Serous cystadenoma of the pancreas communicating with a pancreatic duct

Summary Conclusion To differentiate serous cystadenoma from other cystic lesions communicating with the pancreatic duct, careful radiological examination is necessary. Background Communication between the cystic cavity and the pancreatic duct in an ordinary serous cystadenoma is uncommon, although it is not uncommon in other cystic lesions, such as pseudocyst, mucinous cystadenoma/adenocarcinoma, and intraductal papillary tumor. Methods A serous cystadenoma of the pancreas communicating with main pancreatic duct in a 76-yr-old male is reported. Results The communication was preoperatively demonstrated by endoscopic retrograde cholangiopancreatography and confirmed by histopathological examination of the resected specimen.     

Cystic pancreas adenoma. On the clinical relevance of histologic typing

Although pancreatic cystadenomas are rare neoplasms, they are found today in rising frequencies due to improvement of diagnostic tools. There exist two types of cystadenoma: microcystic serous cystadenoma and mucinous cystadenoma. Usually, histological distinction from cystadenocarcinoma is readily made, but it may be difficult in the case of the mucinous variant. This subtype is supposed to be potentially malignant, whereas microcystic serous cystadenoma is always benign. In order to elucidate the characteristics of both variants, 5 own cases are reported in this article.     

Endoscopic ultrasound characteristics of mucinous cystic neoplasms of the pancreas

OBJECTIVE: Mucinous cystic neoplasms of the pancreas have a more favorable prognosis than ductal adenocarcinoma. Management of a subgroup, intraductal papillary-mucinous neoplasms, is controversial. Endoscopic ultrasound (EUS) with fine-needle aspiration biopsy may emerge as the imaging modality of choice. There are few studies describing the EUS features of these tumors. METHODS: A total of 35 consecutive cases of cystic tumors of the pancreas with an established pathological diagnosis were analyzed for characteristic EUS features. RESULTS: Mucinous cystadenocarcinomas (n = 14) were more likely to be characterized by hypoechoic cystic/solid mass or complex cyst and were frequently associated with a dilated main pancreatic duct. Benign mucinous duct ectasia (n = 6) were characterized by a dilated main pancreatic duct in conjunction with hyperechoic thickening of the duct wall. The two cases of intraductal mucinous hyperplasia additionally showed a hypoechoic mass. Intraductal papillary carcinoma (n = 11) had features in common with mucinous cystadenocarcinoma but also had echogenic foci in the mass and intraductal hyperechoic lesions. The two cases of microcystic cystadenoma showed either a mixed hypoechoic solid/cystic mass or a complex cyst without the additional features seen in mucinous cystadenocarcinoma. CONCLUSIONS: EUS features seem to exist that may help to differentiate cystic neoplasms from adenocarcinoma of the pancreas and, thus, to establish the preoperative diagnosis of cystic tumors of the pancreas.     

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.     

胰腺囊性肿瘤19例的诊断与外科治疗

目的 探讨胰腺囊性肿瘤的诊断及外科治疗方法.方法 对我院普外科2000年1月至2009年8月诊治的19例胰腺囊性肿瘤的临床资料进行回顾性分析.结果 胰腺囊性肿瘤无特征性临床表现,B超和CT是其主要诊断手段,但均不能准确区分其病理类型,与术后病理对照的定性诊断符合率分别为57.9%(11/19)和68.4%(13/19).肿瘤位于胰头颈部5例,胰体尾部14例,最大直径3～15cm.19例均行手术治疗,切除肿瘤16例,总切除率为84.2%.术中误诊误治4例(21.0%).病理证实浆液性囊腺瘤6例,黏液性囊腺瘤6例,黏液性囊腺癌5例,导管内乳头状黏液腺瘤2例.获得随访15例(78.9%),3例囊腺癌患者中1例切除者已存活4年,无复发;2例未切除者分别于术后4个月和7个月病死.12例囊腺瘤患者目前均存活,肿瘤无复发.失访4例,囊腺癌和囊腺瘤各2例.结论 加强对胰腺囊性肿瘤的认识是减少误诊误治的关键;胰腺囊性肿瘤手术切除后疗效满意,故一经诊断即应积极行外科手术切除.     

