Undifferentiated carcinoma of the common bile duct: case report and review of the literature

A 78-year-old Japanese man with undifferentiated carcinoma of the common bile duct is presented. Upon gross examination, the common bile duct was found to be obstructed by a nodule measuring 10 x 10 mm. Microscopically, the nodule was ill-defined and composed of atypical spindle-shaped and pleomorphic tumor cells. The spindle-shaped cells proliferated in a whirled or interlacing pattern simulating a sarcoma, and the pleomorphic tumor cells had abundant eosinophilic cytoplasm and bizarre nuclei. Histochemically, a few tumor cells contained mucosubstances stained with the alcian blue (AB) method in their cytoplasm. Immunohistochemically, the tumor cells were diffusely positive for CAM5.2 and AE1/AE3. The histological diagnosis was undifferentiated carcinoma (spindle cell carcinoma) of the common bile duct. Other than our patient, only four other cases of undifferentiated carcinoma in the extrahepatic bile duct have been reported in the literature.     

Carcinoma of the hepatic hilus with superficial spreading to the intrahepatic segmental bile ducts: Report of a case

A case of superficially-spreading carcinoma of the hepatic hilus is presented. Percutaneous transhepatic biliary drainage was performed to alleviate jaundice and to evaluate the biliary system. A nodular tumor originating in the upper part of the common hepatic duct was found to be invading the confluence of the right and left hepatic ducts. Extensive superficial spread was observed in the proximal portion of the right anterior superior, right anterior inferior, right posterior superior, right posterior inferior, and caudate bile duct branches. Preoperative surgical planning was carried out on the basis of an evaluation of the findings of ultrasonography, computed tomography, percutaneous transhepatic cholangiography, and percutaneous transhepatic cholangioscopy. Absolute curative surgery, which included right hepatic lobectomy with total caudate lobectomy and bile duct resection, was performed. Bilioenteric continuity was reestablished with a Roux-en-Y jejunal loop. The histological diagnosis was well-differentiated tubular adenocarcinoma of the common hepatic duct. Postoperative recovery was very good; the patient has now enjoyed a good active social life for the past 4 years and 10 months, with no signs of recurrence. In this case report, we discuss the precise preoperative diagnosis and rational surgical treatment for carcinoma of the hepatic hilus with superficial spread.     

Composite glandular-neuroendocrine carcinoma of the hilar bile duct: Report of a case

4 , S₅) and bile duct resection with lymphnode dissection were performed. A tumor measuring 6.0 3 3.0 cm was found to be located in the bile duct of the hepatic hilus. Histologically, the tumor was composed of well-differentiated adenocarcinoma and small cell neuroendocrine carcinoma cells, with a histological transition between the two components. Grimelius' method revealed the presence of diffuse positive tumor cells in neuroendocrine carcinoma. The neuroendocrine tumor cells were also diffusely immunoreactive to chromogranin A. To the best of our knowledge, only 22 previous cases of composite glandular-neuroendocrine carcinoma in the biliary tract have been reported; however, this is the first case report of a clearly composite tumor of the hilar bile duct. (Received for publication on Aug. 19, 1996; accepted on May 12, 1997)     

Small pancreatic carcinoma misdiagnosed as superficially spreading cholangiocarcinoma

We report a case of small pancreatic carcinoma misdiagnosed as superficially spreading cholangiocarcinoma using percutaneous transhepatic cholangioscopy (PTCS). The patient was a 72-year-old man admitted to a local hospital with obstructive jaundice. The patient underwent percutaneous transhepatic biliary drainage and PTCS. He was referred to our hospital with a diagnosis of superficially spreading cholangiocarcinoma. Cholangiography revealed a stenosis of the common bile duct, and also revealed some irregularities from the common hepatic duct to the left hepatic duct, suggesting a superficial spread of cancer. No pancreatic tumor was identified by endoscopic retrograde pancreatography or by enhanced computed tomography. Cholangioscopy disclosed an elevated tumor with torsional vessels and granular mucosal lesions, which were extended to the left hepatic duct. Repeated cholangioscopic biopsies of the bile duct mucosa revealed adenocarcinoma. The patient was diagnosed with superficially spreading cholangiocarcinoma extending to the left hepatic duct and the right anterior hepatic duct. Left trisectionectomy combined with pancreatoduodenectomy was performed. The cut surface of the resected specimen showed a pancreatic head tumor that was 8 mm in diameter. Histological findings of the resected specimen revealed adenocarcinoma arising from the pancreatic head with invasion in the common bile duct. Additionally, extensive inflammatory granulation tissue was observed along the surface of the bile duct, without any evidence of carcinoma. This case implies to us that the results of PTCS, even after repeated biopsies, should be interpreted with great caution.     

