A Case of Biliary Cystadenocarcinoma Followed up for Five Years

We report on a case of an 80-year-old male with autopsy-confirmed biliary cystadenocarcinoma. The tumor's growth was followed up for five years. CT findings on first admission revealed that the cyst walls of the tumor were smooth. However, a CT taken four years later showed large cysts with irregular walls which were growing invasively and expanding beyond the liver. The patient died of liver dysfunction caused by obstructive jaundice due to the liver tumor, and an autopsy was performed. Histologic examination of the autopsy material revealed a multilocular tumor that was identified as being a biliary cystadenocarcinoma and microscopic lung metastases were seen. The character of the multilocular cyst with septations covered by papillary proliferation of atypical columnar epithelium and many remaining portions with low grade dysplasia led us to suspect the tumor was derived from a cystadenoma.     

Extrahepatic biliary cystadenocarcinoma arising from the left hepatic duct

A 54-year-old man, who had no clinical symptoms, underwent a routine health checkup at our hospital. Abdominal ultrasonography disclosed a well demarcated tumor containing a solid portion occupying the dilated left hepatic duct and a cystic portion expanding into the parenchyma of the left hepatic lobe, with mild dilatation of the intrahepatic bile ducts. These findings were later confirmed by computed tomography (CT) and magnetic resonance imaging. Endoscopic retrograde cholangiography revealed a complete defect at the level of the left hepatic duct, while drip infusion cholangiographic-CT (DIC-CT) disclosed a defect of the left hepatic duct only, with the distal portions of the left intrahepatic ducts being visualized on the image. Hepatic angiography revealed light stains in the solid portion in the parenchymal phase. At left lobectomy, a multiloculated polyp-like tumor was found arising from the left hepatic duct and expanding into the parenchyma of the left hepatic lobe. Microscopically, all the lining cells in the cysts and the tumor cells in the solid portion showed the features of papillary adenocarcinoma. In this patient with extrahepatic biliary cystadenocarcinoma, DIC-CT was useful in identifying the site of origin of the tumor, and hepatic angiography was also useful in differentiating this rare malignant tumor from benign cystadenoma. (Received July 11, 1997; accepted May 22, 1998)     

Multicystic biliary hamartoma with extremely elevated CA19-9: a case report

Multicystic biliary hamartoma (MCBH) is a rare cystic disease of the liver. We herein report a case of MCBH associated with extremely elevated levels of serum carbohydrate antigen (CA) 19-9. A 53-year-old man was referred to our hospital because of extremely elevated CA19-9 levels (more than 12,000?U/mL). Enhanced abdominal computed tomography and magnetic resonance imaging (MRI) revealed a multicystic tumor with a calcified wall in the left lobe of the liver, although no apparent intracystic nodule was detected. Because of the possibility of a malignant tumor, such as intraductal papillary neoplasm of the bile duct or cystadenocarcinoma, the patient underwent left hepatectomy. Based on the postoperative pathological findings, the lesion was diagnosed as MCBH. The serum CA19-9 level drastically decreased after surgery. We encountered a rare case of MCBH with extremely elevated CA19-9 levels.     

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.     

Hepatobiliary cystadenocarcinoma connected to the hepatic duct: a case report and review of the literature

We present a rare case of hepatobiliary cystadenocarcinoma with biliary communication. A 74-year-old woman had a liver cyst that had enlarged from 5 cm to 8 cm in diameter over the last 2 years. A mural nodule, 1 cm in diameter, was first demonstrated by computed tomography in a multilocular cyst in segment IV. The nodule showed hypervascularity at angiography and computed tomography during arteriography. Percutaneous transhepatic cystography demonstrated a communication between the cyst and the biliary tract. The cyst was filled with mucinous and gelatinous fluid and was revealed to contain cancer cells. The patient underwent total tumor extirpation with the surrounding hepatic parenchyma. We confirmed and closed the biliary fistula connected to the right hepatic duct. Histologically, the cyst wall was composed of cystadenoma and the mural nodule showed in situ papillary adenocarcinoma. The patient is doing well 9 months after surgery. Complete tumor extirpation and closing of the biliary fistula is the treatment of choice.     