Expression of p57/Kip2 protein in pancreatic adenocarcinoma

INTRODUCTION: Evaluation of the biologic character of carcinomas requires understanding of cell cycle regulators. AIMS: To investigate p57 expression in human pancreatic adenocarcinoma and cyst adenoma. METHODOLOGY: We examined the expression of p57(Kip2), a member of the Cip/Kip family, in 45 pancreatic adenocarcinomas, 7 cystadenomas, and 7 chronic pancreatitis cases. RESULTS: The p57 labeling index (LI) in duct epithelia in chronic pancreatitis averaged 32.8+/-8.3 and was significantly higher than in normal duct epithelia (18.8+/-6.6; p = 0.0011). For the carcinoma, the LI averaged 46.0+/-20.9, which was significantly higher than that for normal duct epithelia (p < 0.0001) and cystadenoma (16.0 11.2; p = 0.0007). However, it was significantly reduced in cases with stage IV disease (p = 0.0351), lymph node metastasis (p = 0.0003), larger size (p = 0.0094), capsular invasion (p = 0.0462), lymphatic invasion (p = 0.0351), and cell proliferating activity (p = 0.0002). In multivariate analysis, p57 LI in pancreatic adenocarcinoma was independently linked to high proliferating activity (p = 0.0230). CONCLUSION: These results suggest that p57 plays a role in the hyperplastic change of the ducts in chronic pancreatitis and that pS7 overexpression contributes to the downregulation of cell proliferation, and its decreased expression contributes to the progression of pancreatic adenocarcinoma.     

A report of 5 cases of cystic bile duct carcinoma of the liver and proposal of a new classification

Primary biliary cystadenocarcinoma of the liver is rare. Among 239 patients with primary liver cancer admitted to our service during the last 13 years, there were 5 cases of cystic bile duct carcinoma of the liver. Three of these were cystadenocarcinoma, one was adenocarcinoma arising from a liver cyst, and one was carcinoma of the intrahepatic bile ducts with cystic dilatation. A better classification of these entities seems necessary, and it is suggested that malignant cystic tumors of the liver should be divided into 3 groups: Group A is cystic adenocarcinoma, group B is bile duct carcinoma with primary or secondary intrahepatic bile duct, and group C is degenerative cyst formation by other types of malignant tumors. Cystic adenocarcinoma (Group A) can then be further subdivided into cystadenocarcinoma, cystadenocarcinoma with cystadenoma, and carcinoma in a simple cyst of the liver.     

A report of 5 cases of cystic bile duct carcinoma of the liver and proposal of a new classification.

Primary biliary cystadenocarcinoma of the liver is rare. Among 239 patients with primary liver cancer admitted to our service during the last 13 years, there were 5 cases of cystic bile duct carcinoma of the liver. Three of these were cystadenocarcinoma, one was adenocarcinoma arising from a liver cyst, and one was carcinoma of the intrahepatic bile ducts with cystic dilatation. A better classification of these entities seems necessary, and it is suggested that malignant cystic tumors of the liver should be divided into 3 groups: Group A is cystic adenocarcinoma, group B is bile duct carcinoma with primary or secondary intrahepatic bile duct, and group C is degenerative cyst formation by other types of malignant tumors. Cystic adenocarcinoma (Group A) can then be further subdivided into cystadenocarcinoma, cystadenocarcinoma with cystadenoma, and carcinoma in a simple cyst of the liver.     

