Signet ring cell carcinoma of the extrahepatic bile duct

Most tumors affecting the extrahepatic bile duct are adenocarcinomas; the other histologic types occur only rarely. We herein report the extremely rare case of signet ring cell carcinoma (SRCC) originating from the extrahepatic bile duct. A 55-year-old man was hospitalized for jaundice and pruritus. Computed tomography and positron emission tomography suggested the presence of distal extrahepatic bile-duct cancer. He underwent a pylorus preserving pancreaticoduodenectomy. A histologic study confirmed a signet ring cell neoplasm of the distal common bile duct. Because the upper resection margin was invaded by the tumor, he received postoperative concurrent chemoradiotherapy and four cycles of chemotherapy. The patient has survived with no evidence of recurrence for 2 years. This is the second case of primary SRCC of the distal extrahepatic bile duct reported in the literature; further reports of cases are warranted to determine the nature of SRCC in the extrahepatic bile duct.     

Pathological classification of intrahepatic cholangiocarcinoma based on a new concept

Intrahepatic cholangiocarcinoma (ICC) arises from the lining epithelium and peribiliary glands of the intrahepatic biliary tree and shows variable cholangiocytic differentiation. To date, ICC was largely classified into adenocarcinoma and rare variants. Herein, we propose to subclassify the former, based on recent progress in the study of ICC including the gross classification and hepatic progenitor/stem cells and on the pathological similarities between biliary and pancreatic neoplasms. That is, ICC is classifiable into the conventional (bile duct) type, the bile ductular type, the intraductal neoplasm type and rare variants. The conventional type is further divided into the small duct type (peripheral type) and large bile duct type (perihilar type). The former is a tubular or micropapillary adenocarcinoma while the latter involves the intrahepatic large bile duct. Bile ductular type resembles proliferated bile ductules and shows a replacing growth of the hepatic parenchyma. Hepatic progenitor cell or stem cell phenotypes such as neural cell adhesion molecule expression are frequently expressed in the bile ductular type. Intraductal type includes papillary and tubular neoplasms of the bile duct (IPNBs and ITNBs) and a superficial spreading type. IPNB and ITNB show a spectrum from a preneoplastic borderline lesion to carcinoma and may have pancreatic counterparts. At invasive sites, IPNB is associated with the conventional bile duct ICC and mucinous carcinoma. Biliary mucinous cystic neoplasm with ovarian-like stroma in its wall is different from IPNB, particularly IPNB showing cystic dilatation of the affected ducts. Rare variants of ICC include squamous/adenosquamous cell carcinoma, mucinous/signet ring cell carcinoma, clear cell type, undifferentiated type, neuroendocrine carcinoma and so on. This classification of ICC may open up a new field of research of ICC and contribute to the clinical approach to ICC.     

Pancreatic adenosquamous carcinoma, 9 mm in size,diagnosed preoperatively by transpapillary biopsy. Report of a case

Adenosquamous carcinoma of the pancreas is a rare variant of pancreatic cancer, which is known to be difficult to diagnose. We herein report a case diagnosed by transpapillary biopsy of the pancreatic duct. A 73‐year‐old man was referred to our department with suspicion of pancreatic cancer. Ultrasound showed a hypoechoic mass, 9 mm in diameter, in the body of the pancreas. Endoscopic retrograde cholangiopancreatography demonstrated a stenosis of the main pancreatic duct in the body with upstream dilatation and a shift of the bile duct to the left. Histological examination of the specimens obtained by transpapillary biopsy of the pancreatic duct revealed both adenocarcinoma and squamous cell carcinoma. Pancreatoduodenectomy was performed and, histopathologically, the tumor was diagnosed as adenosquamous carcinoma of the pancreatic body, 9 mm in size. This is the first case of adenosquamous carcinoma of the pancreas preoperatively diagnosed by transpapillary biopsy of the pancreatic duct, and the smallest stage I (TNM classification) case of such carcinoma detected to date.     

