Gd-EOB-DTPA-enhanced magnetic resonance imaging for bile duct intraductal papillary mucinous neoplasms

AIM: To investigate gadolinium-ethoxybenzyl-diethylenetriamine-pentaacetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging (MRI) of intraductal papillary mucinous neoplasms of the bile duct (IPMN-B).METHODS: The imaging findings of five cases of IPMN-B which were pathologically confirmed at our hospital between March 2012 and May 2013 were retrospectively analyzed. Three of these cases were diagnosed by duodenal endoscopy and biopsy pathology, and two cases were diagnosed by surgical pathology. All five patients underwent enhanced and non-enhanced computed tomography (CT), magnetic resonance cholangiopancreatography, and Gd-EOB-DTPA-enhanced MRI; one case underwent both Gd-EOB-DTPA-enhanced MRI and positron emission tomography-CT. The clinical data and imaging results for these cases were compared and are presented.RESULTS: Conventional imaging showed diffuse dilatation of bile ducts and multiple intraductal polypoid and papillary neoplasms or serrated changes along the bile ducts. In two cases, Gd-EOB-DTPA-enhanced MRI revealed dilated biliary ducts and intraductal tumors, as well as filling defects caused by mucin in the dilated bile ducts in the hepatobiliary phase. Gd-EOB-DTPA-enhanced MRI in one case clearly showed a low-signal tumor in the hepatobiliary phase, similar to what was seen by positron emission tomography-CT. In two patients, routine inspection was unable to discern whether the lesions were inflammation or tumors. However, Gd-EOB-DTPA-enhanced MRI revealed a pattern of gradual enhancement during the hepatobiliary phase, and the signal intensity of the lesions was lower than the surrounding liver parenchyma, suggesting tissue inflammation in both cases, which were confirmed by surgical pathology.CONCLUSION: Gd-EOB-DTPA-enhanced MRI reveals the intraductal mucin component of IPMN-B in some cases and the extent of tumor infiltration beyond the bile ducts in invasive cases.     

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.     

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.     

Granular Cell Tumor: Unusual Cause for Mucocele of Gallbladder

A small granular cell tumor of the cystic bile duct is presented. A review of the literature revealed that all previously reported granular cell tumors in the biliary ducts affected women. Among them, 13 cystic bile duct tumors were exclusively small and slow-growing benign lesions. All had been operated on for gallstone disease, but in 10 cases no stone was actually found. Although such a small tumor might be easily overlooked grossly, histological diagnosis is relatively easy if differentiated from a reactive smooth muscle granular lesion.     

Multiple hepatic peribiliary cysts with cirrhosis

Multiple hepatic peribiliary cysts were found in three autopsy cases of patients who had had underlying liver diseases and obstructive jaundice. Macroscopically, the cysts were visible and present exclusively in the hepatic hilum and larger portal tracts. Histologically, the cysts were of varying size and were lined by a single layer of cuboidal or flattened epithelial cells without atypia. Intimate association between the cysts and peribiliary glands was found in the walls of large bile ducts. All three cases were associated with liver cirrhosis in patients with portal hypertension, and two of the patients had also had hepatocellular carcinoma. These findings support the previous assumption that multiple hepatic peribiliary cysts may be closely related to a portal hypertensive condition. Although peribiliary cysts have been considered to be clinically asymptomatic in general, in one of our patients, the cystic dilatation appeared to have been responsible for the progression of obstructive jaundice.     

先天性胆管扩张症合并肝内胆管扩张的手术治疗对策

目的探讨先天性胆管扩张症合并肝内胆管扩张的手术治疗对策,以提高手术的远期疗效。方法第1组不管是否提示合并肝内胆管扩张,均单纯行扩张胆总管、胆囊切除,肝总管空肠Roux—en—Y吻合。第2组在第1组手术术式基础上,对胆管狭窄者行胆管成形术和冲洗处理胰胆管共同通道。采用时序分组进行对比分析研究。结果第1组13例,6例合并肝内胆管扩张,4例为囊样扩张。第2组共46例,18例有肝内胆管扩张,其中11例为囊样扩张,同时存在胆管狭窄。第2组术后腹痛、胆道感染的发生率明显低于第1组。讨论在标准根治术(囊肿切除胆道重建)的基础上对近端肝内胆管狭窄矫治、可能存在的复杂胆道畸形的处理及远端胰胆管共同通道冲洗,可有效降低术后胆道胰管并发症的发生,提高手术远期疗效。     

Bile-duct destruction and collagen deposition: a prominent ultrastructural feature of the liver in cystic fibrosis.