Intraductal papillary growth of liver metastasis originating from colon carcinoma in the bile duct: report of a case

Morphologically, liver metastases from colorectal carcinoma usually form as nodular tumor masses, whereas intraductal papillary growth in the bile duct is rare. A 65-year-old man underwent right hemicolectomy for advanced colon carcinoma, and histology of the primary carcinoma confirmed moderately differentiated adenocarcinoma with subserosal invasion, no vascular infiltration, and no lymph node metastasis. A liver tumor was found in the right paramedian Glisson pedicle and intraductal growth of cholangiocarcinoma was seen on imaging. We performed right hepatectomy and macroscopically, the resected specimen contained a growth in the bile duct lumen similar to cholangiocarcinoma. Histological examination revealed intraductal papillary proliferation of well-differentiated adenocarcinoma without vascular infiltration or lymph node metastasis in the hepatic hilum. Immunohistochemical staining revealed that the tumor cells were negative for cytokeratin-7 and positive for cytokeratin-20. Based on these findings, liver metastasis from colon carcinoma was diagnosed. Liver metastasis from colorectal carcinoma rarely arises as intraductal papillary growth in the bile duct, but the possibility of liver metastases with unusual morphology must be borne in mind for patients with a history of carcinoma in the digestive tract.     

Metastatic Biliary Polypoid Thrombus from Renal Cell Carcinoma: Report of a Case

We report an extremely rare case of metastatic biliary polypoid thrombus with hepatic metastases from renal cell carcinoma. A 74-year-old man was admitted with a low-grade fever and obstruction of the left hepatic duct. He had undergone left nephrectomy 17 years previously due to a diagnosis of renal cell carcinoma. A preoperative diagnosis of left hepatic duct carcinoma was made, and a left lobectomy and left caudate lobectomy with right biliary anastomosis of jejunal loop were performed. The resected specimen showed a polypoid mass in the left hepatic duct with metastases in the caudate lobe, and a histological examination revealed both tumors to be clear cell-type renal cell carcinoma. The mechanism of biliary metastatic thrombus formation was speculated to be as follows: caudate lobe metastases invade the adjacent bile ducts, and a tumor fragment in the bile duct then becomes implanted in the intraluminal left hepatic duct, thus leading to the growth of the biliary polypoid thrombus. Received: April 16, 2001 / Accepted: November 20, 2001     

Mucoepidermoid Carcinoma of the Liver Diagnosed as a Liver Abscess: Report of a Case

Mucoepidermoid carcinoma of the liver is a rare variant of cholangiocarcinoma, containing both mucus-secreting glandular cells and squamous cells mixed in nests. We report a case of mucoepidermoid carcinoma of the liver in a 69-year-old woman who presented with a 1-week history of fever, chills, and right flank pain. On admission, she was not jaundiced, and under a provisional diagnosis of liver abscess, a pigtail catheter was inserted into the abscess cavity. We performed right hepatectomy and partial excision of the diaphragm 1 month later. Microscopically, the tumor was composed of solid and invasive nests of epidermoid and mucin-producing cells with desmoplastic stroma. The epidermoid component of the tumor contained intercellular bridges and individual cell keratinization. Alcian blue and Periodic acid-Schiff (PAS) staining confirmed that there was mucin in the cytoplasm of mucus-secreting cells. The tumor cells, intrahepatic bile ducts, and ductules were consistently reactive with cytokeratin (CK) 7 and negative for CK20. The adjacent nonneoplastic liver cells were CK 7–/CK20–, and P63 immunostaining was positive in the epidermoid cells. The tumor was diagnosed as mucoepidermoid carcinoma arising from the intrahepatic bile duct. Despite aggressive surgical treatment, the patient died of multiple liver metastases 4 months after the right hepatectomy.     

