells were more frequent and the latter were considered to be hyperplasia. The pathological findings for the diffuse multiple cysts suggested cystic disease caused by ductal stenosis, while the mucinous cells may have been related to the malignant tumour.     

Accuracy and agreement of a large panel of endosonographers for endomicroscopy-guided virtual biopsy of pancreatic cystic lesions

BackgroundAlthough emerging data evidences that EUS-guided needle-based confocal laser endomicroscopy (nCLE) accurately diagnoses pancreatic cystic lesions (PCLs), there are a lack of interobserver agreement (IOA) studies utilizing reference histopathological diagnosis and for specific PCL subtypes. Hence, we sought to assess the IOA, intra-observer reliability (IOR), and diagnostic performance of EUS-nCLE using a large cohort of patients with histopathological diagnosis amongst a broad panel of international observers.MethodsEUS-nCLE videos (n = 76) of subjects with PCLs [intraductal papillary mucinous neoplasm (IPMN), mucinous cystic neoplasm (MCN), serous cystadenoma (SCA), pseudocyst, and cystic-neuroendocrine tumors/solid pseudopapillary neoplasm (cystic-NET/SPN)], simulating clinical prevalence rates were obtained from 3 prospective studies. An international panel of 13 endosonographers with nCLE experience, blinded to all PCL data, evaluated the video library twice with a two-week washout for PCL differentiation (mucinous vs. non-mucinous) and subtype diagnosis.ResultsThe IOA (κ = 0.82, 95% CI 0.77–0.87) and IOR (κ = 0.82, 95% CI 0.78–0.85) were “almost perfect” to differentiate mucinous vs. non-mucinous PCLs. For PCL subtype, IOA was highest for SCA (almost perfect; κ = 0.85), followed by IPMN (substantial, κ = 0.72), and cystic-NET/SPN (substantial, κ = 0.73). The IOA was moderate for MCN (κ = 0.47), and pseudocyst (κ = 0.57). Compared to histopathology, observers differentiated mucinous vs. non-mucinous PCLs with high accuracy (94.8%, 95% CI 93.3–96.1). For detecting specific PCLs subtypes, EUS-nCLE was highly accurate in diagnosing non-mucinous cysts (SCA: 98%; cystic-NET/SPN: 96%; pseudocyst: 96%) and slightly less accurate for mucinous lesions (IPMN: 86%; MCN: 84%).ConclusionDiagnosis of PCLs by EUS-nCLE guided virtual biopsy is very accurate and reliable for the most prevalent pancreatic cysts in clinical practice.     

Lymphoepithelial cyst of the pancreas

Summary  Compared to pseudocyst formation after prior pancreatitis, true cysts of the pancreas are rare. Pancreatic cysts with irregular wall components or a mucinous content raise the suspicion for the presence of a cystic neoplasm, and surgical resection is recommended. A case of a patient with a history of prostate cancer is described in whom a cyst of the pancreatic tail was discovered incidentally. Based on the radiographic features, which did not support the presence of a serous cystadenoma, a spleen-preserving distal pancreatectomy was performed. Histologic features were characteristic for a lymphoepithelial cyst (LEC) of the pancreas, lined with thinned squamous epithelium surrounded by benign lymphoid tissue. Since LECs of the parotid gland, which are associated with acquired human immunodeficiency, are frequently related to Epstein-Barr virus (EBV) infection, EBVin situ hybridization was performed and did not reveal evidence for EBV. Twenty-eight instances of pancreatic LECs have been reported, primarily affecting adult males, without evidence of increased numbers of EBV-positive cells. The pathogenesis, differential diagnosis, and clinical implications of lymphoepithelial pancreatic cysts are discussed.     