To study the liver disease of patients with cystic fibrosis, percutaneous liver biopsies were performed in 10 patients with cystic fibrosis aged 6 to 22 yr. Nine of 10 patients had high Shwachman scores, eight had normal serum levels of transaminases. Light-microscopical examination showed steatosis in seven cases and in five slight or moderate inflammatory infiltration. Eight patients showed varying degrees of fibrosis and even cirrhosis. Six patients had bile-duct proliferation and, in one case a bile plug was found. Other signs of cholestasis were not seen. Electron-microscopical investigation showed no specific signs of cholestasis such as ductal plugs or intracellular bile pigments. The canaliculi were not dilated, except in one case. Most patients had bile-duct cells with irregular shapes, protruding into the lumen, and some cases even had necrotic cells. Around the bile ducts and ductules, collagen was deposited and fat-storing cells were a common finding. Our findings do not support the view that cholestasis is the pathogenetic factor in liver disease in cystic fibrosis. A cytotoxic influence on the biliary cells, stimulating collagen deposition, seems more likely.     

Solitary Cystic Dilation of the Intrahepatic Bile Duct: Morphology of Two Autopsy Cases and a Review of the Literature

Solitary cystic dilation of intrahepatic bile ducts with neither extrahepatic biliary anomalies nor renal lesions has been reported previously in only 11 cases. We report two cases which were found as a result of postmortem cholangiography of 149 livers at autopsy. Case 1 had a cystic dilation measuring 1.2 cm in diameter in the left lateral superior area duct. Case 2 showed a cystic dilation measuring 1.0 cm in diameter in the left lateral segment duct. Histologically, the walls of the dilated ducts consisted of fibrous wall with proliferation of periductal mucous glands in both cases. The dilated and adjacent bile ducts in case 2 also contained tiny brown pigment stones and biliary sludge, suggesting that this bile duct lesion is important in the formation of intrahepatic calculi by providing a site for bile stasis and mucous hypersecretion.     

A new method of preventing bile duct injury in laparoscopic cholecystectomy

AIM: Of all the complications of laparoscopic cholectecystomy, bile duct injury (BDI) is the most serious complication. The prevention of injury to the common bile duct (CBD) remains a significant concern in laparoscopic cholecystectomy (LC). Different kinds of methods have been advanced to avoid this injury but no single method has gained wide acceptance. Because of various limitations of current methodologies we began a study using cold light illumination of the extrahepatic biliary system (light cholangiography LCP) to better visualize this area and thereby reduce the risk of bile duct injury. METHODS: Thirty-six patients with cholelithiasis were divided into two groups. Group I (16 cases) received LCP and group II (20 cases) received methelenum coeruleum cholangiography (MCCP). In group I cold light was used to illuminate the common bile duct by leading an optical fiber into the common duct with a duodenoscope at the time of LC. The light coming from the fiber in the CBD could clearly illuminate the location of CBD and hepatic duct establishing its location relative to the cystic duct. This method was compared with the dye injection technique using methelenum coeruleum. RESULTS: In group I thirteen cases were successfully illuminated and three failed. The cause of three failed cases was due to the difficulty in inserting the fiber into the ampulla of Vater. No complications occurred in the thirteen successful cases. In each of these successful cases the location of the common and hepatic ducts was clearly seen differentiating the ductal system from surrounding anatomy. In ten cases both the left and right hepatic ducts could be seen and in three only the right hepatic ducts were seen. In four of the thirteen cases, cystic ducts were also seen. In group II, eighteen of the twenty cases were successful. The location of extrahepatic ducts became blue differentiating the ductal system from surrounding anatomy. Two cases failed due to a stone obstructing the cystic duct, and extravisation of the dye turned the entire area blue. LCP showed the common and hepatic ducts more clearly than MCCP. CONCLUSION: LCP is the only technique that can clearly and directly show the location of the extrahepatic biliary system and may be useful in selecting cases of uncertain anatomy in the prevention of bile duct injury.     

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.     

Diagnostic dilemmas in patients with cystic neoplasms of the pancreas

Cystic neoplasms of the pancreas (CNP) are rare lesions that can be difficult to diagnose preoperatively. Twenty patients with cystic neoplasms of the pancreas including five microcystic adenomas, six benign mucinous cystic neoplasms, three malignant mucinous cystic neoplasms, two solid and papillary epithelial neoplasms, and four cystic neuroendocrine tumors were treated at a single institution between 1962 and 1987. The average duration of symptoms prior to diagnosis was 10 months. Five patients were asymptomatic. Forty percent of patients presented with an abdominal mass. Plain abdominal x-rays and UGI barium contrast studies were never diagnostic. Ultrasonography, computerized tomography (CT) and visceral angiography aided in the correct diagnosis in 28%, 36%, and 75% of patients studied, respectively. Overall a correct diagnosis was made preoperatively in only 35% of patients. Twelve of 13 patients were correctly diagnosed at laparotomy with intraoperative biopsy. Without biopsy the mass was misdiagnosed at laparotomy in five of six cases. CNP must be suspected in any patients who present with an upper abdominal mass with or without abdominal pain and no history of pancreatitis. CT may be diagnostic in up to one third of cases and should be obtained routinely to demonstrate the proximity of the lesion to other structures. Visceral angiography should also be obtained prior to operation. A generous incisional biopsy should be obtained of all pancreatic cysts that are not to be resected.     