Preoperative cholangiography of the caudate lobe: Surgical anatomy and staging for biliary carcinoma

The biliary branches of the caudate lobe (B1) join the right hepatic duct, the left hepatic duct, the confluence of these ducts, and/or the right posterior segmental bile duct. Therefore, in the preoperative staging of biliary tract carcinoma it is important to delineate the anatomy of B1 and the extent of cancer spread into B1. Tube cholangiography through percutaneous transhepatic biliary drainage or selective cholangiography by percutaneous transhepatic cholangioscopy enables us to obtain fine images of B1. We have developed cholangiography in the cephalad anterior oblique position to visualize B1 more clearly and distinctly. Four separate types of biliary branches are identified in the caudate lobe: (1) A duct running from the cranial portion of the right caudate lobe along the inferior vena cava to the hepatic hilus (B1r); (2) a duct from the cranial portion of the left caudate lobe to the hepatic hilus (B1ls); (3) a duct from the left lateral part of the left caudate lobe to the hepatic hilus (B1li); and (4) a duct from the caudate process to the hepatic hilus (B1c). The findings of the root of B1 in resected patients with biliary tract carcinoma were classified into four groups: not stenotic, short segmental stenosis, long segmental stenosis, and poorly imaged. A study of 64 branches of B1 in 42 resected patients with biliary tract cancer revealed carcinoma invasion in or near the root of B1 in all patients with poorly imaged or long segmental stenosis of B1, and in 33% of those with short segmental stenosis of B1.     

Multicentric recurrence of intraductal papillary neoplasms of bile duct in the remnant intrahepatic bile duct after curative resection

IntroductionThere have been few reports on the prognosis of patients with intraductal papillary neoplasms of the bile duct (IPNB). Here we report a case of IPNB in a patient with early-stage carcinoma who had multicentric recurrence in the remnant hepatic bile duct after curative resection.Case presentationA 78-year-old man with hepatic dysfunction and cholestasis was referred to our hospital. Preoperative imaging studies revealed the presence of papillary tumors in the left hepatic duct and common hepatic duct, while no tumor lesions were detected in the right hepatic duct. This patient underwent left hepatectomy, extra-hepatic bile duct resection with biliary reconstruction, and regional lymphnode dissection. On the basis of pathological examination, this patient was diagnosed with multiple IPNB with early-stage adenocarcinoma with negative surgical margin. Postoperative work-up was periodically performed, indicating no evidence of recurrence, while the patient had sustained hepatic dysfunction, cholestasis, and repetitive cholangitis since the early postoperative period. Finally, recurrence in the remnant intrahepatic bile duct of the posterior segment was revealed by double balloon enteroscopy at 29 months after surgery. At 34 months after surgery, internal drainage stents were replaced in both endoscopic and percutaneous manners within the relapsed intrahepatic bile ducts to address repetitive cholangitis. These procedures enabled the patient to remain asymptomatic until death at 41 months after surgery.DiscussionMulticentric recurrence in the remnant intrahepatic bile duct after surgery may occur in IPNB patients with multiple lesions. An endoscopic approach may be useful in such cases, not only in the diagnosis of remnant intrahepatic bile duct recurrence but also for palliation of symptoms.     

Rapid recurrence of spindle cell type undifferentiated carcinoma early after radical surgery in a bile duct cancer patient – A case report

IntroductionSpindle cell type undifferentiated carcinoma of the extrahepatic bile duct is extremely rare and has a poor prognosis. However, its pathology is not fully known, yet.Case presentation76-year-old man with abdominal pain and dark-colored urine was referred to our department. Contrast-enhanced computed tomography showed an enhanced tumor at the junction of the cystic duct and direct invasion of the portal vein. He was diagnosed as having resectable biliary cancer and underwent a subtotal stomach-preserving pancreaticoduodenectomy with a reconstruction of the portal vein. Histopathological findings demonstrated undifferentiated spindle cell carcinoma. Forty-two days post-surgery, he presented with peritoneal dissemination and local recurrence with ascites, and died sixty-five days after his operation.Clinical discussionSpindle cell type undifferentiated carcinoma has highly metastatic potentials and also easily invade adjacent organs. Therefore, the prognosis of an undifferentiated, spindle cell type cholangiocarcinoma was poor. Although only surgery ensures cure, multidisciplinary treatment, including chemotherapy and radiotherapy is required.ConclusionAlthough surgery for spindle cell type undifferentiated carcinoma may provide a cure, we must consider the induction of multidisciplinary treatment.     

原发性肝细胞癌侵入肝胆管的外科治疗

目的 探讨原发性肝细胞癌侵入肝胆管引起阻塞性黄疸的外科治疗效果．方法 ２３例病人均接受外科手术治疗，单纯胆管癌栓清除＋Ｔ管引流１０例；胆管癌栓清除＋肿瘤切除１０例，其中左半肝切除８例，右半肝切除１例，右肝不规则切除４例。结果 手术死亡３例，存活３￣６个月３例，半年￣１年４例占１７．４％，１年￣２年８例占３４．８％，２年￣３年４例占１７．４％，单纯胆管癌栓清除平均存活时间６．５个月，肝叶切除１７．３     