Primary biliary cystadenocarcinoma perforating the duodenum and left intrahepatic biliary tree -mimicking a hydatid cyst

Abstract: We report the case of a 76-year-old woman with biliary cystadenocarcinoma perforating the left biliary tree and exhibiting intra-tumoral gas bubbles resulting from invasion of the duodenum. The clinical history included subfebrile temperatures of 3 months duration, and pains associated with an abdominal mass in the right upper quadrant. Blood tests showed leucocytosis, and radiological studies revealed the features of a partially calcified septated tumor with nodular components combined with multiple gas-fluid levels, mimicking an infected hydatid cyst. Intraoperative ultrasonography, cholangiography and frozen section histology were necessary to prove the malignant nature of this cystic tumor. Provided that complete resection with strict adherence to oncological precepts is possible, the prognosis of cystadenocarcinoma is better than in hepatocellular or cholangiocellular carcinoma.     

Preoperative differential diagnosis between intrahepatic biliary cystadenoma and cystadenocarcinoma: A single-center experience

AIM: To investigate preoperative differential diagnoses made between intrahepatic biliary cystadenoma and intrahepatic biliary cystadenocarcinoma.METHODS: A retrospective analysis of patient data was performed, which included 21 cases of intrahepatic biliary cystadenoma and 25 cases of intrahepatic biliary cystadenocarcinoma diagnosed between April 2003 and April 2013 at the General Hospital of PLA. Potential patients were excluded whose diagnoses were not confirmed pathologically. Basic information (including patient age and gender), clinical manifestation, duration of symptoms, serum assay results (including tumor markers and the results of liver function tests), radiological features and pathological results were collected. All patients were followed up.RESULTS: Preoperative levels of cancer antigen 125 (12.51 ± 9.31 vs 23.20 ± 21.86, P < 0.05) and carbohydrate antigen 19-9 (22.56 ± 26.30 vs 72.55 ± 115.99, P < 0.05) were higher in the cystadenocarcinoma subgroup than in the cystadenoma subgroup. There were no statistically significant differences in age or gender between the two groups, or in pre- or post-operative levels of alanine aminotransferase, aspartate aminotransferase, total bilirubin (TBIL), and direct bilirubin (DBIL) between the two groups. However, eight of the 21 patients with cystadenoma and six of the 25 patients with cystadenocarcinoma had elevated levels of TBIL and DBIL. There were three cases in the cystadenoma subgroup and six cases in the cystadenocarcinoma subgroup with postoperative complications.CONCLUSION: Preoperative differential diagnosis relies on the integration of information, including clinical symptoms, laboratory findings and imaging results.     

Mucinous cystadenocarcinoma of the colon

A rare case of mucinous cystadenocarcinoma arising in the sigmoid colon, found accidentally during an operation for cholecystolithiasis, is reported. The tumor was located 40 cm from the anal verge, and had two histologic variations consisting of a large distended cystic lesion and branching cystic channels with papillary proliferation of the epithelium. Serial sections revealed the existence of a luminal communication between the two lesions. The tumor tissue was seen mainly in the muscularis propria with no mucosal involvement. The papillary portion had a highly differentiated appearance, giving rise to considerable difficulty in determining whether it was benign or malignant. In a localized area, however, the tumor invaded into the subserosa and showed distinctive atypical changes. The tumor cells showed intense reactivity for carcinoembryonic antigen. This mucinous cystadenocarcinoma was considered to be originated from an enterogenous cyst, a possible derivative of duplication of the colon. The differential diagnosis concerning this rare tumor is also discussed.     