Small-cell carcinoma of the extrahepatic bile duct: a case report and review of the literature

A small-cell carcinoma of the extrahepatic bile duct in a 69-year-old woman is herein reported. A tumor measuring approximately 3 cm in diameter was located at the confluence of the common bile duct, cystic duct, and common hepatic duct. Histopathologically, the tumor was small-cell neuroendocrine carcinoma without any gland formation or differentiation to squamous cell carcinoma. Tumor cells were immunoreactive for epithelial markers such as epithelial membrane antigen and cytokeratin and for the neuroendocrine markers such as neuron-specific enolase, chromogranin A, and synaptophysin. Although the carcinomas in more than half of the reported cases have been reported to be associated with well-to-moderately differentiated squamous or glandular components, seven cases, including our case, showed the carcinomas without squamous or glandular components. According to the review of 16 previously reported cases and our case of small-cell carcinoma of the extrahepatic bile ducts, there is no significant difference in the clinicopathological findings, namely, age, sex, site of carcinoma, and prognosis between the cases with or without squamous or glandular components. No CD34-positive multipotent adult progenitor cells, which might be the origin of the small-cell carcinoma, were detected in the bile duct epithelium in our case.     

A case of adenosquamous carcinoma of the papilla of Vater]

Adenosquamous carcinoma of the papilla of Vater is a rare tumor and only a few cases have been reported so far. Here, we report a case of adenosquamous carcinoma in a 76-year-old male who presented with jaundice and right upper quadrant abdominal pain. Ultrasonography and enhanced abdominal CT scans showed dilated common bile duct (CBD) and intrahepatic bile duct (IHD) with a suspicious obstructing mass in distal CBD. On endoscopy, obstructing and ulcerated mass was noted on the papilla of Vater. Histopathological inspection of the biopsied specimens from mass showed adenosquamous cell carcinoma of the papilla of Vater. Since the patient refused operation, we inserted a self-expandable metallic stent in distal CBD. This is the first case report on adenosquamous carcinoma of the papilla of Vater in Korea.     

Mucosal bile duct carcinoma with superficial spread

We describe a case of mucosal bile duct carcinoma with superficial spread in a 69-year-old man with gallstone pancreatitis. The patient was seen at the hospital because of abdominal pain, fever, and jaundice. Endoscopic retrograde cholangiography (ERC) demonstrated a protruding lesion in the lower third of the common bile duct (CBD) showing wall irregularity suggestive of malignancy. Percutaneous transhepatic cholangioscopy (PTCS) disclosed a papillary tumor with granular mucosa extending continuously to the middle third of the CBD. Cholangioscopic biopsy specimens taken from both the papillary tumor and surrounding granular mucosa revealed papillary adenocarcinoma. After this assessment of extent of cancer by PTCS, we performed pancreatoduodenectomy with extrahepatic bile duct resection and regional lymph node dissection. Pathology examination revealed papillary adenocarcinoma limited to the mucosal layer. The resected margin of the bile duct was free of tumor. We also reviewed 25 cases of early mucosal bile duct carcinoma described in detail in the Japanese literature, and we discuss the diagnostic advantages of PTCS.     

Groove pancreatitis: a rare segmental form of chronic pancreatitis

Groove pancreatitis is a rare form of segmental chronic pancreatitis which is localized within the head of the pancreas, the duodenum and the common bile duct. Symptoms are due to common bile duct stenosis or duodenal stenosis. Radiologically, there is a pancreatic mass, which hinders differential diagnosis with pancreatic carcinoma. We report here a case of groove pancreatitis observed in a 41-year-old man treated by pancreatoduodenectomy. Histological features of the groove scar were noted. Our case and cases reported in the literature lead to hypotheses concerning the pathogenesis and clinical, biological, and radiological features suggestive of the diagnosis.     

A Case of Choledochocele Associated with Carcinoma of the Pancreas

A-59-year old woman was admitted with lumbago and back pain. An abdominal ultrasonography and abdominal computed tomography demonstrated a tumor of the body of the pancreas and multiple space occupying lesions in the liver. Endoscopic retrograde chorangiopancreatography revealed a 11 mm cystic dilatation of the terminal portion of the common bile duct protruding into the duodenal lumen and an obstruction of the pancreatic duct in the body portion. Celiac artery angiography showed an irregularity in the wall of the splenic artery. In line with these findings, the patient was diagnosed as suffering from choledochocele associated with carcinoma of the pancreas. As she already had multiple metastatic liver tumors, her treatment was limited to amelioration of her symptoms. Increasing stenosis of the common bile duct, however, required endoscopic sphinctomy and endoscopic retrograde biliary drainage. Type 3 Choledochocele of Alonso-Lej's classification is very rare, and only a few cases have been reported in association with malignant bile duct tumors. We believe this case to be the first one in our country linked with carcinoma of the pancreas.     