Hepatectomy and pylorus-preserving pancreatoduodenectomy with reconstruction of the hepatic artery, using the middle colic artery, for bile duct carcinoma: A case report

To curatively resect advanced bile duct carcinoma which spread from the hilus to the intrapancreatic bile duct and invaded the portal vein and the hepatic artery, left hepatic lobectomy, caudate lobectomy, hepatoduodenal ligamenteetomy, and pylorus-preserving pancreatoduodenectomy were performed. The hepatic artery was reconstructed by anastomosis of the middle colic artery to the right hepatic artery, and the portal vein was also reconstructed. Gastro-intestinal reconstruction was performed using Traverso's procedure. The patient had a relapsing liver abscess post-operatively and hospital stay was therefore prolonged. However, she was discharged. 3 months after the surgery. A histological study showed that this operation made it possible to remove the entire cancerous lesion in advanced bile duct carcinoma.     

A case of polypoid carcinoma of the left hepatic duct

A case of polypoid carcinoma of the left hepatic duct in 50-year-old male was reported. Extended left hepatic lobectomy with total caudate lobectomy and resection of the right hepatic duct were performed because the tumor involved the right hepatic duct and bile duct branches of caudate lobe, medial and lateral segment. Papillary growth of the tumor was diagnosed definitely by percutaneous transhepatic cholangioscopy (PTCS) and computed tomography. The tumor infiltrated the liver parenchyma of medial segment and compressed the middle hepatic vein. These findings were revealed by selective middle hepatic venography preoperatively.     

Intrahepatic bile duct recurrence of hepatocellular carcinoma without a detectable liver tumor

INTRODUCTIONInvasion of the portal and hepatic veins by hepatocellular carcinoma (HCC) is common, but macroscopic bile duct invasion is rare. Once a tumor thrombus completely obstructs the main bile duct, it causes obstructive jaundice. This type of HCC, known as icteric-type HCC (IHCC), has a poor prognosis.PRESENTATION OF CASEA 72-year-old woman had been treated for chronic hepatitis C since 1997. In 2002, percutaneous ethanol injection therapy was performed for HCC in segment 8. HCC recurrence occurred in 2004, and she underwent transarterial embolization (TAE) and radiofrequency ablation (RFA). In 2006, an S8 segmentectomy was performed for re-recurrence of HCC. Three years after surgery, computed tomography (CT) revealed a tumor occupying the right anterior intrahepatic bile duct and extending into its right main branch. With a preoperative diagnosis of HCC recurrence in the bile duct, we performed a right hepatectomy and thrombectomy. Histological examination showed moderately to poorly differentiated HCC. No tumor tissue other than the intrahepatic bile duct tumor was detected in the resected liver specimen.DISCUSSIONHCC with biliary tumor thrombus is associated with a poor prognosis. In general, IHCC is difficult to diagnose and treat in the early stages. A characteristic radiological finding for this type of IHCC is the hypervascularity of the tumor thrombus.CONCLUSIONTo the best of our knowledge, this is a rare case of IHCC recurrence as a tumor thrombus without recurrence in the resected liver specimen.     

Mucin-producing bile duct carcinoma originating at the hepatic hilus

We describe a mucin-producing bile duct carcinoma that was resected and analyzed pathologically. Endoscopic retrograde cholangiography disclosed amorphous filling defects in the dilated extrahepatic bile duct, suggesting that a large amount of mucin was excreted from the tumor. An intraductal amorphous filling defect showed shape change at different examination times. Pathology examination revealed a papillary tumor which showed mucus production, in the right hepatic duct. The cut section of the resected specimen showed a protuberant papillary lesion, measuring 14×14mm in diameter, forming a cystic mucous lake within the duct wall. Many cancer cells were also found in the mucous lake, the contents of which were strongly positive for mucin stain. The mucous lake developed laterally and communicated with the peribiliary glands, suggesting that the tumor had originated in these glands.     

Primary sclerosing cholangitis in which differential diagnosis from gallbladder carcinoma was difficult

We report a case of localized primary sclerosing cholangitis (PSC) which was difficult to distinguish from gallbladder carcinoma. A 75-year-old woman with elevated serum bilirubin was hospitalized and underwent endoscopic nasobiliary drainage (ENBD). There was no history of diseases such as gallbladder stone, pancreatitis, or ulcerative colitis. Cholangiography through the ENBD tube showed localized stenosis of the common bile duct; the gallbladder could not be seen. Angiography showed no encasement of the hepatic artery. Ultrasonography showed a tumor in the cystic duct, and the tumor had invaded the gallbladder and common bile duct. We diagnosed gallbladder carcinoma on radioimaging, and performed an S4aS5 subsegmentectomy of the liver and resection of the extrahepatic biliary tree. Pathologically, no malignant cells were detected, and fibrosis around bile ducts and infiltration of inflammatory cells into hepatic tissue were found. It is well known that PSC is sometimes difficult to differentially diagnose from cholangiocarcinoma. Our case is of high interest because ultrasonography showed findings suggestive of gallbladder carcinoma. It is therefore necessary to keep the possibility of PSC in mind for the diagnosis and treatment of such localized biliary stenosis.     