Multiple hilar cysts of the liver in patients with alcoholic cirrhosis: Report of three cases

The present report concerns the clinicopathological study of three patients with alcoholic cirrhosis (a 40-year-old man, a 52-year-old woman and a 48-year-old man) who had multiple cysts along their intrahepatic bile ducts. The cysts were visible on gross examination of the liver and, in two cases, an enlargement of the cysts had been detected in abdominal computed tomography scans performed 1 year apart. Histologically, the cysts consisted of proliferating and dilated peribiliary glands. The cysts occasionally compressed the original bile ducts. The latter showed mucosal hyperplasia with antral-type gland metaplasia. Neoplastic changes and necroinflammation were not seen. Immuno-histochemical assays revealed that the peribiliary glands and antral-type glands expressed not only cytokeratin and carbohydrate antigen 19-9, but also c-MET protein, the hepatocyte growth factor receptor which may be related, at least in part, to the cystic dilatation of the peribiliary glands.     

DUPLICATE GALLBLADDER DIAGNOSED WITH ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY AND TREATED WITH LAPAROSCOPIC CHOLECYSTECTOMY

A 44‐year‐old man was admitted to hospital because of sudden severe postprandial right epigastric pain. Abdominal echography showed multiple calculi in the gallbladder and evidence of a probable septum at the neck. Based on the results, tentative diagnoses were made of gallbladder calculosis with associated gallbladder curvature or malformation, and gallbladder adenomyomatosis. Preoperative endoscopic retrograde cholangiopancreatography (ERCP) revealed a duplicate gallbladder consisting of two cystic ducts with two connected gallbladders. Laparoscopic cholecystectomy (Lap‐C) was performed. Inflammation of the Calot's triangle was mild. The two cystic ducts with one common bile duct were identified, and the cystic ducts separated by individual clipping. Duplicate gallbladder, a malformation of the gallbladder, has been reported to occur at an incidence of 0.02%. To date, a total of 60 cases have been reported in Japan, and recent cases have been treated with Lap‐C. In the present case, the surgery could be safely performed because the bifurcation of the cystic ducts could be identified by preoperative ERCP.     

Primary cholesterol hepatolithiasis

Stones extracted from patients with hepatolithiasis were analysed by infrared spectrophotometry. Cholesterol stones containing 70% or more cholesterol were found in 12 out of 55 cases. Judging from the lodging site of the stones, the degree of dilatation of the cystic duct, and the presence of cholecystitis, five of the cases were considered to be cholesterol stones produced in the liver. Two out of the five cases were a 44-year-old female and a 46-year-old female, respectively, with normal bifurcation of intrahepatic ducts, and stones were found in the lateral branches of dilated cystic bile ducts. The other three were 2 males and 1 female with an average age of 33. In these cases, the posterior descending branch bifurcated from the left hepatic duct, and stones were lodged in the dilated bile ducts distal to the junction of the left hepatic duct and the posterior descending branch. It is our conclusion that at least the former two were cases of “primary cholesterol hepatolithiasis” in view of the shape of the stones conforming to the hepatic duct, their easy morcellation, and the high cholesterol contents.     

Clinicopathologic review of 41 cases of pancreatic mucinous cystic neoplasms]

BACKGROUND/AIMS: Intraductal papillary mucinous neoplasms (IPMN) and mucinous cystic neoplasms are included in mucin-producing pancreatic tumors. The reports about IPMN are not uncommon but those about the mucinous cystic neoplasms are relatively few. The aims of this study were to define the natural history of resected mucinous cystic neoplasms of the pancreas and to identify the findings which suggest malignancy. METHODS: The authors retrospectively evaluated the clinical outcomes of 41 patients with mucinous cystic neoplasms who were surgically resected at Asan Medical Center between 1995 and 2004. RESULTS: Women (n=33) were more frequently affected than men (n=8). Thirty three patients (80.6%) had adenoma, 1 (2.4%) borderline malignancy, 1 (2.4%) carcinoma in situ, and 6 (14.6%) invasive mucinous cystadenocarcinoma. The most frequent symptom was abdominal pain (39%). About half of the enrolled patients were asymptomatic. Unilocular type (79%) was more frequent than the multilocular type (21%) on gross morphology. The tumor size of invasive mucinous cystic neopolasms was larger than that of non-invasive mucinous cystic neoplalsms (p=0.01). Abdominal pain was more frequent in invasive mucinous cystic neoplasms (p=0.026). On gross morphology, mural nodules were detected in 4 of 6 patients with invasive mucinous cystic neoplasms. However, they were not detected in any patients with non-invasive mucinous cystic neoplasms. Recurrence developed in none of the 35 patients with non-invasive mucinous cystic neoplasms, however 2 of the 6 patients with invasive mucinous cystic neoplasms died within 5 years. CONCLUSIONS: Clinical predictors of invasive mucinous cystic neoplasms are suggested to be tumor size and abdominal pain. The prognosis of the non-invasive mucinous cystic neoplasms is excellent when curative resection is performed.     

