Hemorrhagic hepatic cysts mimicking biliary cystadenoma

The hemorrhagic simple hepatic cyst is extremely rare and can sometimes be confused with biliary cystadenoma or cystadenocarcinoma. Here we present two cases of huge hemorrhagic simple hepatic cysts. Case 1 was a 43-year-old man with a cystic lesion measuring 13 cm× 12 cm in the right hepatic lobe. Ultrasound and computed tomography showed several mural nodules on the irregularly thickened wall and high-density straps inside the cyst. Case 2 was a 60-year-old woman with a huge cyst measuring 15 cm × 14 cm in the central liver. Ultrasound and magnetic resonance imaging showed the cystic wall was unevenly thickened and there were some flame-like prominences on the wall. The iconographic representations of the two cases mimicked biliary cystadenoma. Cystectomy and left hepatectomy were performed for the two patients, respectively. Both patients recovered quickly after their operations and showed no recurrence.     

Mucinous cystic neoplasm in a young male patient

A 25-year-old Japanese man was admitted to our hospital with a history of recurrent pancreatitis and a pseudocyst of the pancreas. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) revealed an encapsulated multilocular cystic mass 5 cm in diameter in the pancreatic tail. Endoscopic ultrasonography demonstrated a mural nodule, and endoscopic retrograde pancreatography showed a communication of the lesion with the main pancreatic duct. A neoplastic cystic tumor was suspected, and a resection of the body tail of the pancreas was performed. The lesion was a multilocular cyst having a common fibrous capsule and viscous content. Histologically, the cystic lesion was lined with a single layer of columnar cells with low-grade atypia. Ovarian-type stroma (OS) was confirmed, and it showed positive for antiestrogen receptor and antiprogesteron receptor staining. Based on these findings, the lesion was diagnosed as mucinous cystic neoplasm (MCN), an adenoma that shows extraordinarily high prevalence in women. Further study on the pathogenesis of MCN in male patients should be undertaken to elucidate the process of development.     

Chronic asymptomatic pseudocyst with sludge aggregates masquerading as mucinous cystic neoplasm of the pancreas

Pseudocyst of the pancreas is sometimes difficult to distinguish from mucinous cystic neoplasm of the pancreas. A 37-year-old asymptomatic Japanese man was diagnosed with hypertension. He had a 20-years history of habitual drinking of alcohol, but no history of pancreatitis or abdominal trauma. During examinations to ascertain the cause of hypertension, ultrasonography and computed tomography incidentally demonstrated a huge cyst in the head of the pancreas. Laboratory data were within normal limits, including serum levels of amylase, carcinoembryonic antigen, and carbohydrate antigen 19-9. Imaging studies showed a huge unilocular cyst, measuring 7 cm, in the head-to-body of the pancreas, and two small unilocular cysts, measuring 1.4 and 1.5 cm, in the tail and head of the pancreas, respectively. A mural nodule was suspected in the largest cyst. Endoscopic retrograde cholangiopancreatography demonstrated communication of the main pancreatic duct with the two small cysts in the head and tail of the pancreas but not with the huge cyst. There were no ductal changes suggesting chronic pancreatitis. Laparotomy was performed under the tentative diagnosis of potentially malignant mucinous cystic neoplasms of the pancreas. However, inflammatory adhesion was dense around the pancreas and the mural nodule suspected preoperatively was found to be sludge aggregates in a pseudocyst. The diagnosis of an intraoperative frozen section of the cyst wall was pseudocyst of the pancreas. Cystojejunostomy was performed. We report this case because the pre-operative diagnosis was mucinous cystic neoplasm of the pancreas, but the diagnosis changed with careful intraoperative examinations, to pseudocyst of the pancreas. We discuss the differential diagnosis of the two conditions. (Received Oct. 1, 1997; accepted Jan. 23, 1998)     

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     

Role of endoscopic intervention in biliary complications of hepatic hydatid cyst disease