Biliary cystadenocarcinoma associated with atrophy of the left hepatic lobe and hepatolithiasis mimicking intrahepatic cholangiocarcinoma: a case report

Biliary cystadenocarcinoma and its benign counterpart, biliary cystadenoma, are rare hepatic cystic tumors arising from the hepatobiliary epithelium. We report the case of a 68-year-old Taiwanese woman who presented initially with acute cholangitis. A series of imaging studies including abdominal ultrasound, computerized tomography, endoscopic retrograde cholangiopancreatography, and percutaneous transhepatic cholangiography showed bilateral intrahepatic duct (IHD) and common bile duct (CBD) stones with IHD and CBD dilatation, and an ill-defined tumor within the atrophied left hepatic lobe. The patient underwent surgical resection of the tumor and choledocholithotomy. The pathologic diagnosis was biliary cystadenocarcinoma. We review this rare disease entity and discuss its unusual radiologic features mimicking intrahepatic cholangiocarcinoma.     

Early Detection of High-grade Biliary Intraepithelial Neoplasia (BilIN-3) in the Cystic Duct Visualized by SpyGlass DS Cholangioscopy

An 84-year-old man was admitted with epigastralgia. Computed tomography showed contrast-enhanced wall thickness in the cystic duct. An endoscopic examination revealed short irregular stricture in the cystic duct, and per-oral cholangioscopy revealed a reddish papillary tumor at the stricture site. Surgical resection revealed high-grade biliary intraepithelial neoplasia (BilIN) at the stricture site of the cystic duct. To our knowledge, this is the first case of a solitary high-grade BilIN epithelium in the cystic duct detected by per-oral cholangioscopy.     

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.     

A case of a mucin-producing bile duct tumor diagnosed over the course of 6 years

We report a case of a mucin-producing intraductal papillary neoplasm of the intrahepatic bile duct (M-IPNB) diagnosed over a period of 6 years. A 64-year-old man underwent follow-up evaluations for an abdominal aortic aneurysm at our hospital. In 2009, a computed tomography (CT) scan revealed a simple hepatic cyst in segment 3 of the liver. Annual CT scans initially showed almost no change in the size or shape of the cyst. The cystic lesion, which measured 5 cm in 2014, had increased to 11 cm by 2015, and a solid component was detected within the cyst. A biliary cystic tumor was suspected and we performed a left lateral hepatectomy. Pathological examination showed that the papillary lesion in the cyst included adenocarcinoma and adenoma components. We diagnosed M-IPNB in 2015. Identification of the solid component of the cyst, as well as an increase in cyst diameter in the image analyses, was critical for diagnosis of M-IPNB.     

Intracystic hemorrhage of a simple liver cyst mimicking a biliary cystadenocarcinoma

Simple liver cysts are rarely complicated by intracystic hemorrhage. We encountered a case of simple liver cyst that was morphologically similar to biliary cystadenocarcinoma, which was complicated by asymptomatic intracystic hemorrhage and successfully treated by right lobectomy. A large cystic lesion of the liver was detected in a 57-year-old woman during a mass screening health check. Abdominal ultrasonography (US) revealed that the cystic lesion, containing many hyperechoic papillary structures, occupied almost the entire region of the right hepatic lobe. In addition, a round mural nodule, measuring approximately 5 cm in diameter, was detected in the cystic wall. Abdominal computed tomography (CT) revealed that the inner part of the cystic lesion showed homogeneous low density, but CT did not show the round nodule detected by US. On T1-weighted sequence of magnetic resonance imaging (MRI), the lesion showed homogeneous high signals, together with a low-signal tumorous lesion in the cystic wall. T2-weighted sequence of MRI showed unhomogeneous high signals, together with high signals in the tumorous part. These findings did not exclude the possibility of a malignant cystic tumor, such as biliary cystadenocarcinoma. Therefore, right lobectomy was performed. Histological examinations of resected tissue specimens revealed that the lesion was a liver cyst containing a large amount of blood clot, and that the tumorous lesion detected by US and MRI was a large mass of blood clot which was partly liquefied. This case indicates the diagnostic importance of the morphological discordance between CT and US or MRI findings for liver cyst containing a large amount of blood clot. Received: May 7, 2001 / Accepted: December 27, 2001 Reprint requests to: Y. Kitajima     

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.     

