Cystic micropapillary neoplasm of peribiliary glands with concomitant perihilar cholangiocarcinoma

We report a case of a 75-year-old man with cystic micropapillary neoplasm of peribiliary glands detected preoperatively by radiologic examination. Enhanced computed tomography showed a low-density mass 2.2 cm in diameter in the right hepatic hilum and a cystic lesion around the common hepatic duct. cholangiocarcinoma, right hepatectomy with caudate lobectomy and bile duct resection were performed. Pathological examination revealed perihilar cholangiocarcinoma mainly involving the right hepatic duct. The cystic lesion was multilocular and covered by columnar lining epithelia exhibiting increased proliferative activity and p53 nuclear expression; it also contained foci of micropapillary and glandular proliferation. Therefore, the lesion was diagnosed as a cystic micropapillary neoplasm of peribiliary glands and resembled flat branch-type intraductal papillary mucinous neoplasm of the pancreas. Histological examination showed the lesion was discontinuous with the perihilar cholangiocarcinoma. Immunohistochemistry showed the cystic neoplasm was strongly positive for MUC6 and that the cholangiocarcinoma was strongly positive for MUC5 AC and S100 P. These results suggest these two lesions have different origins. This case warrants further study on whether this type of neoplasm is associated with concomitant cholangiocarcinoma as observed in pancreatic intraductal papillary mucinous neoplasm with concomitant pancreatic duct adenocarcinoma.     

European experts consensus statement on cystic tumours of the pancreas

Cystic lesions of the pancreas are increasingly recognized. While some lesions show benign behaviour (serous cystic neoplasm), others have an unequivocal malignant potential (mucinous cystic neoplasm, branch- and main duct intraductal papillary mucinous neoplasm and solid pseudo-papillary neoplasm). European expert pancreatologists provide updated recommendations: diagnostic computerized tomography and/or magnetic resonance imaging are indicated in all patients with cystic lesion of the pancreas. Endoscopic ultrasound with cyst fluid analysis may be used but there is no evidence to suggest this as a routine diagnostic method. The role of pancreatoscopy remains to be established. Resection should be considered in all symptomatic lesions, in mucinous cystic neoplasm, main duct intraductal papillary mucinous neoplasm and solid pseudo-papillary neoplasm as well as in branch duct intraductal papillary mucinous neoplasm with mural nodules, dilated main pancreatic duct >6 mm and possibly if rapidly increasing in size. An oncological partial resection should be performed in main duct intraductal papillary mucinous neoplasm and in lesions with a suspicion of malignancy, otherwise organ preserving procedures may be considered. Frozen section of the transection margin in intraductal papillary mucinous neoplasm is suggested. Follow up after resection is recommended for intraductal papillary mucinous neoplasm, solid pseudo-papillary neoplasm and invasive cancer.     

Simultaneous liver mucinous cystic and intraductal papillary mucinous neoplasms of the bile duct:A case report

Cystic hepatic neoplasms are rare tumors,and are classified into two separate entities:mucinous cystic neoplasms(MCNs)and intraductal papillary mucinous neoplasms of the bile duct(IPMN-B).We report the case of a 56-year-old woman who presented with abdominal pain and jaundice due to the presence of a large hepatic multilocular cystic tumor associated with an intraductal tumor.Partial hepatectomy with resection of extrahepatic bile ducts demonstrated an intrahepatic MCN and an intraductal IPMN-B.This is the first report of the simultaneous occurrence of these two histologically distinct entities in the liver.     

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.     

Cystic Lesions of the Pancreas

Opinion statement Pancreatic cystadenomas are a group of benign, premalignant, and malignant cystic tumors of the pancreas. Serous cystadenomas are benign lesions that often do not require surgical excision unless they are complicated by bleeding, obstruction, or pain. Mucinous cystadenomas are premalignant lesions that may be surgically excised if there is a concern regarding malignant degeneration. However, it may be difficult to predict the timing and risk of malignant change. Also, it is controversial whether all mucinous cystadenomas should be resected. Cystadenocarcinomas should be surgically managed if they are resectable, that is, there is no evidence of metastatic disease. Intraductal papillary mucinous tumors share many features with mucinous cystadenomas. However, intraductal papillary mucinous tumors arise from the pancreatic duct and are often associated with a dilated pancreatic duct. These lesions are often managed with surgical resection, including total pancreatectomy for diffuse lesions with evidence of localized malignancy.     