OBJECTIVE: Biliary complications of hepatic hydatidosis are often difficult to detect and manage. The aim of this study was to present our experience on the effectiveness of endoscopic treatment modalities in cases of biliary complications of hepatic hydatid cysts. MATERIAL AND METHODS: Over the past 10 years, 15 patients diagnosed with hepatic hydatidosis and manifesting symptoms and signs indicative of biliary involvement were examined by means of endoscopic retrograde cholangiopancreatography (ERCP) in our Gastroenterology Endoscopic Unit; 7 patients had already been operated on for hepatic hydatid cysts; one of them had a concomitant hydatid cyst in the lung. Diagnosis of the disease was based on a combination of ultrasonography (US), computed tomography (CT) and specific immunologic and/or microbiologic studies. RESULTS: Biliary complications of hydatid cysts were detected by ERCP in 9 patients (60%). Eight (88.9%) patients displayed a communication between the hydatid cyst or its residual cavity and the biliary tree; 5 patients had daughter cysts or residual hydatid material within the biliary tree, 1 patient had a biliocutaneous fistula, 1 patient a postoperative biliary leakage and 1 patient had only an opacification of the hydatid cyst during ERCP. In one patient, ERCP showed stenoses of both of the main hepatic ducts due to their compression by the cyst. Jaundice (88.9%), fever (33.3%) and right upper quadrant abdominal pain (88.9%) were the most frequent manifestations. These complications were demonstrated by US and CT imaging in only 25% of the cases. Four patients underwent ERCP before surgery and 5 after surgery. Endoscopic management was successful in all patients, resulting in clearance of the biliary tree, closure of fistulas, stopping of biliary leakage and jaundice remission. No serious endoscopy-related complications were recorded, with the exception of a pulmonary hydatid cyst rupture during ERCP. CONCLUSIONS: This study suggests that endoscopic treatment modalities are helpful and safe methods in the treatment of biliary complications of hepatic hydatidosis before and after definitive surgical management of the hydatid cysts.     

A pancreatobiliary-type carcinoma in situ at the periphery of a mural nodule developed from a gastric adenoma in an intraductal papillary mucinous neoplasm

We report a rare case of an intraductal papillary mucinous neoplasm (IPMN) with a pancreatobiliary-type carcinoma in situ (CIS) that originated around a mural nodule formed in a gastric-type adenoma. A 64-year-old man was admitted to our hospital in December 2001 for dilation of the main pancreatic duct, detected on abdominal ultrasonography. Branch-duct-type IPMN (diameter 25 mm) was diagnosed, and because mural nodules were absent, the patient was annually followed up for 5 years. In 2006, computed tomography scans revealed thickening of the tumor wall and the development of a mural nodule (diameter approximately 6 mm); pancreatoduodenectomy with regional lymph-node dissection was performed. Histopathologic examination showed an intraductal papillary mucinous carcinoma arising from an adenoma. Hematoxylin and eosin (H&E) staining revealed that most of the tumor components, including the mural nodule, had adenomatous changes, indicating the tumor to be of the gastric type; however, immunohistochemistry showed positive MUC2 expression. Histologically, the tissues around the nodule, including those showing a cribriform pattern, were diagnosed as CIS. These tissues were classified as the pancreatobiliary-type on the basis of the results of both H&E staining and immunohistochemistry. The patient remained disease-free for 3 years after surgery.     

Intraductal oncocytic papillary neoplasm of the pancreas with strong accumulation on FDG-PET

This paper reports a case of resected intraductal oncocytic papillary neoplasm (IOPN) of the pancreas. A 69-year-old woman was admitted with right hypochondriac pain. Ultrasonography, contrast-enhanced computed tomography and magnetic resonance imaging showed a cystic lesion, 10cm in diameter, in the head of the pancreas. The lesion contained a number of mural nodules 1cm in diameter with thick walls, which demonstrated hypervascularity. The main pancreatic duct was slightly dilated, 7mm in diameter. 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) showed very strong uptake in the thick wall and mural nodules of the tumor. Substomach-preserving pancreatoduodenectomy (SSPPD) was performed. The patient is presently alive and well without any evidence of recurrent disease 18 months after the operation. IOPN has the potential to develop into invasive carcinoma, and therefore, should be completely resected whenever possible.     

