Cystic ductal adenocarcinoma of pancreas: an unusual variant.

Cystic lesions of the pancreas are usually pseudocysts (90%); only 10% of them are cystic tumors. These cystic tumors constitute less than 10% of all pancreatic neoplasms, making them an extremely uncommon type of pancreatic malignancy. What is more important is that these tumors are very easily misdiagnosed as pseudocysts because their characteristics are very similar to those of the benign pseudocysts. This gains importance as the cystic tumors have a high cure rate and good prognosis if diagnosed and treated on time. Of all the cystic tumors, the most common are the benign serous cystadenomas, mucinous cystic tumors, intraductal papillary mucinous neoplasms etc. Ductal adenocarcinoma of pancreas presenting in cystic form is an uncommon type of cystic tumor, making it extremely rare among all pancreatic malignancies (solid or cystic). We present the following case report. The review of literature concerning the diagnosis and management has also been discussed.     

Cystic and solid lesions of the pancreas

More than 95% of malignant tumours of the pancreas are exocrine carcinomas. The exocrine carcinomas have to be distinguished from benign serous cystadenomas and tumours, the latter including mucinous cystic neoplasms, serous cysts, and solid pseudopapillary neoplasms. Cystic lesions have to be separated from pseudocysts, which are the most common cysts. Pseudocysts are due to extensive confluent autodigestive tissue necrosis caused by alcoholic, biliary, or traumatic acute pancreatitis. This review focuses on the classification of the different types of solid and cystic lesions based on histological criteria. The various imaging procedures are also discussed, along with their strengths and limitations.     

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.     

EUS in the evaluation of pancreatic cystic lesions

BACKGROUND: The differential diagnosis in pancreatic cystic lesions is often difficult despite the availability of various modern imaging modalities. This study assessed the role of EUS in the following: (1) discrimination of pseudocysts from pancreatic cystic tumors, (2) differential diagnosis between serous cystadenoma and mucinous cystic tumor, and (3) prediction of accompanying malignancy in intraductal papillary mucinous tumor. METHODS: EUS findings in 75 patients with pancreatic cystic lesions (58 cystic tumors, 17 pseudocysts) were evaluated. In the comparison of pseudocysts and cystic tumors, the latter included intraductal papillary mucinous tumor, mucinous cystic tumors, and serous cystadenomas, but not solid-pseudopapillary tumors. RESULTS: In univariate analysis, pseudocysts exhibited echogenic debris and parenchymal changes more often than cystic tumors did (respectively, 29% vs. 6%, p < 0.05; and 65% vs. 4%, p < 0.001). In contrast, septa and mural nodules were found more frequently in cystic tumors than pseudocysts (respectively, 69% vs. 12%, p < 0.001; 56% vs. 12%, p < 0.01). Multivariate analysis revealed that parenchymal changes (odds ratio [OR] = 83.59; p < 0.01); septa (OR = 30.75; p < 0.05); and mural nodules (OR = 21.38; p < 0.05) were independent predictors of differentiation between pseudocysts and cystic tumors. Serous cystadenoma exhibited diverse EUS features, as well as a honeycomb appearance. Mural nodules were found more often in mucinous cystic tumors than in serous cystadenomas (p < 0.05). There were no factors that predicted malignancy in intraductal papillary mucinous tumor. CONCLUSIONS: EUS is a useful complementary imaging method for differentiation of pancreatic cystic lesions.     

Cystic pancreatic lesions: current evidence for diagnosis and treatment

Pancreatic cystic neoplasms are detected at an increasing frequency due to an increased use and quality of abdominal imaging. There are well known differential diagnostic difficulties concerning these lesions. The aim is to review current literature on the diagnostic options and the following treatment for cystic lesions in the pancreas focusing on serous cystadenomas, primary mucinous neoplasm of the pancreas and mucinous cystadenocarcinomas, as well as intraductal papillary mucinous neoplasms, starting with excluding pseudocysts. A conservative approach is feasible in patients with a clinical presentation suggestive of an asymptomatic serous cystadenoma. Surgical management, as well as follow-up, is discussed for each of the types of neoplastic lesions, including an uncharacterized cyst, based on patient data, symptoms, serum analysis, cyst fluid analysis and morphological features. Aspects for future diagnostics and management of these neoplasia are commented upon.     

Cystic pancreatic lesions: Current evidence for diagnosis and treatment

Abstract

Pancreatic cystic neoplasms are detected at an increasing frequency due to an increased use and quality of abdominal imaging. There are well known differential diagnostic difficulties concerning these lesions. The aim is to review current literature on the diagnostic options and the following treatment for cystic lesions in the pancreas focusing on serous cystadenomas, primary mucinous neoplasm of the pancreas and mucinous cystadenocarcinomas, as well as intraductal papillary mucinous neoplasms, starting with excluding pseudocysts. A conservative approach is feasible in patients with a clinical presentation suggestive of an asymptomatic serous cystadenoma. Surgical management, as well as follow-up, is discussed for each of the types of neoplastic lesions, including an uncharacterized cyst, based on patient data, symptoms, serum analysis, cyst fluid analysis and morphological features. Aspects for future diagnostics and management of these neoplasia are commented upon.     