Operative indications for cystic lesions of the pancreas with malignant potential--our experience

BACKGROUND/AIMS: There are still many important but unclear points regarding the differential diagnosis and operative indications of cystic lesions of the pancreas with malignant potential. Studies of the clinicopathological and molecular biological characteristics of such diseases are necessary. In this paper, we discuss operative indications for this condition based on a review of the literature and our own experience. METHODOLOGY: Seven cases of serous cystadenoma and 9 cases of mucinous cystadenoma or cystadenocarcinoma of the pancreas that were operated on or autopsied in our department from 1980 to 1996 were analyzed clinicopathologically. Small cystic lesions incidentally found in 300 autopsied cases were also studied. Finally, mucin-producing tumors described in several reports were reviewed, and the branch type of this tumor was especially investigated. RESULTS: A marked disappearance of pancreatic acini in the upstream pancreas was found when serous cystadenoma became large. Papillary projection was histologically found in all of the cases. Tumorous invasion to the interstitium was suspected in tumors more than 5 cm in diameter, and malignancy was reported when tumors were larger than 6 cm. As for mucinous cystadenocarcinoma, the patients had a poor prognosis. In 2 of 42 cases with a pseudocyst, small duct cell carcinoma was incidentally found adjacent to the pseudocyst on the duodenal side. With regard to branch-type intraductal papillary neoplasm, 80% of the tumors larger than 4 cm were malignant. Most of the small cystic lesions found in elderly autopsy cases were accompanied by hyperplastic epithelia without evidence of malignancy. CONCLUSIONS: Based on our experience, an operation should be considered and resection is recommended under the following circumstances: 1) cystic lesions in the body and tail of the pancreas in middle-aged women; 2) typical serous cystadenoma larger than 4 cm; 3) mucinous cystadenoma of any size; 4) branch-type intraductal papillary neoplasm larger than about 3 cm; and, 5) pseudocysts of unknown cause. Small cystic lesions in elderly patients should not necessarily be operated on, but should be followed-up carefully.     

Macrocystic type of serous cystadenoma with a communication between the cyst and pancreatic duct

A 42-year-old woman with a cystic lesion in the head of the pancreas was evaluated by using abdominal ultrasonography, a computed tomographic scan, magnetic resonance imaging and endoscopic retrograde pancreatography. Multiple cystic lesions, 5 cm in diameter, which had papillary protrusion inside the cyst in the head of the pancreas and had the communication between the cysts and pancreatic duct, were determined. Pylorus-preserving pancreaticoduodenectomy was performed under the diagnosis of mucinous cystic neoplasm of the pancreas. Although the cut surface of the tumor showed a macrocystic tumor of 3 cm in diameter, part of the cyst wall was cavernous. A histopathological examination showed single-layered cuboidal cells, which lead to the diagnosis as being serous cystadenoma of the pancreas. Serous cystadenoma is a rare, almost benign pancreatic tumor. The macrocystic subtype of serous cystadenoma is even more rare. We describe a patient who had this macrocystic subtype of serous cystadenoma with a communication between the cyst and pancreatic duct. This case illustrates the difficulty in the diagnosis of cystic lesions in the pancreas, and might support the single category of cystic lesions of the pancreas.     

Ki-ras codon 12 point mutation and p53 mutation in pancreatic diseases.

BACKGROUND/AIMS: The Ki-ras gene located at 12p, encodes the GTP binding protein involving the signal transduction system and concerns cell proliferation and differentiation. METHODOLOGY: Pancreatic tissues were obtained from 37 patients with various pancreatic diseases. Ki-ras codon 12 point mutation and p53 (exon 5-8) mutation were examined in 3 patients with chronic pancreatitis, 9 mucinous adenoma of the pancreas (2 with mucinous cystadenoma and 7 with intraductal papillary-mucinous adenoma), 22 pancreatic ductal carcinoma, and 3 serous cystadenoma. RESULTS: On usual pancreatic exocrine ductal lesions, Ki-ras point mutation was evident in 0% (0/3) of chronic pancreatitis, in 56% (5/9) of mucinous adenoma, and in 57% (12/21) of ductal carcinoma, the mutation being located in the second letter in 18 and in the 1st letter in 2. One Ki-ras codon 12 positive pancreatic cancer showed Ki-ras codon 12 point mutation in the surrounding pancreas (2nd letter mutation in both areas). p53 mutation was present in 0% (0/1) of chronic pancreatitis, in 0% (0/8) of mucinous adenoma, while it was evident in 29% (6/21) of pancreatic ductal carcinoma, the mutation being situated in exon 5 in 3, in exon 6 in 1, and in exon 7 in 2. In 3 patients with serous cystadenoma, there was no mutation in Ki-ras codon 12 or p53 (exon 5-8). CONCLUSIONS: These findings suggest that Ki-ras point mutation is involved in the early events of pancreatic ductal carcinoma, while p53 mutation is intricated in the late phase of pancreatic ductal carcinogenesis and the histogenesis of serous cystadenoma is different from that of pancreatic exocrine ductal lesions including mucinous adenoma and ductal carcinoma.     