Role of endoscopic intervention in biliary complications of hepatic hydatid cyst disease

Objective. Biliary complications of hepatic hydatidosis are often difficult to detect and manage. The aim of this study was to present our experience on the effectiveness of endoscopic treatment modalities in cases of biliary complications of hepatic hydatid cysts. Material and methods. Over the past 10 years, 15 patients diagnosed with hepatic hydatidosis and manifesting symptoms and signs indicative of biliary involvement were examined by means of endoscopic retrograde cholangiopancreatography (ERCP) in our Gastroenterology Endoscopic Unit; 7 patients had already been operated on for hepatic hydatid cysts; one of them had a concomitant hydatid cyst in the lung. Diagnosis of the disease was based on a combination of ultrasonography (US), computed tomography (CT) and specific immunologic and/or microbiologic studies. Results. Biliary complications of hydatid cysts were detected by ERCP in 9 patients (60%). Eight (88.9%) patients displayed a communication between the hydatid cyst or its residual cavity and the biliary tree; 5 patients had daughter cysts or residual hydatid material within the biliary tree, 1 patient had a biliocutaneous fistula, 1 patient a postoperative biliary leakage and 1 patient had only an opacification of the hydatid cyst during ERCP. In one patient, ERCP showed stenoses of both of the main hepatic ducts due to their compression by the cyst. Jaundice (88.9%), fever (33.3%) and right upper quadrant abdominal pain (88.9%) were the most frequent manifestations. These complications were demonstrated by US and CT imaging in only 25% of the cases. Four patients underwent ERCP before surgery and 5 after surgery. Endoscopic management was successful in all patients, resulting in clearance of the biliary tree, closure of fistulas, stopping of biliary leakage and jaundice remission. No serious endoscopy-related complications were recorded, with the exception of a pulmonary hydatid cyst rupture during ERCP. Conclusions. This study suggests that endoscopic treatment modalities are helpful and safe methods in the treatment of biliary complications of hepatic hydatidosis before and after definitive surgical management of the hydatid cysts.     

Mucin-producing biliary papillomatosis associated with gastrobiliary fistula

We report a case of mucin-producing biliary papillomatosis in a 78-year-old woman. Abdominal ultrasound (US) and computed tomography (CT) showed wall thickening and dilatation of the intrahepatic bile duct (IHBD), as well as a nodular lesion, 1.2 cm in diameter, in the left branch of the IHBD. Gastric endoscopy revealed excretion of bile-containing mucin on the anterior wall of the body of the stomach. Endoscopic ultrasonography (EUS) showed gastrobiliary fistula and discharge of mucin into the stomach. Needle biopsy of the biliary tumor revealed papillary proliferation, but no malignant cells were recognized histologically. Therefore this patient was diagnosed as having mucin-producing biliary papillomatosis forming gastrobiliary fistula. She did not present with obstructive jaundice, probably because of the fistula. She is alive, without obstructive jaundice, 16 months after the diagnosis without having had surgery. This is, to our knowledge, the first reported case of biliary papillomatosis forming gastrobiliary fistula and with the patient free of obstructive jaundice. (Received May 22, 1998; accepted Aug. 28, 1998)     

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.     

Parapapillary choledochoduodenal fistula associated with cholangiocarcinoma

Parapapillary choledochoduodenal fistula is a rare disorder. We herein report a case of parapapillary choledochoduodenal fistula associated with cholangiocarcinoma. A 61‐year‐old woman was admitted to our hospital for further examination of a liver tumor. She had no clinical symptoms, but computed tomography scans showed an irregularly contoured liver tumor which was histologically confirmed to be adenocarcinoma, by a needle biopsy examination. Duodenal fiberscopy revealed a fistula orifice 1.0 cm proximal to the orifice of the papilla of Vater, and endoscopic retrograde cholangiography through the fistula showed a communication to the common bile duct. Hypotonic duodenography demonstrated reflux of contrast material into the choledochoduodenal fistula. The bile sample collected from the common bile duct showed extremely high levels of pancreatic enzymes, including amylase, phospholipase‐A2, and elastase‐I. Furthermore, Helicobacter DNA was detected in bile by polymerase chain reaction (PCR) analysis. This experience suggests to us that parapapillary choledochoduodenal fistula may be a risk factor for biliary tract carcinoma, and surgical management is the treatment of choice for this rare condition, even when the patient has no significant clinical symptoms.     