Mucinous cystadenoma of the pancreas 17 years after excision of gallbladder because of a choledochal cyst

A 56-year-old woman who had undergone excision of the gallbladder because of a choledochal cyst had a tumorous lesion of the pancreas identified by upper abdominal ultrasonography, but an operation was not carried out, because there was no apparent increase in the cystic mass and no elevation of serum tumor markers. In October 2001, she was admitted to our hospital to check for malignancy because of elevated levels of the tumor marker Dupan-2. Abdominal enhanced computed tomography and upper abdominal ultrasonography revealed a large multilocular cystic mass in the body to tail of the pancreas. Endoscopic retrograde cholangiopancreatography showed elongation of the common duct that communicates with the common bile duct and the main pancreatic duct, indicating an anomalous arrangement of the biliary and pancreatic duct system. No apparent communications between the cystic mass and the main pancreatic duct were observed. In January 2002, the patient underwent a spleen-preserving distal pancreatectomy, and histopathological and immunohistochemical examinations led to the diagnosis of pancreatic mucinous cystadenoma with ovarian-like stroma. The mucinous cystadenoma was detected 17 years after the operation for the choledochal cyst. To the best of our knowledge, no documented case reports of mucinous cystadenoma of the pancreas associated with a choledocal cyst have been reported to date. We present here the first case report of pancreatic mucinous cystadenoma occurring in the body to tail of the pancreas, associated with a choledocal cyst.     

Cystic tumors of the liver： A practical approach

Biliary cyst tumors （cystadenoma and cystadenocarcinoma） are an indication for liver resection. They account for only 5% of all solitary cystic lesions of the liver, but differential diagnosis with multiloculated or complicated biliary cysts, atypical hemangiomas, hamartomas and lymphangiomas may be difficult. The most frequent challenge is to differentiate biliary cyst tumors from hemorrhagic cysts. Computerized tomography （CT） and magnetic resonance imaging （MRI） are often not diagnostic and in these cases fine needle aspiration （FNA） is used to confirm the presence of atypical biliary cells. FNA, however, lacks adequate sensitivity and specificity and should always be used in conjunction with imaging. Pre-operative differentiation of cystadenoma from cystadenocarcinoma is impossible and surgery must be performed if a biliary cyst tumor is suspected. When multiple cystic lesions are observed throughout the liver parenchyma, it is important to exclude liver metastasis, of which colonic cancer is the most common primary site. Multiple biliary hamartomas （von Meyenburg complex） can appear as a mixture of solid and cystic lesions and can be confused with cystic metastasis. Strong and uniform T2 hyperintensity on MRI is usually diagnostic, but occasionally a percutaneous biopsy may be required.     

Clinical features and imaging diagnosis of biliary cystadenocarcinoma of the liver

Biliary cystadenocarcinoma of the liver is a relatively rare disease. Herein, we reported a case of biliary cystadenocarcinoma with a review of the literature. A 71-year-old female was admitted with the chief complaint of epigastralgia. The imaging studies revealed a biliary cystadenocarcinoma in the left hepatic lobe with suspicion of direct invasion to the left and middle hepatic veins and inferior vena cava. However, there was no direct invasion of the tumor to these veins in operation findings, and an extended left hepatic resection was performed without resection of inferior vena cava. The tumor was histologically diagnosed as biliary cystadenocarcinoma of the liver. Diagnosis of biliary cystadenocarcinoma is usually difficult preoperatively, however, a diagnosis was possible with the use of imaging studies. It was suggested that this tumor originated from a benign cystadenoma because of the existence of a transitional zone between normal cells and atypical cells in the cystic wall. Systematic hepatectomy was recommended as the initial treatment in consideration of the features of cystadenocarcinoma.     