Selective Endoscopic Retrograde Pancreatography (Selective ERP) Using a Radifocus Gllidewire–A New Technique for a Diagnostic Pancreatogram

Abstract: We have developed a new method called the “Selective Endoscopic Retrograde Pancreatograpy (Selective ERP)” which uses a Radifocus guidewire to obtain a more precise pancreatogram than a standard ERP. The deep cannulation of a catheter guided by a Radifocus guidewire following standard ERP was easily and rapidly accomplished and precise pancreatograms were successfully obtained. The Selective ERP was very useful not only for obtaining detailed pancreatograms beyond severe stenosis of the main pancreatic duct in ductal cell carcinoma of the pancreas but also in revealing whether mucinous cystadenoma/cystadenocarcinoma communicated with the pancreatic duct or not. In the case of a mucin producing pancreatic tumor, Selective ERP successfully enabled us to obtain a whole pancreatogram and the location and extent of a tumor that standard ERP often fails to reveal because of an abundant accumulation of mucin in the duct. A Selective ERP is a simple and useful method for diagnosing pancreatic tumors and can provide precise and whole pancreatograms. We recommend Selective ERP as it enables us to obtain more complete diagnostic pancreatograms following standard ERP.     

Macrocystic pancreatic cystadenoma: The role of EUS and cyst fluid analysis in distinguishing mucinous and serous lesions

BACKGROUND: Benign pancreatic serous cystadenoma usually is morphologically distinguishable from mucinous cystadenomas, which require resection because of their malignant potential. A macrocystic variant of serous cystadenoma recently has been described, rendering this important distinction more difficult. The aim of this study was to determine the EUS and tumor marker characteristics of mucinous cystadenoma compared with macrocystic serous cystadenomas. METHODS: Medical records for consecutive patients seen between 1995 and 2002, with a histopathologic diagnosis of mucinous cystadenoma or macrocystic serous cystadenoma after surgery, who had undergone a detailed EUS examination, including EUS-guided FNA, were retrospectively reviewed. RESULTS: A resection specimen was available for 32 mucinous cystadenomas and 9 macrocystic serous cystadenomas. No significant differences were observed with regard to clinical data (age, gender, presence of symptoms), lesion size, and location within the pancreas. All mucinous cystadenomas had a discernible cyst wall (thickened, 66%; focal parietal nodules, 25%) compared with 56% of macrocystic serous cystadenomas (p<0.0001). A thick echo content also was more frequent in mucinous cystadenoma (56% vs. 11%; p=0.04; statistical significance removed by the Bonferroni correction). Microcysts were only observed in macrocystic serous cystadenomas (44%; p=0.0008). The combination of a cyst wall that is thickened and the absence of microcysts had a sensitivity of 100% and specificity of 78% for the diagnosis of mucinous cystadenoma compared with macrocystic serous cystadenoma. Although intracystic carbohydrate-associated antigen 72-4 and mucins M1 were non-discriminatory, low carcinoembryonic antigen (<5 ng/mL) and carbohydrate-associated antigen 19-9 (<50,000 U/mL) values were found in macrocystic serous lesions (respectively, 100% and 100%; p=0.0002 and p=0.0002). CONCLUSIONS: Although there is considerable overlap, helpful EUS characteristics that differentiate mucinous cystadenoma from macrocystic serous cystadenoma include a thick cyst wall and microcysts. These features, coupled with analysis of aspirated fluid for tumor markers (especially carcinoembryonic antigen), should help to confirm the diagnosis.     