The role of total pericystectomy in hepatic hydatidosis

Between 1977 and 1995, 495 patients were operated on for hepatic hydatidosis. Total pericystectomy was performed in 26 patients (closed technique in 21 and open technique in 5). Twenty-one patients had single cysts and 5 had two cysts. The median cyst diameter was 8cm (range; 3–20cm). The requirements for total pericystectomy were: (1) the cyst(s) should be located away from the hepatic veins, large bile ducts, or major branches of the portal vein and hepatic artery and (2) the patient should be fit to undergo a major operation. There was no procedure-related morbidity. One patient developed a biliary fistula that closed after endoscopic sphincterotomy. The median hospital stay was 7 days (range; 3–22 days). The median follow up was 24 months (range; 9–114 months). There was disease recurrence in a non-adjacent segment in 1 patient at 4 years. Pericystectomy is a potentially dangerous operation, but it avoids problems with cavity management and has low rates of biliary fistula, spillage, and recurrence. Its success stems primarily from careful patient selection.     

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.     

Biliary cystadenocarcinoma followed up as benign cystadenoma for 10 years

We describe a case of biliary cystadenocarcinoma that showed a longterm clinical course. A 69-year-old Japanese man was admitted to our hospital because of abdominal discomfort. The patient had been diagnosed with benign cystadenoma of the liver at another hospital in 1987 and had been followed up for 10 years. Abdominal ultrasonography (US) and computed tomography (CT) scan demonstrated a unilocular cystic lesion, which included multiple conspicuous papillary protrusions in the left hepatic lobe. Left lateral segmentectomy was performed, and intraoperative cholangiography revealed a communication between the cystic tumor and intrahepatic bile duct. The tumor contained clear mucinous fluid and enfolded multiple yellowish papillary projections on the cystic wall. Histological examination showed the tumor to be biliary cystadenocarcinoma. The patient is doing well 4 years after the operation.     

Spontaneous external biliary fistula arising from an intrahepatic duct

A spontaneous external biliary fistula is most commonly a cholecystocutaneous fistula secondary to acute cholecystitis. A fistula arising from an intrahepatic duct is extremely rare. An 87-year-old man presented with swelling of the epigastric region and right upper quadrant abdomen. He had a history of cholecystectomy and endoscopic sphincterotomy. After antibiotic treatment and surgical opening of both lesions, abdominal computed tomography demonstrated a soft tissue mass cephalad to the umbilicus. We excised the mass, and found it to be associated with a fistula through the linea alba. Fistulography showed an abscess cavity communicating with the intrahepatic duct in segment III. Histopathological examination of the mass showed an abscess without malignancy. The fistula closed spontaneously without laparotomy. In this case, the underlying pathology was considered to be associated with a subcapsular hepatic cyst in segment III.     

Cholangitis complicated by infection of a simple hepatic cyst

An 87-year-old man was admitted to our hospital due to fever and elevated liver enzymes. Computed tomography (CT) scan revealed bile duct stones with a dilated biliary system, which confirmed the diagnosis of cholangitis. A 12-cm simple hepatic cyst was also seen in the right liver, which had been detected on CT scan 5 years before, and did not change in size. Fever did not subside even after endoscopic biliary drainage and a repeated CT scan showed an enlarged cyst up to 14 cm, suggesting cyst infection. An enlarged hepatic cyst collapsed after percutaneous transhepatic drainage, along with resolution of fever. Simple hepatic cysts are common and most of them are asymptomatic. Infection of simple hepatic cysts is a rare condition and the major entry route is considered as the biliary tract as communication between the biliary tract and cysts is reportedly observed in those cases. However, in our case, no communication was seen on cholangiogram or cystogram on fluoroscopy and bilirubin level of the cyst aspirate was low. Given the fact that patients with cholangitis are rarely complicated by hepatic cyst infection, other routes of bacterial entry to simple hepatic cysts should also be considered.     

