Topical carbonic anhydrase inhibition increases ocular pulse amplitude in high tension primary open angle glaucoma

We report herein an unusual case of a composite glandular-neuroendocrine carcinoma of the hilar bile duct. A 71-year-old Japanese woman was admitted to our hospital suffering from general fatigue, progressive jaundice, and a high fever. Computed tomography and angiography findings revealed a solid hypervascular mass in the hepatic hilus. Thus, a subsegmentectomy of the liver (S4, S5) and bile duct resection with lymph node dissection were performed. A tumor measuring 6.0 x 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.     

Retinal dystrophy in long chain 3-hydroxy-acyl-coA dehydrogenase deficiency

We report a case of Bellini duct carcinoma of the left kidney with invasive growth pattern. A 39-year-old man was admitted to our hospital with the chief complaint of gross hematuria. Ultrasonography showed left renal swelling but normal reniform configuration of the kidney was maintained. Computed tomography demonstrated a low density tumor infiltrating into the renal cortex and with tumor extension into the renal vein. Renal angiography revealed a hypovascular tumor. We suspected a left renal cell carcinoma with tumor extension into the left renal vein, and performed radical nephrectomy. Macroscopically, the resected kidney had a normal outer contour. The tumor with infiltrative growth pattern existed in renal medulla. Histopathologic examination revealed a papillary adenocarcinoma originated in Bellini duct (pT3bN2M0). The patient underwent systemic chemotherapy (M-VAC). This case showed invasive growth pattern, which were different from the usual renal cell carcinoma and Bellini duct carcinoma.     

Use of the recursion formula of the Gompertz function for the quantitation of PCR-amplified templates

A new human extrahepatic bile duct carcinoma cell line (ICBD-1) was established from surgically resected tumor of a 71-year-old Japanese male patient. ICBD-1 cells proliferate in a layer with a population doubling time of 31.5 h and secrete tissue polypeptide antigen. ICBD-1 cells have a tetraploid pattern with a DNA index of 1.83 and chromosome counts showed equally distribution in a range from 65 to 69. IC50 values for ICBD-1 cells were 200 ng/ml for adriamycin, 400 ng/ml for mitomycin C, 2 microg/ml for cisplatin and 300 ng/ml for 5-fluorouracil. ICBD-1 cells were successfully transplanted to male nude mice, inducing progressive tumor growth. Histologically, nude mouse tumors were less differentiated than the original human tumor. Tumor cells showed alveolar structures with thin fibrous stroma, classified as poorly-differentiated adenocarcinoma. ICBD-1 is the fourth established cell line that originate from extrahepatic bile duct carcinoma and it will be applicable for the experimental studies of this disease.     

Bile duct cancer associated with extramammary Paget's disease

We report a case of bile duct cancer associated with anogenital Paget's disease. The patient was a 80-yr-old Japanese woman whose chief complaint was exanthema from the left vulva to the anus for the previous 4 yr. Histological examination of the skin biopsy of the vulva showed numerous Paget's cells. Resection of the lesion and the rectum were performed, and a permanent colostomy was created. More than 1 month after the operation, the patient suddenly developed obstructive jaundice. Percutaneous transhepatic cholangiography performed simultaneously with endoscopic retrograde cholangiography showed complete obstruction of the middle part of the bile duct. Bile cytology was class V. On the basis of these results, bile duct cancer associated with extramammary Paget's disease (EMPD) was diagnosed. About 5 months after the operation, the patient died of liver failure. Microscopically the tumor in the bile duct was poorly differentiated adenocarcinoma. Although EMPD has a tendency to be associated with underlying internal malignancies, this is the first reported case, to our knowledge, of bile duct cancer associated with EMPD.     

Imaging of polypoid endoluminal growing bile duct tumors

We reviewed the radiological documents and protocols of 196 cases of bile duct tumors examined over a period of 12 years: 20 of them (10.2%) presented with a polypoid endoluminal growth. The aim of this study was to provide a better knowledge about the radiological features of this less frequent kind of tumor. In these 20 cases, the correct diagnosis of bile duct lesion was provided in 100% by E.R.C.P., P.T.C., U.S. as well as C.T., and in 42% by arteriography. The correct diagnosis of tumor was made by E.R.C.P. in 86%, by P.T.C. in 88%, by U.S. in 61%, by C.T. in 63%, and by arteriography in 25%. A correct diagnosis of tumor could be reached in all cases by combination of several examination techniques. The anatomopathological diagnosis was: bile duct adenocarcinoma (7 cases), adenocarcinoma of the ampulla of Vater (4), villous adenoma of Vater's ampulla (2), cystadenoma (1), cystadenocarcinoma (1), hepatocellular carcinoma (1), apudoma (1), and metastases (3).     