Mixed serous cystadenoma with mucinous cystadenoma of the pancreas

We report a case of serous cystadenoma of the pancreas mixed with mucinous cystadenoma. A 65-year-old woman was admitted to our hospital for evaluation of a palpable, elastic, hard mass measuring 6 cm in diameter in the right upper quadrant of the abdomen. A diagnosis of mucinous cystadenocarcinoma of the pancreas was made, and pancreatoduodenectomy was performed. The tumor was composed of a dominant compartment of macroscopic cyst, and its thick wall was filled with numerous microscopic cysts. The light microscopy findings with hematoxylin and eosin staining, and by the periodic acid-Schiff reaction, were almost perfectly consistent with the characteristics of microcystic or glycogen-rich cystadenoma, but the apical portion of the cytoplasm of the neoplastic cells was stained with Alcian blue at pH 2.4 and by the mucicarmine method. Neoplastic cells containing epithelial acidic mucin are usually found in mucinous cystadenomas. No K-ras point mutations were detected at the sites where neoplastic cells were present, whether or not they contained epithelial acidic mucin. Pancreatic serous cystadenomas that include a mucinous-cystadenoma component are extremely rare, and the difference between serous and mucinous cystadenomas is not always distinct.     

Surgical Management of Pancreatic Cysts: A Shifting Paradigm Toward Selective Resection

Due to the widespread use of high-quality cross-sectional imaging, pancreatic cystic neoplasms are being diagnosed with increasing frequency. Clinicians are therefore asked to counsel a growing number of patients with pancreatic cysts diagnosed incidentally at an early, asymptomatic stage. Over the last two decades, accumulating knowledge on the biologic behavior of these neoplasms along with improved diagnostics through imaging and endoscopic cyst fluid analysis have allowed for a selective therapeutic approach toward these neoplasms. On one end of the management spectrum, observation is recommended for typically benign lesions (serous cystadenoma), and on the other end, upfront resection is recommended for likely malignant lesions (main duct IPMN, mucinous cystadenoma, solid pseudopapillary tumor, and cystic pancreatic neuroendocrine tumors). In between, management of premalignant lesions (branch duct IPMN) is dictated by the presence of high-risk features. In general, resection should be considered whenever the risk of malignancy is higher than the risk of the operation. This review aims to describe the evolution and current status of evidence guiding the selection of patients with pancreatic cystic neoplasms for surgical resection, along with a specific discussion on the type of resection required and expected outcomes.     

Pancreatic Cystic Neoplasms: Predictors of Malignant Behavior and Management

Background/Aim:

Pancreatic cystic neoplasms are being increasingly identified with the widespread use of advanced imaging techniques. In the absence of a good radiologic or pathologic test to preoperatively determine the dianosis, clinical characteristics might be helpful. The objectives of this analysis were to define the incidence and predictors of malignancy in pancreatic cysts.

Patients and Methods:

Patients with true pancreatic cysts who were treated at our institution were included. Patients with documented pseudocysts were excluded. Demographic data, clinical manifestations, radiological, surgical, and pathological records of those patients were reviewed.

Results:

Eighty-one patients had true pancreatic cyst. The mean age was 47 ± 15.5 years. There were 28.4% serous cystadenoma, 21% mucinous cystadenoma, 6.2% intraductal papillary tumors, 8.6% solid pseudopapillary tumors, 1.2% neuroendocrinal tumor, 3.7% ductal adenocarcinoma, and 30.9% mucinous cystadenocarcinoma. Malignancy was significantly associated with men (P = 0.04), older age (0.0001), cysts larger than 3 cm in diameter (P = 0.001), presence of solid component (P = 0.0001), and cyst wall thickening (P = 0.0001). The majority of patients with malignancy were symptomatic (26/28, 92.9%). The symptoms that correlated with malignancy included abdominal pain (P = 0.04) and weight loss (P = 0.0001). Surgical procedures were based on the location and extension of the lesion.