Long-term follow-up of intraductal papillary adenoma of the pancreas

Intraductal papillary mucinous tumors of the main pancreatic duct are often considered to be premalignant or malignant, and therefore surgical resection is recommended. We report two autopsy cases of intraductal papillary mucinous tumor of the pancreas following long-term observation. The first patient was an 84-year-old man with early gastric cancer treated by endoscopic mucosectomy. The second patient was a 77-year-old man with hepatocellular carcinoma treated by percutaneous pure ethanol injection and transarterial embolization. In both patients, endoscopic retrograde pancreatography showed a diffusely dilated main pancreatic duct, with intraductal filling defects expressing mucus, as well as dilated side branches. Obvious intramural nodules were not detected. Due to their advanced age and personal requests, both patients were managed conservatively and followed nonoperatively. In the first patient, serial pancreatograms showed progression of the pancreatic duct dilatation. Both patients died of gastric cancer, the first patient 7¹/₂ years and the second, 10 years after first presentation, respectively. Autopsies revealed extensive intraductal papillary adenoma throughout the dilated mucus-filled main pancreatic duct. However, there was no evidence of progression to adenocarcinoma. Based on these observations, we suggest that, in patients with intraductal papillary mucinous tumor of the pancreas without obvious intramural nodules, even if the tumor is in the main pancreatic duct, pancreatectomy may not be mandatory, particularly in the elderly. Received: March 21, 2001 / Accepted: July 6, 2001 Reprint requests to: T. Kamisawa     

Intraductal papillary mucinous tumors and mucinous cystic tumors of the pancreas: imaging

Most cystic lesions of the pancreas are nonneoplastic and inflammatory in nature. However, approximately 5%–15% of cystic pancreatic masses may be neoplastic. Among the cystic neoplasms are the mucin-producing tumors, both the intraductal papillary mucinous neoplasms and the mucinous cystic neoplasms. Their imaging features on contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) can assist in the differentiation of these lesions. The imaging findings of both intraductal papillary mucinous neoplasm and mucinous cystic neoplasm are reviewed with attention to CT and MRI.     

p53 protein expression in intraductal papillary mucinous tumors (IPMT) of the pancreas as an indicator of tumor malignancy

BACKGROUND/AIMS: Intraductal papillary mucinous tumors, as a cystic disease in the pancreas, clinically has a more indolent and favorable course than invasive ductal pancreas carcinoma. However, some cases of intraductal papillary mucinous tumors show invasive and rapid progression like ductal pancreas carcinoma and the prognosis of such patients is sometimes poor. In the current study, we carried out immunohistochemical staining of intraductal papillary mucinous tumor tissues for p53 and investigated whether positive staining indicates tumor malignancies and has a prognostic value for intraductal papillary mucinous tumors. METHODOLOGY: Nineteen (19) patients who underwent pancreatic resection under the diagnosis of intraductal papillary mucinous tumors at the Chiba University Hospital between April 1992 and December 1996 were studied. We performed immunohistochemical staining of p53 as well as of PCNA, Ki-67 and Bcl-2 using their respective antibodies. Pathological findings revealed that 9 cases were intraductal papillary adenoma, 9 were intraductal papillary adenocarcinoma, and one was invasive ductal papillary adenocarcinoma. RESULTS: p53 expression could only be detected in the 1 case with invasive ductal papillary adenocarcinoma. Significant association could not be found between histological features and immunohistochemical staining of PCNA, Ki-67 and Bcl-2. CONCLUSIONS: p53 protein expression could be detected after progression to invasive type of intraductal papillary mucinous tumors. The present results demonstrate that p53 expression might be an indicator of invasive progression in intraductal papillary mucinous tumors, and might represent a surgical indicator of intraductal papillary mucinous tumors.     

Pancreatoscopy assisted by endoscopic sphincter dilation

To investigate tumor extension to the main pancreatic duct in patients with intraductal papillary mucinous tumors, we conducted pancreatoscopy assisted by endoscopic sphincter dilation (ESD). Two patients with intraductal papillary mucinous tumors were included in this study. The endoscopic sphincter dilation was performed using an Olbert balloon-tipped catheter. A mother-baby scope system was used for endoscopic examination. After dilation of the orifice, the babyscope was inserted smoothly. Tumorous lesions (especially the typical salmon-roe appearance of the tumors) were clearly observed in the main pancreatic duct in one patient, while no tumorous lesions were identified in the other patient. The ESD procedure may be applicable for assisting insertion of the endoscope through the papilla of Vater into the pancreatic duct. ESD is useful both for treating and for diagnosing pancreatic lesions. Received: June 19, 2001 / Accepted: February 22, 2002 Reprint requests to: N. Ueno, Present address: Third Department of Internal Medicine, Yokohama City University School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama 236-0004, Japan     

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.     