Primary ductal adenocarcinoma of the lacrimal gland

The morphological similarity of salivary and lacrimal gland tumors is known. Many clinicopathological studies and characteristics of salivary duct carcinoma, which bears histological similarities to mammary duct carcinoma, have been recently reported; however, only one case of lacrimal duct carcinoma is reported. A second case of lacrimal duct carcinoma is presented. A 67-year-old male with a painless mass in the right upper eyelid underwent total removal of the tumor mass. Microscopic examination of the tumor mass revealed ductal adenocarcinoma of the lacrimal gland, which was the equivalent of salivary duct carcinoma. The immunohistological studies of the lacrimal duct carcinoma showed similar results to those reported for salivary duct carcinoma. The recurrent tumor in the subdural spaces was removed 2 years after the initial surgery and the patient is followed as an outpatient.     

The effect of atropine on insulin stimulated gastric secretion and gastrin release

A 28-years-old patient with a palpable mass of two fist's size in the upper abdomen rapidly developed an obstructive jaundice. A pancreatic tumor was suspected and therefore ERCP was carried out. Unusual alterations caused by metastatic lesions of a post mortem diagnosed testicular teratoma narrowing and invading the common bile duct and displacing the main pancreatic duct were visualized.     

Liver metastases from colon cancer with intra-bile duct tumor growth: radiologic features

PURPOSE: Our goal was to characterize the radiologic features of liver metastases from colon cancer with intrahepatic bile duct (IHBD) dilatation. METHOD: Radiologic findings of liver metastases from colon cancer with IHBD dilatation of four patients were compared with pathologic findings. RESULTS: The cause of bile duct dilatation in all cases was due to papillary tumor growth in the bile duct. In two patients, intra-bile duct tumor growth (IBDTG) was observed on imaging. In the other two patients, IBDTG was not observed, but a nontapered abrupt obstruction of a dilated bile duct was seen, corresponding to the microscopically proven papillary tumor growth in the ductal lumen. In three patients who underwent an extensive hepatic resection, there has been no recurrence. In one patient who had a nonanatomic limited resection, a recurrence was seen 1 year after surgery. CONCLUSION: When liver tumor with IBDTG is suspected on imaging, liver metastases should be considered in the differential diagnosis besides hepatocellular carcinoma or cholangiocellular carcinoma. Careful preoperative assessment for IBDTG by imaging is essential to determine the extent of surgical resection.     

Long-term survival of a patient with bile duct carcinoma by endoscopic biliary drainage

An 80-yr-old female presented with obstructive jaundice. Endoscopic retrograde cholangiopancreatography showed a carcinoma in the middle extrahepatic bile duct, and a biliary endoprosthesis was inserted. Exfoliative cytology of the bile and forceps biopsy of the tumor revealed a papillary adenocarcinoma. Surgical resection could not be done because of her cardiovascular complications, and neither chemotherapy nor radiotherapy was administered. Stents were exchanged and cleaned 21 times because of occlusion and cholangitis. Subsequent serial cholangiogram showed a slow growth of the papillary tumor, but local invasion to the adjacent organs or distant metastasis was not observed. The patient survived for 7 yr and 6 months after insertion of the biliary endoprosthesis.     

Prognosis and treatment of bile duct carcinoma

Bile duct carcinomas present a therapeutic challenge because of different histologies, tumor locations, and resectabilities. The goal of our study was to identify prognostic factors to better delineate therapeutic options. Forty patients (30 males and 10 females) diagnosed with bile duct cancer, treated between 1985 and 1996, at Kaiser Permanente Medical Center, Los Angeles were retrospectively reviewed. Three prognostically significant variables were identified: tumor histology, tumor location, and resection. Papillary histology was the most significant determinant of long-term survival. Of six patients with papillary adenocarcinoma, four patients (67%) underwent resection, with all four achieving long-term survival. Lower-third lesions also demonstrated a survival advantage. Four out of 12 (33%) lower-third tumors were resected, with a median survival of 36 months. Irrespective of tumor histology or tumor location, tumor resection always afforded longer survival times than did palliative treatments. A prognostic classification system based on weighted values of significant variables is presented that accurately predicted long-term survival. In conclusion, bile duct tumors in general are incurable, except perhaps for a small subset of patients with papillary adenocarcinoma. Papillary histology is the most significant determinant of ultimate survival and cure. A multifunctional prognostic classification system can be helpful for this perplexing disease.     