Collagenous nodule mixed simple cyst and hemangioma coexistence in the liver

A 20-year-old female patient presented with two masses located in the left liver.In this patient, a computed tomography(CT) scan revealed a hypodense mass and a second well-defined mass with a calcified nodule in the left hepatic lobe.No enhancements were apparent in or around the masses.A laparotomy was performed due to the patient's symptoms, namely, the atypical CT findings and a risk of rupture of the subcapsular lesion.The operation revealed two masses in the left hepatic lobe and a left liver resection was subsequently performed.One of the masses involved segment Ⅲ and the other mass was located in segment Ⅳ.The histopathologic findings supported a diagnosis of collagenous nodule mixed simple cyst and hemangioma.A diagnosis of collagenous nodule mixed simple hepatic cyst is extremely rare and radiologically mimics a teratoma, hepatolithiasis, parasitic cyst, or hemangioma.Although hepatic hemangiomas are the most common benign tumors found in the liver, the present case showed atypical radiographic features.     

25例肝包虫囊肿破入胆道的诊治体会

目的探讨肝包虫囊肿破入胆道的诊断及治疗方式。方法回顾分析我院2001年～2011年行手术治疗的25例肝包虫囊肿破入胆道患者的临床表现、实验室检验、影像学检查、手术方式及治疗效果。结果超声、CT、磁共振胰胆管成像(MRCP)及内镜逆行胰胆管造影(ERCP)对于肝包虫囊肿破入胆道均具有良好的诊断价值,其中ERCP诊断价值最高,确诊率可达100%。25例患者中22例手术方式为胆囊切除、胆总管探查、T管引流+肝包虫残腔引流,其余3例行胆囊切除、胆总管探查、T管引流+肝包虫病灶根治性切除。所有患者均痊愈出院。结论超声因普及易行,应作为诊断肝包虫囊肿破入胆道的首选辅助检查,MRCP检查具有诊断准确率高和无创等优点,ERCP则对肝包虫囊肿破入胆道诊断率最高。胆囊切除、胆总管探查、T管引流+肝包虫残腔引流应作为肝包虫囊肿破入胆道首选手术方式,对于部分复杂病例可行胆囊切除、胆总管探查、T管引流+肝包虫病灶根治性切除,效果良好。     

A case of an epithelial cyst arising in the intrapancreatic accessory spleen]

A 59-year old woman was admitted to our hospital for further examination of a cystic lesion detected in the tail of the pancreas. Imaging studies showed a 35-mm-diameter cystic lesion with septa and mural nodule at the same site. The mural nodule of the cyst was thick and was enhanced. Partial resection of the tail pancreas was performed with a preoperative diagnosis of pancreatic cystic tumor. The resected specimen showed mulitlobular cyst. Histologically, the internal surface of the cyst was covered with stratified squamous cells and the mural nodule of the cyst was spleen tissue. The final diagnosis was an epithelial cyst derived from an accessory spleen in the pancreas.     

Endoscopic management of hepatic hydatid cyst with biliary communication

Background and Aim: A serious complication of hepatic hydatid cyst disease is communication between the cyst and the biliary tree. Surgical management of biliary fistulas is associated with high morbidity and mortality. We carried out a prospective study of endoscopic management of hydatid cysts communicating with bile ducts in 28 patients. Methods: Presence of biliary fistula was suspected by jaundice and/or persistent external biliary fistula after surgical excision and was confirmed by endoscopic retrograde cholangiopancreatography (ERCP). Patients underwent endoscopic sphincterotomy, and either biliary stenting or nasobiliary drainage. Nasobiliary drains were removed when bile leakage stopped and closure of the fistula was confirmed by cholangiography; stents were removed after 4–6 weeks. Results: Of 120 patients with hepatic hydatid cyst seen over a 10‐year period, 28 patients developed fistula between the hepatic hydatid cyst and intrahepatic bile ducts (right intrahepatic bile ducts in 20 patients, left intrahepatic bile ducts in eight patients). Nine of 28 patients had persistent external biliary fistula after surgery. Ten patients showed membranes in bile ducts on cholangiography. We carried out either sphincterotomy with insertion of a nasobiliary drain (n = 6) or sphincterotomy with biliary stenting (n = 22). In 10 patients, the membranes were removed from bile ducts during ERCP. Fistulas healed in all patients after a median time of 11 days (range 5–45 days) after endoscopic treatment. We were able to remove nasobiliary drainage catheters and stents 8–45 days after placement. Conclusions: Endoscopic therapy is an effective mode of treatment for biliary fistulas complicating hepatic hydatid cyst.