Intraductal papillary mucinous tumors of the pancreas

Cystic neoplasms of the exocrine pancreas are a small fraction of pancreatic tumors. Within that group of cystic neoplasms, intraductal papillary mucinous tumors (IPMTs) can be distinguished from mucinous cystic neoplasms, serous cystic neoplasms, and pseudopapillary cystic tumors. Awareness of IPMTs has increased since the World Health Organization classified these tumors as its own group in 1996. Because of their favorable prognosis, an extensive diagnostic workup for IPMTs should be performed in patients presenting with cystic lesions of the pancreas. This workup often leads to the diagnosis and the predominant tumor location and size, although the extent of the ductal changes can only be established by histopathology. Surgical resection is the therapy of choice for IPMTs. The type of resection depends upon the extent of the quantitative and qualitative ductal involvement. Total pancreatectomy is currently the treatment for an IPMT that comprises the entire main duct.     

Analysis of small cystic lesions of the pancreas

Summary There have been few reports on (1) the nature and pathogenesis of small cystic lesions of the pancreas, (2) their incidence, age distribution, and location, and (3) their significance as potential precursors of intraductal papillary tumors, mucinous cystic tumors, and duct cell carcinomas. Materials: Epithelial growth of small cystic lesions in 300 consecutive autopsy cases and in seven cases of small duct cell carcinoma from among 2300 elderly autopsy cases, was evaluated by histopathological analysis. One hundred eighty-six cystic lesions were found in 73 of 300 autopsy cases (24.3%). The incidence of cystic lesions increased with age. Cystic lesions were equally distributed throughout the pancreas. Epithelial atypia was histologically classified into five groups: normal epithelium; papillary hyperplasia without atypia; atypical hyperplasia; carcinomain situ; and invasive carcinoma. The incidence of each group was 47.5, 32.8, 16.4, 3.4, and 0%, respectively. Epithelia of atypical hyperplasia or carcinomain situ were more prevalent in small cystic lesions (less than 4 mm in diameter) than in larger lesions (chi-square test,p<0.05). Epithelia of dilated ductular branches adjacent to cystic lesions showed a similar degree of atypia as the epithelia of the cystic lesions themselves (p<0.01). Epithelial atypia of the main pancreatic duct was mild in all of the cases but two, and was not related to that of the cystic lesion. Among the seven cases of small duct cell carcinoma, two cases had small cancerous cystic lesions, 4.1 and 5.3 mm in diameter, within the tumor. Small cystic lesions appear to have the potential to progress to malignancy but definitive evidence has not been demonstrated. Additional studies, including molecular biological examinations, are necessary to fully understand the biology of these lesions.     

Laparoscopic resection of synchronous intraductal papillary mucinous neoplasms: A case report

We describe herein a 68-year-old woman who was diagnosed with a quite rare entity of intraductal papillary mucinous neoplasms (IPMNs) occurring simultaneously in the left lateral lobe of liver and the tail of pancreas. Abdominal computed tomography and magnetic resonance cholangiopancreatography showed a cystic dilatation of the pancreatic duct in the pancreatic tail, which suggested an IPMN, and multiple intrahepatic duct stones in the left lateral lobe. The patient underwent a laparoscopic left lateral hepatolobectomy and spleen-preserving distal pancreatectomy. Intra-operative finding of massive mucin in the dilated bile duct implied an intraductal mucinous tumor in the liver. The diagnosis of synchronous IPMNs in the liver and pancreas was confirmed by pathological examination. The patient was followed up for 6 mo without signs of recurrence. Although several cases of IPMN of liver without any pancreatic association have been reported, the simultaneous occurrence of IPMNs in the liver and pancreas is very rare. To the best of our knowledge, it is the first reported case treated by laparoscopic resection.     

Precursors to pancreatic cancer

Infiltrating ductal adenocarcinoma of the pancreas is believed to arise from morphologically distinct noninvasive precursor lesions. These precursors include the intraductal papillary mucinous neoplasm, the mucinous cystic neoplasm, and pancreatic intraepithelial neoplasia. Intraductal papillary mucinous neoplasms are grossly visible mucin-producing epithelial neoplasms that arise in the main pancreatic duct or one of its branches. The cysts of mucinous cystic neoplasms do not communicate with the major pancreatic ducts, and these neoplasms are characterized by a distinct ovarian-type stroma. Pancreatic intraepithelial neoplasia is a microscopic lesion. This article focuses on the clinical significance of these three important precursor lesions, with emphasis on their clinical manifestations, detection, and treatment.     

Endoluminal ultrasonography for pancreatic diseases.