Avoiding an overdiagnosis of pancreatic pseudocysts

BACKGROUND/AIMS: Thirty-six cases of pancreatic pseudocysts were retrospectively analyzed, to evaluate the clinical features of the pseudocysts which could not be differentiated from the neoplastic cysts until laparotomy. METHODOLOGY: Thirty-one out of 36 cases were diagnosed correctly to be a pseudocyst, in which 10 cases (32.3%) were treated by surgery. Five out of 36 cases were diagnosed to be neoplastic pancreatic cysts (mucinous cystadenoma or cystadenocarcinoma in 4 cases, serous cystadenoma in 1) in which all cases were treated by surgery (100%). To determine the clinical factors contributing to a correct or false diagnosis of pseudocysts, 14 clinical objects were categorized into several factors and analyzed using a contingency table. RESULTS: The clinical factors, including a "history of pancreatitis" (P = 0.070), "upper abdominal pain" (P = 0.083), an "age of less than 42 years" (P = 0.070), and an "elevated serum amylase level on admission" (> or = 200 IU/L, P = 0.067) were all thought to be helpful in establishing a correct diagnosis of pancreatic pseudocyst. In the morphological studies of computed tomography and ultrasonography, "multicystic lesions" (P = 0.045) and "nodular or irregular thickening of the cyst wall" (P = 0.006) significantly mislead us into making a diagnosis of a neoplastic cyst. CONCLUSIONS: In conclusion, the morphological features of a multicystic pattern, with either nodular or irregular thickening of the cyst wall, also belong to the common features of the pancreatic pseudocysts. Since these features tended to be diagnosed as neoplastic, other clinical factors should thus be referred to, in a comprehensive manner, to establish a correct diagnosis of pancreatic pseudocyst.     

Pancreatic Cysts and Guidelines

Pancreatic cysts, especially incidental asymptomatic ones seen on noninvasive imaging such as CT or MR imaging, remain a clinical challenge. The etiology of such cysts may range from benign cysts without any malignant potential such as pancreatic pseudocysts and serous cystadenomas to premalignant or frankly malignant cysts such as mucinous cystic neoplasms, intraductal papillary mucinous neoplasms, cystic degeneration associated with solid tumors such as pancreatic ductal adenocarcinoma or pancreatic endocrine neoplasms, and solid pseudopapillary neoplasms. The clinical challenge in 2017 is to accurately preoperatively diagnose them and their malignant potential before deciding about surgery, surveillance or doing nothing. This review will focus on the currently available clinical guidelines for doing so.     

Mediastinal pancreatic pseudocyst caused by obstruction of the pancreatic duct was eliminated by bromhexine hydrochloride

A 49-year-old man, who had a 30-year history of drinking the equivalent of 80 g of ethanol per day, underwent a detailed medical examination for cough and dyspnea. Chest-abdominal computed tomography and endoscopic retrograde pancreatography led to the diagnosis of a mediastinal pancreatic pseudocyst resulting from obstruction of the pancreatic duct by a protein plug. The pseudocyst rapidly improved with conservative treatment with camostat mesilate, H2-receptor antagonist and digestive enzymes. Although the patient abstained from alcohol for approximately 6 months, he resumed drinking, leading to recurrent attacks of pancreatitis. Bromhexine hydrochloride was then administered for 6 months, with the expectation that it would have a mucolytic effect on the pancreatic juice, resulting in improvement in the clinical symptoms, pancreatic enzymes and pancreatic exocrine function, as well as elimination of the protein plug. Bromhexine hydrochloride may be a new therapy for pathological states, such as alcoholic chronic pancreatitis, in which there is increased viscosity of the pancreatic juice because of elevated protein concentration, leading to protein plug formation and temporary blockage of the pancreatic duct.     

Periampullary cyst: a surgically remediable cause of pancreatitis.

We report two patients with periampullary cysts associated with recurrent attacks of acute pancreatitis. In both patients the diagnosis was made preoperatively by upper gastrointestinal endoscopy and ERCP, which was also useful in determining the relationship of the cysts to the biliary and pancreatic ductal systems. Simple marsupialisation of the cysts resulted in long term relief of symptoms. Congenital cystic anomalies in the second part of the duodenum should be diligently sought in patients with pancreatitis of unexplained cause, as surgical therapy is safe and effective.     

A Case of Autoimmune Pancreatitis Manifested by a Pseudocyst and IgG4-Associated Cholangitis

Autoimmune pancreatitis (AIP) is a benign disorder and a unique form of chronic pancreatitis with several characteristic features. A cystic formation that mimics a pseudocyst is a rare finding. There have been a few reports of AIP complicated by pancreatic cysts. We present a case of AIP with multiple pseudocysts and obstructive jaundice caused by IgG4-associated cholangitis. We initially missed the diagnosis due to the pseudocyst. Based on the computed tomography images, laboratory findings and the therapeutic response to steroids, the case was diagnosed as AIP with pseudocysts and associated cholangiopathy.     