Intraductal oncocytic papillary neoplasm of the bile duct: the first case of peribiliary gland origin

We report herein the first case of intraductal oncocytic papillary neoplasm of the bile duct arising from a peribiliary gland of the left hepatic duct. The patient was a 63-year-old Japanese man. Radiological and cholangioscopic examinations revealed intraductal tumor of the left hepatic duct. After pathological diagnosis of adenocarcinoma by cholangioscopic biopsy, a surgical hepatobiliary resection was performed. Pathological examination revealed papillary tumor in the left hepatic duct. Histologically, the tumor was identified as papillary neoplasm comprising oncocytic cells and delicate fibrovascular cores. Interestingly, this tumor originated from the cystic space in the bile duct wall. This cystic space was histologically identified as a cystically dilated peribiliary gland. Carcinoma in situ was observed in this cystic peribiliary gland at the bottom of the tumor, but not on any areas of biliary epithelium. This case suggests that intraductal papillary neoplasm can arise from both biliary epithelium and peribiliary glands.     

Contrast enhanced ultrasound features of hepatic cystadenoma and hepatic cystadenocarcinoma

Objective: Hepatic (biliary) cystic tumor (HBCT) is a rare focal cystic liver lesion, which has been rarely described in the literature. In our current multicenter, retrospective study, we aimed to analyze contrast enhanced ultrasound (CEUS) features and its diagnostic performance in histologically proved HBCT.

Material and methods: Twenty-three patients with single HBCT were retrospectively analyzed. Histologically, 17 (73.9%) were benign hepatic (biliary) cystadenoma (HBCA), 6 (26.1%) were hepatic (biliary) cystadenocarcinoma (HBCAC). All CEUS examinations were assessed by two independent radiologists in consensus. Criteria of CEUS imaging evaluation included the contrast enhancement pattern of lesion (hypoenhancing, hyperenhancing, isoenhancing in comparison to the surrounding liver parenchyma) during the arterial, portal venous and late phases.

Results: After injection of ultrasound contrast agents, most of the HBCTs (78.3%, 18/23) had typical honeycomb enhancement pattern of the cystic wall, septa or mural nodules. Comparing between HBCA and HBCAC, hyperenhancement of the honeycomb septa during the arterial phase was more common in HBCA (p?=?.047). However, hypoenhancement during the portal venous and late phases was the characteristic of HBCAC (p?=?.041).

Conclusions: The EFSUMB algorithm for CEUS for characterization of solid focal liver lesions is also applicable to HBCT. CEUS evaluation can avoid further diagnostic investigations or invasive biopsy procedure.     

Case Report: Multiple Cancers: Hepatocellular carcinoma and adenocarcinomas of the common bile duct and the gall-bladder in a woman with primary biliary cirrhosis

We present a case of primary biliary cirrhosis associated with simultaneous triple cancers: a hepatocellular carcinoma and adenocarcinomas of the common bile duct and gall-bladder. A 70 year old Japanese woman, who had been diagnosed with primary biliary cirrhosis (stage 2 by Scheuer) 15 years before, was admitted to Koseiren Kamo Hospital in a comatose state. Laboratory data were as follows: the ammonia level was high (164.0 μg/dL), the antimitochondrial antibody showed a 320-fold increase, a high level of alpha-fetoprotein was indicated (2677 ng/mL), hepatitis B surface antigen was negative and hepatitis C antibody by enzyme immunoassay was negative, although a test for the RNA of hepatitis C virus by polymerase chain reaction was positive (10^3.5 copies/50 μL). The patient's condition gradually worsened and the patient died of liver failure. Autopsy showed triple cancers in the liver (hepatocellular carcinoma; trabecular type, moderately differentiated), the common bile duct (well-differentiated papillary adenocarcinoma) and the gall-bladder (well-differentiated papillary adenocarcinoma) with primary biliary cirrhosis (stage 4). Primary biliary cirrhosis has been believed to be a low risk for the development of hepatocellular carcinoma, despite the high risk of extrahepatic malignancy. The simultaneous occurrence of triple cancers with primary biliary cirrhosis, to the best of our knowledge, has never been reported. The present case may provide additional evidence for a predisposition to malignancy in primary biliary cirrhosis.