Mucosa-associated lymphoid tissue lymphoma of the extrahepatic bile duct

Mucosa-associated lymphoid tissue lymphoma of the extrahepatic bile duct has not yet been reported. Much more common than this is secondary involvement of the extrahepatic bile duct in cases of disseminated lymphoma. A 59-year-old man manifesting jaundice was referred to our hospital. PTC revealed an extrahepatic bile duct stenosis from the hilum to the lower part of the choledochus. On the operative specimen, we examined L26/CD20, Bcl-2, UCHL-1/CD45RO, cyclin D1 and p53. Histologically, follicular colonization, centrocyte-like cells and lymphoepithelial lesion was observed. Tumor cells were positive for L26/CD20 and Bcl-2 and were negative for intracytoplasmic immunoglobulins, UCHL-1/CD45RO, cyclin D1 and p53. Pathological diagnosis was mucosa-associated lymphoid tissue lymphoma of the extrahepatic bile duct. The authors present herein the first case of mucosa-associated lymphoid tissue lymphoma of the extrahepatic bile duct. It was very difficult to distinguish from hilar cholangiocarcinoma clinically. Only incomplete stenosis of the bile duct and 18-F fluoro-2-deoxyglucose positron emission tomography (FDG-PET) could suggest this unusual clinical entity.     

Extensive bone marrow necrosis associated with carcinomatosis 7 years after operation for gastric cancer

A case of extensive bone marrow necrosis due to cancer metastasis is reported. A 55-year-old female, who had a history of subtotal gastrectomy for signet ring cell carcinoma of the stomach 7 years ago, was admitted to our hospital with a complaint of lumbago on October 25, 1987. Red blood cell count was 92 X 10(4)/microliters, hemoglobin 2.7 g/dl, hematocrit 8.0%, platelet 6.4 X 10(4)/microliters, and white blood cell count 13,400/microliters with leukoerythroblastosis. Bone marrow aspiration of the sternum, left iliac crest, and bilateral posterior superior iliac supine showed extensive bone marrow necrosis. Serum ALP was increased to 7410IU/l, dominated isozyme of bone type. Hemostatic findings suggested a complication of consumption coagulopathy. Skull, vertebrae, iliac and pelvic bone X-ray showed multiple osteolytic lesions, and irregular isotope uptake was recognized on the bone scintigraphy using 99mTc. Sixth bone marrow examination at the right iliac crest revealed signet ring cell carcinoma metastasis. In spite of detailed examinations, there was no evidence of primary carcinoma, including the remnant of stomach. We speculated that the signet ring cells were originated from the respected gastric cancer. The patient has received anti-cancer chemotherapy with UFT and OK432, and is still alive 9 months after diagnosis.     

A case of groove pancreatitis (segmental form) presented with obstructive jaundice associated with pancreatobiliary maljunction

A 52-year-old-man was admitted to our hospital for obstructive jaundice. Percutaneous transhepatic cholangio drainage (PTCD) and endscopic retrograde cholangiopancreatography (ERCP) were performed, and pointed out stenosis of lower common bile duct (CBD) and pancreatobiliary maljunction. Brushing cytology of this lesion was negative for malignancy. CT and MRI revealed chronic inflammatory change in groove lesion with no mass formation suggesting tumor. So we diagnosed groove pancreatitis (segmental form) associated with pancreatobiliary maljunction, and operation (resection of the bile duct and biliary reconstruction by Roux-en-Y) was done. Resected specimen was revealed stenosis of the bile duct formed by fibrous tissue with no malignancy compatible to groove pancreatitis pathologically. This is first reported case of groove pancreatits associated with pancreatobiliary maljunction.     

Minute signet ring cell carcinoma occurring in gastric hyperplastic polyp

We describe a 45-year-old woman with minute signet ring cell carcinoma occurring in a gastric hyperplastic polyp. A biopsy specimen obtained from the gastric hyperplastic polyp revealed signet ring cell carcinoma. Endoscopic mucosal resection （EMR） was performed to confirm the diagnosis. Histological examination of the EMR specimen revealed focal signet ring cell carcinoma in the hyperplastic polyp. There are few cases of gastric hyperplastic polyp associated with signet ring cell carcinoma.     