Pancreatic pseudocyst

Pancreatic pseudocysts are complications of acute or chronic pancreatitis. Initial diagnosis is accomplished most often by cross-sectional imaging. Endoscopic ultrasound with fine needle aspiration has become the preferred test to help distinguish pseudocyst from other cystic lesions of the pancreas. Most pseudocysts resolve spontaneously with supportive care. The size of the pseudocyst and the length of time the cyst has been present are poor predictors for the potential of pseudocyst resolution or complications, but in general, larger cysts are more likely to be symptomatic or cause complications. The main two indications for some type of invasive drainage procedure are persistent patient symptoms or the presence of complications （infection, gastric outlet or biliary obstruction, bleeding）. Three different strategies for pancreatic pseudocysts drainage are available： endoscopic （transpapillary or transmural） drainage, percutaneous catheter drainage, or open surgery. To date, no prospective controlled studies have compared directly these approaches. As a result, the management varies based on local expertise, but in general, endoscopic drainage is becoming the preferred approach because it is less invasive than surgery, avoids the need for external drain, and has a high long-term success rate. A tailored therapeutic approach taking into consideration patient preferences and involving multidisciplinary team of therapeutic endoscopist, interventional radiologist and pancreatic surgeon should be considered in all cases.     

Patient- and Cyst-Related Factors for Improved Prediction of Malignancy within Cystic Lesions of the Pancreas

Background and Aims: Early diagnosis of cancer in pancreatic cysts is important for timely referral to surgery. The aim of this study was to develop a predictive model for pancreatic cyst malignancy to improve patient selection for surgical resection. Methods: We performed retrospective analyses of endoscopic ultrasound (EUS) and pathology databases identifying pancreatic cysts with available final pathological diagnoses. Main-duct intraductal papillary mucinous neoplasms (IPMNs) were excluded due to the clear indication for surgery. Patient demographics and symptoms, cyst morphology, and cyst fluid characteristics were studied as candidate riskfactors for malignancy. Results: 270 patients with pancreatic cysts were identified and analyzed (41% men, mean age 61.8 years). Final pathological diagnoses were branch-duct IPMN (n = 118, 50% malignant), serous cystadenoma (n = 71), pseudocyst (n = 37), mucinous cyst adenoma/adenocarcinoma (n = 36), islet cell tumor (n = 4), simple cyst (n = 3), and ductal adenocarcinoma with cystic degeneration (n = 1). Optimal cut-off points for surgical resection were cyst fluid carcinoembryonic antigen (CEA) ≥3,594 ng/ml, age >50, and cyst size >1.5 cm. Cyst malignancy was independently associated with white race (OR = 4.1, p = 0.002), weight loss (OR = 3.9, p = 0.001), cyst size >1.5 cm (OR = 2.4, p = 0.012), and high CEA >3,594 (OR = 5.3, p = 0.04). In white patients >50 years old presenting with weight loss and cyst size >1.5 cm, the likelihood of malignancy was nearly sixfold greater than in those patients who had none of these factors (OR = 5.8,95% CI = 2.1-16.1, p = 0.004). Conclusions: Riskfactors other than cyst size are important for determination of malignancy in pancreatic cysts. Exceptionally high cyst fluid CEA levels and certain patient-related factors may help to better predict cyst malignancy and the need for surgical treatment.     

Cyst fluid glucose: An alternative to carcinoembryonic antigen for pancreatic mucinous cysts

Pancreatic cystic lesions(PCLs) have been increasingly recognized in clinical practice. Although inflammatory cysts(pseudocysts) are the most common PCLs detected by cross-sectional imaging modalities in symptomatic patients in a setting of acute or chronic pancreatitis, incidental pancreatic cysts with no symptoms or history of pancreatitis are usually neoplastic cysts. For these lesions,it is imperative to identify mucinous cysts(intraductal papillary mucinous neoplasms and mucinous cystic neoplasms) due to the risk of their progression to malignancy. However, no single imaging modality alone is sufficient for a definitive diagnosis of all PCLs. The cyst fluid obtained by endoscopic ultrasound-guided fine needle aspiration provides additional information for the differential diagnosis of PCLs. Current recommendations suggest sending cyst fluid for cytology evaluation and measurement of carcinoembryonic antigen(CEA) levels. Unfortunately, the sensitivity of cytology is greatly limited, and cyst fluid CEA has demonstrated insufficient accuracy as a predictor of mucinous cysts. More recently, cyst fluid glucose has emerged as an alternative to CEA for distinguishing between mucinous and nonmucinous lesions. Herein, the clinical utility of cyst fluid glucose and CEA for the differential diagnosis of PCLs was evaluated.     