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.     

Primary cholesterol hepatolithiasis

Stones extracted from patients with hepatolithiasis were analysed by infrared spectrophotometry. Cholesterol stones containing 70% or more cholesterol were found in 12 out of 55 cases. Judging from the lodging site of the stones, the degree of dilatation of the cystic duct, and the presence of cholecystitis, five of the cases were considered to be cholesterol stones produced in the liver. Two out of the five cases were a 44-year-old female and a 46-year-old female, respectively, with normal bifurcation of intrahepatic ducts, and stones were found in the lateral branches of dilated cystic bile ducts. The other three were 2 males and 1 female with an average age of 33. In these cases, the posterior descending branch bifurcated from the left hepatic duct, and stones were lodged in the dilated bile ducts distal to the junction of the left hepatic duct and the posterior descending branch. It is our conclusion that at least the former two were cases of "primary cholesterol hepatolithiasis" in view of the shape of the stones conforming to the hepatic duct, their easy morcellation, and the high cholesterol contents.     

Clinicopathological features of "intraductal papillary neoplasm of the bile duct" and patient outcome after surgical resection

BACKGROUND/AIMS: Intraductal papillary neoplasm of the bile duct (IPNB) represents a biliary papillary tumor mainly growing in the bile duct lumen resembling intraductal papillary mucin-producing neoplasm of the pancreas. However, its clinical spectrum and characteristics have not been fully evaluated. METHODOLOGY: To define the clinicopathologic characteristics and prognosis of IPNB patients, 6 cases of IPNB who underwent surgical resection are presented. RESULTS: Patients were 3 males and 3 females, aged between 47 and 79 years. Five patients had histories of hepatobiliary disease. Imagery showed cystic or diffuse dilatation of the bile ducts. Tumor markers were not valuable for diagnosis. All patients underwent hemihepatectomy with or without resection of the caudate lobe or extrahepatic bile duct. Examination showed polypoid tumors in 5 cases though 1 case had no evident tumor. Mucin was observed in 3 cases. Five cases were well-differentiated adenocarcinoma and 1 had poorly differentiated adenocarcinoma. Vascular invasion was rare and lymph node metastasis was not observed. In-situ spread of carcinoma was seen along biliary mucosa in 3 cases. Five cases survived without tumor relapse for long periods but 1 died of tumor recurrence at 31 months. CONCLUSIONS: Complete resection of IPNB based on accurate preoperative assessment of tumor extension provides a good prognosis.     

Diagnostic dilemmas in patients with cystic neoplasms of the pancreas

Summary Cystic neoplasms of the pancreas (CNP) are rare lesions that can be difficult to diagnose preoperatively. Twenty patients with cystic neoplasms of the pancreas including five microcystic adenomas, six benign mucinous cystic neoplasms, three malignant mucinous cystic neoplasms, two solid and papillary epithelial neoplasms, and four cystic neuroendocrine tumors were treated at a single institution between 1962 and 1987. The average duration of symptoms prior to diagnosis was 10 months. Five patients were asymptomatic. Forty percent of patients presented with an abdominal mass. Plain abdominal x-rays and UGI barium contrast studies were never diagnostic. Ultrasonography, computerized tomography (CT) and visceral angiography aided in the correct diagnosis in 28%, 36%, and 75% of patients studied, respectively. Overall a correct diagnosis was made preoperatively in only 35% of patients. Twelve of 13 patients were correctly diagnosed at laparotomy with intraoperative biopsy. Without biopsy the mass was misdiagnosed at laparotomy in five of six cases. CNP must be suspected inany patients who present with an upper abdominal mass with or without abdominal pain and no history of pancreatitis. CT may be diagnostic in up to one third of cases and should be obtained routinely to demonstrate the proximity of the lesion to other structures. Visceral angiography should also be obtained prior to operation. A generous incisional biopsy should be obtained of all pancreatic cysts that are not to be resected.