Case report: mucinous cholangiocarcinoma featuring a multicystic appearance and periportal collar in imaging

A case of mucinous cholangiocarcinoma (CC), a rare histological type of CC, featuring unusual images is reported. The patient was hospitalized because of acute development of jaundice and fever. Computed tomography demonstrated multiple cystic lesions in the liver and a band-like low density area parallel to the intrahepatic portal vein, a so-called 'periportal collar'. Endoscopic cholangiography revealed a stricture of the hepatic duct with slight upstream dilatation. Cytology of the bile juice and fine-needle aspiration of the cystic lesion in the liver disclosed mucinous carcinoma. The patient died of multiorgan failure 3 weeks after admission. The autopsied liver showed that multiple mucus lakes were lined with adenocarcinoma cells and signet ring cells were floating in the mucus lakes. The cancer cells had spread along the portal tract and invaded into the hepatic parenchyma.     

Signet ring cell carcinoma of the bile duct: a case report

A 72-year-old man was admitted with obstructive jaundice. Computed tomography revealed a 4cm tumor with multiple cystic components obstructing the common bile duct. Endoscopic ultrasonography, endoscopic retrograde cholangiopancreatography and intraductal ultrasonography demonstrated the tumor, which derived from the lower bile duct, grew into the bile duct lumen. Peroral cholangioscopy revealed distended tumor vessels on the surface of the tumor. Signet ring cell carcinoma of the bile duct was diagnosed by biopsy. The patient died 3 months after the first hospital admission despite chemotherapy.     

Superficial Epithelium in the Biopsied Sections Taken from Malignant Bile Duct Stenosis

Abstract: Although percutaneous transhepatic cholangioscopy (PTCS) with biopsy is useful for the diagnosis of superficial spreading carcinoma of the bile duct, sections of biopsy specimens sometimes do not contain superficial epithelium. We sought to clarify the factors influencing the presence of superficial epithelium in biopsy specimens in a retrospective microscopic analysis of 168 hematoxylin and eosin-stained sections of specimens from 54 patients with malignant bile duct stenosis. We correlated the presence of superficial epithelium with various factors using logistic regression analysis. Factors examined were whether the site was stenotic, the mucosal pattern at the biopsy site (papillo-granular or smooth), specimen size, and whether the biopsy specimen was the first one taken at that same site. Bile duct tissues were present in 165 of 168 specimens (98%), and 93 (55%) contained superficial epithelium. By logistic regression analysis, mucosal pattern and order of biopsy at the same site were significantly related to presence of superficial epithelium on the histologic section (odds ratio with papillogranular mucosal pattern, 6.082; with first biopsy at the site, 2.628). Therefore, it is important to carefully choose biopsy forceps and to take multiple specimens at various sites to obtain superficial epithelium, especially with the smooth bile duct mucosal pattern.     

Early-stage primary signet ring cell carcinoma of the colon

Primary signet ring cell carcinoma of the colorectum detected at an early stage is very rare; most cases are detected at an advanced stage. Therefore, its progno-sis is poorer than that of ordinary colorectal cancer. A 56-year-old Korean man was seen at this hospital for management of signet ring cell carcinoma of the co-lon. Colonoscopic examination revealed a Ⅱa-like, ill-defined and flatly elevated 9-mm residual tumor in the cecum. Endoscopic mucosal resection was preformed. Pathological examination of the resected specimen re-vealed signet ring cell carcinoma that had invaded the lamina propria without venous or perineural invasion. Abdominal computed tomography (CT) and positron CT showed no evidence of primary lesions or distant me-tastasis. An additional laparoscopic right-hemicolectomy was performed; no residual tumor or lymph node me-tastasis was found. We report a case of primary signet ring cell carcinoma of the colon detected at an early stage and provide a review of the literature.     