磁共振扩散加权成像在胰腺囊性病变中的鉴别诊断价值

目的 评价磁共振扩散加权成像（DWI）在胰腺囊性病变中的鉴别诊断价值.方法 收集经临床和（或）病理证实的34例胰腺囊性病变,包括非肿瘤性病变的假性囊肿11例、单纯囊肿5例,肿瘤性病变的浆液性囊腺瘤6例、黏液性囊腺瘤10例、黏液性囊腺癌2例.34例均行常规MRI平扫及动态增强、DWI（b值0,600 s/mm2）扫描,记录病变DWI信号特点,测量病变及毗邻胰腺实质表观扩散系数（ADC）值,计算病变-胰腺实质ADC值比（ADCR）.采用受试者工作特征（ROC）曲线评价病灶ADC值及ADCR的诊断价值.结果 16例非肿瘤性囊性病变组中2例DWI上呈稍高信号,14例呈等信号,18例肿瘤性囊性病变组中17例呈高或稍高信号,l例呈等信号,两组差异具有统计学意义（P ＜0.001）.非肿瘤性、肿瘤性囊性病变的ADC值分别为（3.30 ±0.30）×10-3、（2.74±0.34）×10-3mm2/s;ADCR分别为1.85 ±0.20、1.31 ±0.21,差异均具有统计学意义（P值均＜0.001）.ADC值及ADCR诊断胰腺肿瘤性囊性病变的ROC曲线下面积分别为0.94±0.04、0.98±0.02.ADC值以3.105×10-3 mm2/s为阈值,诊断肿瘤性囊性病变的敏感性为81.3％,特异性为94.4％,ADCR值以1.525为阈值,诊断肿瘤性囊性病变的敏感性为100％,特异性为88.9％.结论 DWI在胰腺囊性病变的诊断与鉴别诊断中具有重要价值,可作为常规MRI的重要补充.     

A case of mixed adenoneuroendocrine carcinoma of the pancreas mimicking intraductal papillary mucinous carcinoma

A previously healthy 52-year-old man was referred to our hospital for further evaluation of main pancreatic duct dilatation. The preoperative work-up was consistent with intraductal papillary mucinous carcinoma (IPMC) derived from a mixed type intraductal papillary mucinous neoplasm (IPMN), because multilocular cysts with enhancing thickened pancreatic head walls and dilated pancreatic ducts lined with dysplastic mucinous epithelium, with papillary proliferation from the pancreatic body to the tail, were observed; in addition, the pancreatic juice cytology was class V, which is suggestive of adenocarcinoma. Total pancreatectomy was performed because a definite mass was not found before surgical resection and the tumors could have spread to the tail. The pathological diagnosis was mixed adenoneuroendocrine carcinoma of the pancreatic head. IPMN with high- or low-grade dysplasia was not observed anywhere in the pancreatic duct. The pancreatic ductal adenocarcinoma consisted of large caliber malignant glands with intraluminal flat or papillary structures; therefore, we were unable to recognize a definite pancreatic mass before surgical resection, and suspected an IPMC derived from a mixed type IPMN.     

Management of mucinous cystic neoplasms of the pancreas

The purpose of this study was to investigate the actual management of mucinous cystic neoplasm (MCN) of the pancreas. A systematic review was performed in December 2009 by consulting PubMed MEDLINE for publications and matching the "pancreatic mucinous cystic neoplasm", "pancreatic mucinous cystic tumour", "pancreatic mucinous cystic mass", "pancreatic cyst", and "pancreatic cystic neoplasm" to identify English language articles describing the diagnosis and treatment of the mucinous cystic neoplasm of the pancreas. In total, 16 322 references ranging from January 1969 to December 2009 were analysed and 77 articles were identified. No articles published before 1996 were selected because MCNs were not previously considered to be a completely autonomous disease. Definition, epidemiology, anatomopathological findings, clinical presentation, preoperative evaluation, treatment and prognosis were reviewed. MCNs are pancreatic mucinproducing cysts with a distinctive ovarian-type stroma localized in the body-tail of the gland and occurring in middle-aged females. The majority of MCNs are slow growing and asymptomatic. The prevalence of invasive carcinoma varies between 6% and 55%. Preoperative diagnosis depends on a combination of clinical features, tumor markers, computed tomography (CT), magnetic resonance imaging, endoscopic ultrasound with cyst fluid analysis, and positron emission tomography-CT. Surgery is indicated for all MCNs.