Successful resection of cholangiocellular carcinoma with tumor thrombus in the main trunk of the portal vein

A space-occupying lesion in the right hepatic lobe, with dilated peripheral bile ducts, was observed by ultrasonography and computed tomography in a 50-year-old man with right upper quadrant abdominal pain. One month later, this lesion evidenced rapid growth and a tumor thrombus, which completely occluded the main trunk and the left primary branch of the portal vein, had developed. The tumor was diagnosed as a cholangiocellular carcinoma with an unusual pattern of intravascular extension. The primary tumor and the portal tumor thrombus were resected via a right hepatic trisegmentectomy combined with resection of the portal vein and extrahepatic bile duct, using a superior mesenteric vein—left femoral vein catheter bypass (SMV—FV bypass). The SMV—FV bypass was found to effectively reduce intraoperative hemorrhage.     

Surgical treatment for the excluded bile leakage from Spiegel lobe after right hemihepatectomy: A case report

InroductionThe treatments of excluded bile duct leakage after hepatectomy are not easy and various strategies have been reported, such as surgery, ethanol or fibrin glue injection, and portal vein embolization.Presentation of caseA 72-year-old man with a surgical history of laparoscopic ileocecal resection for diverticular bleeding was diagnosed as having hepatocellular carcinoma. Right hemihepatectomy was performed, and computed tomography examination on postoperative day 9 showed abdominal fluid collection in the right subphrenic space. Percutaneous intra-abdominal fluid drainage was performed and it was diagnosed as bile leakage. After that it was diagnosed as excluded bile leakage from the Spiegel lobe by drip infusion cholangiographic-computed tomography and endoscopic retrograde cholangiography. To improve this clinical condition, we performed the Spiegel lobe excision on postoperative day 48. The postoperative course was uneventful and the patient was discharged.DiscussionAccording to the postoperative examination, it appeared that the bile duct from the Spiegel lobe joined to the right main bile duct or the bile duct of the right posterior section. This bile duct anomaly was not detected preoperatively on imaging examination. It is most likely that the bile duct from the Spiegel lobe was cut when the hepatoduodenal ligament in the hepatic hilum was peeled. To prevent excluded bile leakage, the hepatoduodenal ligament should be carefully peeled and ligated instead of using energy devices.ConclusionWe consider that surgical treatment for postoperative excluded bile leakage is both a quick and reliable procedure in patients with acceptable liver function and anatomical subject.     

Hilar bile duct resection for bile duct carcinoma at the hilus of the liver

Hilar bile duct resection, by which only the bile duct is resected, was carried out in 31 patients with bile duct carcinoma at the hepatic hilus. However, curative resection was possible in only 4 patients (12.9%). The postoperative 1-, 3-, and 5-year survival rates were 58.1%, 19.4%, and 7.7%, respectively. These results indicate that treatment of this hilar bile duct carcinoma by hilar bile duct resection is of limited value. We believe that this operative procedure should be used only for papillary or nodular carcinoma at the hepatic confluence at relatively early stages of Bismuth's type I or II.     

Small cell carcinoma of the cystic duct: A case report

Small cell carcinoma usually involves the lung and rarely affects the biliary tract, especially the cystic duct. In this article we report a case of small cell carcinoma of the cystic duct in a 46-year-old Japanese man. The patient presented with abdominal pain and jaundice. Imaging showed a small nodule in the cystic duct invading the common bile duct with dilatation of the proximal biliary tree. The hepatic artery and portal vein were free from invasion. Extended right hepatic lobectomy, cholecystectomy, and resection of the extrahepatic proximal bile ducts were performed together with lymph node dissection under the tentative diagnosis of carcinoma of the cystic duct. Histopathologic examination of the resected specimen revealed small cell carcinoma arising in the cystic duct and extending into the common bile duct. The postoperative clinical course was uneventful, and the patient is doing well without any signs of recurrence 1 year after the operation. To our knowledge this is the first documented case of a small cell carcinoma arising in the cystic duct.