Evaluation and management of cystic pancreatic tumors: emphasis on the role of EUS FNA.

Cystic lesions of the pancreas are increasingly recognized and usually represent pseudocysts or cystic pancreatic tumors (CPTs), but also include congenital cysts, acquired cysts, extrapancreatic cysts, or cystic degeneration of solid tumors. It is important to distinguish CPT lesions given their varied prognosis and therapy. Mucinous varieties of CPTs (mucinous cystic neoplasms and intraductal papillary mucinous tumors) are premalignant or malignant, and surgical resection is generally recommended in good operative candidates. In contrast, nonmucinous CPTs include serous cystadenomas with a very low malignant potential, or pseudocysts, which are always benign. As a result, nonmucinous CPTs are generally resected only when inducing symptoms or complications. Review of the clinical, imaging, laboratory, and pathology information may clarify the specific tumor type. The relatively limited accuracy of any one modality requires that we consider the combined results when making management decisions.     

Rare case of adult pancreatic hemangioma and review of the literature

Pancreatic hemangiomas are a rare type of cystic tumor, with very few cases reported in the literature. Herein, we present the case of a 28-year-old woman who was admitted to our hospital for abdominal pain. A physical examination failed to reveal any abnormalities that could explain her symptoms. A contrast-enhanced computed tomography showed a multilocular cyst with moderately enhanced septa and fluid-fluid levels in the body and tail of the pancreas. A serous cystadenoma or pseudocyst of the pancreas was initially suspected, and the patient underwent a subtotal pancreatectomy and splenectomy. The pathologic diagnosis was a pancreatic hemangioma. This is the second case of pancreatic hemangioma with fluid-fluid levels reported in the literature. Upon imaging, the presentation of this tumor can resemble serous or mucinous cystadenomas, pseudocysts of the pancreas, and side-branch type intraductal papillary mucinous neoplasms. This report reviews the clinical symptoms, radiologic features, pathologic characteristics, differential diagnoses, and treatment of this rare lesion type.     

Management of the rare entity of primary pancreatic cystic neoplasms

Primary cystic neoplasms of the pancreas constitute a rare entity and are composed of a variety of neoplasms with a wide range of malignant potential. Approximately 90% of these lesions are serous cystic neoplasms or mucin-producing neoplasms. In contrast to serous cystadenomas which are nearly always benign, the mucinous cystic neoplasms represent a more diverse, heterogenous spectrum of related neoplasms. Intraductal papillary mucinous neoplasms manifest a much greater latent or overt malignant potential than other cystic neoplasms of the pancreas. The various subgroups of cystic neoplasms of the pancreas are evaluated and compared through a review of current literature. No symptoms or signs are pathognomonic for the cystic pancreatic neoplasms. While identification of a cystic tumor is relatively easy, the identification of the specific tumor type may be difficult. Most investigators agree that accurate differentiation of benign from malignant neoplasms can be made only at histopathologic examination of the entire resected segment of the pancreas. Because of the low mortality and low postoperative morbidity, surgical resection is indicated in all patients with cystic tumors.     

Differentiation of cystic pancreatic neoplasms and pseudocysts by conventional and echo-enhanced ultrasound

BACKGROUND AND AIMS: Echo-enhanced sonography is a non-invasive and increasingly used procedure for the differentiation of pancreatic tumors. However, the diagnostic accuracy of this procedure compared to conventional ultrasound for the differential diagnosis of cystic pancreatic neoplasms from pseudocysts has never been investigated in a prospective study. METHODS: Thirty-one patients with a cystic pancreatic lesion at the conventional ultrasound (mean age 57 years, range 36-82 years) were included in the study. Sonography was performed by an experienced examiner who was unaware of the patients' clinical diagnosis. The exact diagnosis was based on histological evidence from biopsy examination (surgical or transabdominal fine needle biopsy for all cystic neoplasms and five pseudocysts), or a follow-up of at least 18 months (four pseudocysts). RESULTS: Of the 31 patients, 19 had cystadenomas, three had cystadenocarcinomas, and nine had pseudocysts. Only 27% of the cystadenomas and 67% of the pseudocysts could be correctly classified by conventional ultrasound. Conversely, 95% of the cystadenomas (P = 0.0001) and all pseudocysts were diagnosed correctly by echo-enhanced sonography. The sensitivity of echo-enhanced sonography with respect to diagnosing cystadenoma was 95% and its specificity was 92%. The corresponding values for pseudocysts were both 100%. CONCLUSION: Echo-enhanced sonography has a high sensitivity and specificity in the differential diagnosis of cystic pancreatic tumors. With this procedure the differentiation of cystadenomas and pseudocysts can be improved. However, histology is the standard of reference.     