An autopsy case of colon cancer associated ulcerative colitis complicated focal squamous cell carcinoma]

A 28-year-old woman with ascites was admitted to our hospital. We diagnosed peritonitis carcinomatosa caused by colon cancer complicated by ulcerative colitis. We performed peritoneal tap and infusion of mitomycin C, and administered 5-fluorouracil. Her clinical status gradually worsened, and she died 5 months later. At autopsy, the histological examination showed many mucinous adenocarcinoma and signet ring cell carcinoma with dysplasia. There were also some areas of squamous cell carcinoma with squamous metaplasia and dysplasia far from rectum. Squamous cell carcinoma and adenosquamous cell carcinoma of the colon are rare complications of ulcerative colitis. We reported this case as an addition to the literature on the subject.     

Mucosa-associated lymphoid tissue-type lymphoproliferative lesion of the common bile duct

Only a few cases of primary lymphoma of the common bile duct have been reported up to now. Here, we report a mucosa-associated lymphoid tissue (MALT)-type lymphoproliferative lesion of the common bile duct with some considerations about MALT lymphoma and benign lymphoproliferation. The patient, a 71-year-old woman, was admitted to our hospital because of progressive epigastric pain. Abdominal ultrasonography, magnetic resonance cholangiopancreatography, endoscopic retrograde cholangiopancreatography, and abdominal computed tomography showed a non-specific finding of stenosis of the common bile duct due to thickening of the wall. Angiography provided little diagnostic information. Bile duct carcinoma was diagnosed and pylorus-preserving pancreaticoduodenectomy was performed. Histological examination revealed that the common bile duct was occupied by full-thickness proliferation of lymphoplasma cells with many reactive lymph follicles. The histological features were somewhat consistent with those of MALT lymphoma. However, we could not determine the clonal proliferation of lymphoplasma cells in either immunohistochemical immunoglobulin light chain restriction of the plasma cells or in polymerase chain reaction based immunoglobulin heavy chain gene rearrangement. The present case may be categorized as a borderline lesion close to benign lymphoproliferation. To the best of our knowledge, there have been no reports of cases similar to the present case.     

How many biopsies should be performed during percutaneous transhepatic cholangioscopy to diagnose biliary tract cancer?

BACKGROUND: The sensitivity of biopsy in the diagnosis of cholangiocarcinoma using percutaneous transhepatic cholangioscopy is not well defined. METHODS: Patients with a biliary tract malignancy (n = 52) underwent directed biopsy during percutaneous transhepatic cholangioscopy using a 1.8 mm diameter forceps. Histologic findings were correlated with endoscopic appearance. RESULTS: A diagnosis of carcinoma was made in all four patients with a tumor of the major duodenal papilla and in all 15 patients with a polypoid bile duct tumor with two biopsies from the mass. In 19 patients with stenotic bile duct cancer, a positive diagnosis was made in 95% of cases when three biopsies were taken from the margin of the stenotic area. When cholangioscopy showed a tortuous, dilated vessel (n = 10), the diagnosis of cancer was made with two biopsies taken from the margin of the stenosis. In 14 patients with metastatic bile duct cancer, the diagnosis was made in only 43% of cases when three biopsies were taken from the margin of the stenosis. When combined with results from the three biopsies taken from within the area of stenosis, the sensitivity for diagnosing pancreatic cancer improved from 20% to 60%. CONCLUSIONS: Directed cholangioscopic biopsies are highly sensitive for the diagnosis of cholangiocarcinoma but less sensitive for cancer metastatic to the bile duct. The numbers and locations of the biopsies required to make a diagnosis of carcinoma depend on the origin and cholangioscopic appearance of the tumor.     

Endoskopische und perkutane Interventionen in der Langzeitbehandlung benigner Gallengangstenosen

Benign biliary stenosis can have various causes and requires differentiation from disorders caused by malignant disease. Treatment of benign stenosis is often difficult and includes treatment modalities such as endoscopic, percutaneous or surgical interventions. Exact knowledge of the etiology and localization of the stenosis is essential when selecting the appropriate method of treatment. Here we present the case of a 71-year-old patient admitted to our hospital with cholangitis 13 years after undergoing radiotherapy of the renal bed due to hypernephroma of the right kidney. The patient was diagnosed with common bile duct stenosis due to the secondary effects of radiation, which is rarely reported in the literature. Our case covers a total treatment period of 15 years, enabling us to also discuss a viable sequence of treatment modalities in the treatment of benign bile duct stenosis.