Conclusion:

The most common pancreatic cysts were serous and mucinous cysts. These tumors were more common in females. Old age, male gender, large tumor, presence of solid component, wall thickness, and presence of symptoms may predict malignancy in the cyst.     

Hemolymphangioma: A rare differential diagnosis of cystic-solid or cystic tumors of the pancreas

We report a case of pancreatic hemolymphangioma. Hemolymphangioma is a malformation of both lymphatic vessels and blood vessels. The incidence of this disease in the pancreas is extremely rare. To the best of our knowledge, only seven cases have been reported worldwide (PubMed). A 39-year-old woman with a oneday history of abdominal pain was admitted to our hospital. There was no obvious precipitating factor. The preoperative examination, including ultrasonography and computed tomography, showed a cystic-solid tumor in the pancreas, and it was considered to be a mucinous cystadenoma or cystadenocarcinoma. Pancreaticbody-tail resection combined with splenectomy was performed. After the operation, the tumor was pathologically demonstrated to be a pancreatic hemolymphangioma. Although pancreatic hemolymphangioma is rare, we believe that it should be considered in the differential diagnosis of cystic-solid tumors of the pancreas, particularly when there is no sufficient evidence for diagnosing cystadenoma, cystadenocarcinoma or some other relatively common disease of the pancreas.     

Primary Biliary Cystic Tumors of the Liver

Five cases of primary cystic tumors of the intrahepatic bile duct are documented over a 5-yr period. Clinically, two cases had recurrent episodes and one had a first attack of jaundice, fever, and pain in the upper abdomen; one complained of abdominal fullness and one was asymptomatic. The appearance of the tumors were single, large, multilocular, and cystic. Important radiological findings included irregular thickness of the cystic wall, internal septation, and papillary projection. Marked biliary dilation was demonstrated in four of the patients. On the cholangiogram, amorphous filling defects were seen in the dilated extrahepatic bile ducts, and mucinous material could be removed from the ducts as well. Obstruction of the bile ducts by this mucinous bile was assumed to be the cause of cholangitis in three patients. Histological examination revealed three cases of cystadenoma and two cases of cystadenocarcinoma.     

Obstructive Jaundice Caused by Mucinous Cystic Tumor of Gallbladder: A Case Report and Literature Review

Mucinous cystic tumor of the gallbladder is an extremely rare benign tumor, with potential for malignant degeneration. Mucinous cystic tumors of the cystic duct are divided into mucinous cystadenoma and mucinous cystadenocarcinoma. Currently, cystadenoma is generally considered to be a precancerous lesion of cystadenocarcinoma. At present, there are few cases reported worldwide, and there are no relevant guidelines for diagnosis and treatment of this disease. This article presents the collected clinical data of a patient with mucinous cystic tumor of the gallbladder who was admitted to the First Affiliated Hospital of Hunan Normal University, with the characteristics of the disease summarized in combination with a focused literature review.     

A case of macrocystic serous cystadenoma of the pancreas

Summary A case of macrocystic serous cystadenoma of the pancreas is presented, and literature is reviewed. A 35-yr-old woman presented with mild upper abdominal pain. Abdominal ultrasonography and an abdominal computed tomography revealed a multiloculated and calcified cyst in the body of the pancreas. A T1-weighted image, using magnetic resonance imaging, revealed a low-intensity mutiloculated, pancreatic mass. In contrast, T2-imaging of the tumor showed a high-intensity mass. Endoscopic retrograde cholangiopancreatography showed no contact between the main pancreatic duct and the tumor. The preoperative diagnosis was a mucinous cystic neoplasm. Tumor enucleation was performed. Subsequent microscopic examination of this tumor suggested the diagnosis of a macrocystic serous cystadenoma of the pancreas.