Zystische Pankreasl?sionen

Cystic changes of the pancreas comprise a large spectrum of neoplastic and non-neoplastic lesions. Among these are five entities, i.e. pseudocysts, intraductal papillary mucinous neoplasms (IPMN), mucinous cystic neoplasms (MCN), serous cystic neoplasms (SCN) and solid pseudopapillary neoplasms (SPN), which represent 95% of all cystic pancreatic lesions. While SCN and SPN have a good prognosis, IPMN and MCN have a high risk of malignancy as they are potential precursors of pancreatic ductal adenocarcinoma. The differential diagnosis between these five entities is based on epidemiology, pathology and clinical criteria. Consideration of these criteria allows in many cases a preoperative stratification that is the basis for an adequate therapy.     

Risk of malignancy in serous cystic neoplasms of the pancreas

BACKGROUND: In contrast to mucinous cystic neoplasms of the pancreas, which are known to have considerable malignant potential, the serous variant is generally thought to be benign. There are, however, several reports of malignancy in serous cystic neoplasms of the pancreas. AIMS: To assess the risk of malignancy of serous cystic tumors of the pancreas and to investigate specific clinical and histological features. METHODS: Clinical and pathological characteristics of benign and malignant serous cystic neoplasms of the pancreas were investigated by a review of the literature and documented by a case of a serous cystadenocarcinoma and immunohistochemical analysis of a series of serous cystadenomas. Reviewing the literature prevalence, age and sex distribution of serous cystic neoplasms were analyzed. RESULTS: The prevalence of cancer among serous cystic neoplasms reported since 1989 was 3%. Serous cystadenoma of the pancreas present at an earlier age (61 years) than serous cystadenocarcinoma (66 years; p = 0.056) and are symptomatic in the majority of patients.Pathological examination of the primary tumor was not able to distinguish cystadenoma from cystadenocarcinoma in 38% of cases. In 25% the diagnosis of cancer was established only after growth of metachronous metastases. In the present case, nuclear atypia, papillary structures, proliferation marker Ki-67 and p53 protein were increased in the primary tumor and/or metachronous metastasis. CONCLUSION: Serous cystic neoplasms of the pancreas do have malignant potential with a risk of malignancy of 3% and should be surgically treated if the operative risk is acceptable. Routine analysis of genetic and proliferation markers may improve diagnosis of malignancy in these tumors.     

Cystic neoplasms of the pancreas

Cystic neoplasms of the pancreas, including 4 patients with serous cystadenoma and 11 with mucinous cystic neoplasm, were studied. Serous cystadenomas composed of epithelial cells with glycogen in the cytoplasm were benign in all, whereas mucinous cystic neoplasms consisted of four benign, two borderline, and five malignant lesions. The average size was 1.8 cm in the greatest diameter in benign mucinous cystadenomas, about 5 cm in borderline tumors, and more than 8 cm in mucinous cystadenocarcinomas. Histologically, in all borderline and malignant lesions, the cysts contained areas lined with epithelium and had a benign appearance, comparable with that of mucinous cystadenoma. These observations suggest a borderline or malignant disease in the case of a benign mucinous cystadenoma. An immunohistochemical study for carcinoembryonic antigen and carbohydrate antigen 19-9 showed denser and diffuse localizations of such materials in the cytoplasm and the stroma in the case of malignant lesions, as compared to findings in borderline and benign cases.     

Molecular assessment of endoscopically collected pancreatic juice and duodenal fluid from patients with pancreatic diseases

One concern associated with pancreatic diseases is the poor prognosis of pancreatic cancer. Even with advances in diagnostic modalities, risk stratification of premalignant lesions and differentiation of pancreatic cysts are challenging. Pancreatic lesions of concern include intraductal papillary mucinous neoplasms, mucinous cystic neoplasms, serous cystadenomas, pseudocysts, and retention cysts, as well as cystic degeneration of solid tumors such as solid pseudopapillary neoplasms and pancreatic neuroendocrine neoplasms. Pancreatic juice obtained during endoscopic retrograde cholangiopancreatography has previously been used for the detection of KRAS mutation. Recently, duodenal fluid, which can be obtained during the relatively minimally invasive procedures of endoscopic ultrasound (EUS) and esophagogastroduodenoscopy, and cyst fluid collected by EUS-guided fine-needle aspiration (FNA) were used for molecular biological analysis. Furthermore, advanced analytic methods with high sensitivity were used for the detection of single and multiple markers. Early detection of malignant pancreatic tumors and risk stratification of premalignant tumors can be performed using duodenal fluid samples with a single marker with high sensitivity. Technological advances in simultaneous detection of multiple markers allow for the differentiation of cystic pancreatic tumors. One thing to note is that the clinical guidelines do not recommend pancreatic cyst fluid and pancreatic juice (PJ) sampling by EUS-FNA and endoscopic retrograde cholangiopancreatography, respectively, in actual clinical practice, but state that they be performed at experienced facilities, and duodenal fluid sampling is not mentioned in the guidelines. With improved specimen handling and the combination of markers, molecular markers in PJ samples may be used in clinical practice in the near future.     