Endoluminal ultrasonography was performed on 146 patients with pancreatobiliary diseases by using high-frequency, thin ultrasonic probes, and the usefulness of the new technique in diagnosis of pancreatic diseases was reported. The ultrasound probe could be inserted into the main pancreatic duct in 43 of 46 patients (93.5%), and images of the lesions could be obtained in 42 patients (91.3%). Endoluminal ultrasonography revealed a hypoechoic mass with clear margins and central echogenicity in patients with pancreatic carcinoma. Endoluminal ultrasonography showed normal pancreatic parenchyma as a fine reticular pattern and did not reveal the tumors surrounding the stenosis in patients with focal pancreatitis. Endoluminal ultrasonography in patients with intraductal papillary adenocarcinoma of the pancreas revealed cystic lesions with mural nodules more than 4 mm, mucus echoes, and solid tumors with mixed echo patterns. There were no severe complications, and acute pancreatitis occurred in none of 46 patients, but high-level serum amylase after examination occurred in 5 patients (10.9%). Endoluminal ultrasonography is useful for differential diagnosis in patients with small pancreatic tumors or cystic lesions, especially intraductal papillary tumors of the pancreas. Endoluminal ultrasonography is recommended as a precise examination for the diagnosis of cystic lesions of the pancreas or stenosis of the main pancreatic duct after ERCP and EUS.     

Hemosuccus pancreaticus caused by a mucinous cystic neoplasm of the pancreas

Hemosuccus pancreaticus is a gastrointestinal hemorrhage through the main pancreatic duct. Here, we report a rare case of hemosuccus pancreaticus due to a mucinous cystic neoplasm of the pancreas. A 62-year-old woman who had been followed for a branch duct intraductal papillary mucinous neoplasm visited our emergency room due to severe abdominal pain and bloody discharge. Computed tomography revealed that the pancreatic cyst increased the tension of the wall and a high-density area indicative of bleeding into the cyst was observed. Endoscopy showed opening of and hemorrhaging from the papilla of Vater. The patient was diagnosed with hemosuccus pancreaticus caused by hemorrhaging into the cyst from the branch duct intraductal papillary mucinous neoplasm. Based on this diagnosis, elective distal pancreatectomy was performed. The histopathological diagnosis was a mucinous cystic neoplasm with intermediate-grade dysplasia based upon the pathological findings that fibrous ovarian-type stroma existed abundantly and the stroma cells were positive for progesterone receptor and inhibin. Hemosuccus pancreaticus caused by a mucinous cystic neoplasm is extremely rare and there has been only one case reported to date. In conclusion, it should be recognized that pancreatic cystic neoplasms including mucinous cystic neoplasms may cause hemosuccus pancreaticus.     

Tumeurs intracanalaires papillaires et mucineuses du pancréas découvertes fortuitement : à propos d’un cas

Intraductal papillary mucinous neoplasms of pancreas (IPMN) are cystic lesions of the pancreas than can be associated with extra-pancreatics malignancies. We report a case of intraductal papillary mucinous neoplasm discovered incidentally during the staging of rectal adenocarcinoma. This observation illustrates the high frequency of IPMN incidentally discovered, for instance during the work-up of an extrapancreatic tumor.     

A resected case of multiple intraductal papillary mucinous tumors of the pancreas with US-guided ductal branch-oriented partial pancreatectomy.

A 69-year-old man with epigastralgia was admitted on August 26, 2002 and diagnosed with multiple intraductal papillary mucinous tumors by various imagings. The cystic tumor of pancreas head had a diameter of 2 cm, and the mural nodule of the cystic tumor measured only 3 mm. In the pancreas body the cystic tumor was measured at 1.5 cm with the mural nodule of the cystic tumor measuring 3 mm. It was believed that the tumors were benign. However, a mural nodule of the cystic lesion was recognized, thus, the possibility of malignancy could not be completely ruled out. The reduction operation for preservation of pancreatic parenchyma should be selected for these circumstances. Ductal branch-oriented partial pancreatectomy was performed on September 6, 2002 with intraoperative ultrasonography and a Cavitron Ultrasonic Aspirator, preserving the main pancreatic duct and normal pancreatic parenchyma. The operation was successful, and the histopathological diagnosis of the tumors was intraductal papillary adenoma of the pancreas.     

Intrahepatic biliary papilloma morphologically similar to biliary cystadenoma

A 37-year-old man presented complaining of epigastralgia. Abdominal ultrasonography revealed the presence of a papillary tumor (9 mm in diameter) in the cystic lesion (18 mm in diameter) in hepatic segment 4, which was accompanied by mild intrahepatic bile duct dilatation. Although abdominal computed tomography also showed the cystic lesion, it did not show papillary tumors inside the lesion. Endoscopic retrograde cholangiography showed the communication between the cystic lesion and the left hepatic duct. In addition, mucus was observed in the common bile duct. When transpapillary intraductal ultrasonography was performed through the left hepatic duct using a fine ultrasonic probe, a hyperechoic papillary and lobulated tumor was clearly shown in the cystic lesion. The wall of the cyst was smooth and there was no sign of tumor infiltration. Based on these findings, biliary cystadenoma was diagnosed and an extended left lobectomy was carried out. However, pathological findings postoperatively revealed that the lesion was a localized biliary papilloma, developing and extending to the intrahepatic duct. This case is rare and there have been no published reports describing a biliary papilloma morphologically similar to biliary cystadenoma.     

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.