Serous cystic neoplasm of the pancreas-indications for surgery

BACKGROUND/AIMS: Serous cystic neoplasm is a rare pancreatic tumor. Almost all of these tumors are benign and only 9 cases of serous cystadenocarcinoma have been reported. Although serous cystic neoplasm is typically a microcystic lesion, there is a wide range of cyst sizes from micro to macro and even unilocular cysts have been reported. Thus, the diagnosis is difficult and indications for surgery are controversial. We aimed to elucidate the clinicopathological and imaging features of serous cystic neoplasm of the pancreas. METHODOLOGY: We investigated 15 cases of resected and 6 cases of nonresected cases of serous cystic neoplasm, evaluating the symptoms, imaging findings, preoperative diagnosis, macroscopic morphology, microscopic findings, and results of follow-up. RESULTS: Imaging diagnosis of serous cystic neoplasm was not easy, because not so many tumors had the typical microcystic pattern. Most of the resected serous cystic neoplasms were non-microcystic or were small tumors, which could not be precisely evaluated. CONCLUSIONS: Small serous cystic neoplasms, which can be diagnosed by imaging, do not need to be resected because serous cystadenocarcinoma is rare. Tumors of the pancreas that cannot be confirmed to be serous cystic neoplasm should be resected because of the possibility of pancreatic cancer, mucinous cystadenocarcinoma, or mucinous cystadenoma with malignant potential.     

Serous cystadenoma of the pancreas presenting as a third primary neoplasm.

Serous cystadenomas are the most common cystic neoplasms of the pancreas. They may occur solely or coexist with other neoplasms. A 10 cm mass involving the body of the pancreas was observed in the computed tomography of a 61-year-old man with a previous history of bladder and prostate carcinoma. Ultrasonography and computed tomography of the mass demonstrated multiple small cysts associated with a central calcified scar. A distal pancreatectomy was performed. Pathological examination confirmed the diagnosis of serous microcystic adenoma. This is the first report of a serous cystadenoma of the pancreas with two metachronous neoplasms. This feature should be kept in mind during the diagnosis and evaluation of patients with serous cystadenoma.     

Cystic pancreatic neoplasms: computed tomography and magnetic resonance imaging findings.

Cystic pancreatic neoplasms include serous cystadenomas (SCA), mucin-producing cystic tumors, cystic islet-cell tumors, and cystic solid and papillary epithelial neoplasms. Imaging techniques are of great value in the demonstration and differential diagnosis of these tumors. In this article we present the computed tomography (CT) and magnetic resonance imaging (MRI) findings of the aforementioned cystic neoplasms. Although the radiological features are in many cases informative, a significant overlap does exist; fine-needle aspiration biopsy and cytology or excisional biopsy and histological examination are necessary to determine a definitive diagnosis.     

Cystic pancreatic neoplasms Computed tomography and magnetic resonance imaging findings

Cystic pancreatic neoplasms include serous cystadenomas (SCA), mucin-producing cystic tumors, cystic islet-cell tumors, and cystic solid and papillary epithelial neoplasms. Imaging techniques are of great value in the demonstration and differential diagnosis of these tumors. In this article we present the computed tomography (CT) and magnetic resonance imaging (MRI) findings of the aforementioned cystic neoplasms. Although the radiological features are in many cases informative, a significant overlap does exist; fineneedle aspiration biopsy and cytology or excisional biopsy and histological examination are necessary to determine a definitive diagnosis.     

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.     

Laparoscopic fenestration of multiple giant biliary mucinous cystadenomas of the liver

Biliary cystadenomas of the liver are rare, cystic neoplasms of the biliary ductal system usually occur in middle aged women. We report a case of synchronous multiple huge biliary mucinous cystadenomas with unique features. This is, according to our knowledge, the first report in the literature about three synchronously occurring hepatobiliary cystadenomas. Cystadenomas have a strong tendency to recur, particularly following incomplete excision, and a potential of malignant transformation. A therapeutic re-evaluation may be necessary when the diagnosis of hepatobiliary cystadenoma is made after the operation and an open liver resection should be considered.