Usefulness of cholangioscopy in patients with focal stricture of the intrahepatic duct unrelated to intrahepatic stones

BACKGROUND: Intrahepatic duct strictures are usually caused by intrahepatic duct stones and cholangitis. However, focal strictures of the intrahepatic duct unrelated to intrahepatic stones often pose diagnostic problems. This study was undertaken to prospectively evaluate the usefulness of percutaneous transhepatic cholangioscopy in patients with focal intrahepatic duct stricture and no evidence of a stone. METHODS: Seventeen patients with focal strictures of the intrahepatic duct without any evidence of a stone were included. Percutaneous transhepatic cholangioscopic examination including procurement of biopsy specimens was performed after percutaneous transhepatic biliary drainage. RESULTS: A histopathologic diagnosis was obtained in all patients (9 adenocarcinomas, 1 squamous cell carcinoma, 2 hepatocellular carcinomas, 2 adenomas, and 3 benign strictures). Of the 9 patients with bile duct adenocarcinoma, 8 underwent surgery and a curative resection was possible in 7 patients (88%). Five patients (63%) had early-stage bile duct cancer in which cancer invasion was limited to the mucosa or fibromuscular layer and there was no evidence of lymph node metastasis. CONCLUSIONS: Percutaneous transhepatic cholangioscopy in patients with focal stricture of the intrahepatic duct unrelated to choledocholithiasis is useful for diagnosis including the detection of early bile duct cancer.     

An entrapped epidural catheter in a postpartum patient

We report a rare case of synchronous testicular seminoma and adrenocortical carcinoma. A 57-year-old man had a left testicular seminoma (clinical stage IIIB) with metastases to the lung and paraaortic lymph node. A complete response was obtained after 3 courses of chemotherapy with single-agent carboplatin. However, a left adrenal tumor was detected 1 2 months later and demonstrated a tumor volume doubling time of 2.1 months. Chemotherapy with bleomycin, etoposide and cisplatin failed to stop the tumor growth. A laparoscopic adrenalectomy was performed and pathology revealed an adrenocortical carcinoma. The patient has been free of recurrence for 42 months postoperatively.     

Combination therapy with 5'-DFUR, MMC and CDDP in patient with far advanced gastric cancer: report of a case

A 48-year-old man was referred to our hospital for a Borrmann 3 type advanced gastric cancer. Endoscopic biopsy disclosed poorly differentiated adenocarcinoma. Ultrasonography and CT scan revealed left hydronephrosis. Endoscopic retrograde cholangiography detected a stenosis of common bile duct at the hepatic hilum due to lymph nodal metastasis, and laparoscopy revealed peritoneal dissemination. Because the tumor was diagnosed as not for curative resection, the patient was treated by 4 courses of combination therapy with 5'-DFUR, MMC and CDDP. No adverse effect of chemotherapy was observed. As a result, lymph nodal metastasis and peritoneal dissemination were reduced. Curative intent total gastrectomy was performed, together with pancreatico-splenectomy, left hemicolectomy, cholecystectomy, and extended lymph nodal dissection. The patient is well and alive with no sign of recurrence 2 years after operation.     

Current problems with intrahepatic bile duct stones in Japan--congenital biliary malformations as a cause

BACKGROUND/AIMS: In patients with primary intrahepatic bile duct stones, strictures of the biliary duct are often present, but the relationship between these strictures and the formation of the stones remains controversial. Intrahepatic bile duct carcinoma in association with intrahepatic bile duct stones has recently been reported. The present study attempted to ascertain whether bile stasis induced by congenital biliary strictures is the basis for the formation of stones and occurrence of carcinoma. MATERIALS AND METHODS: We analyzed the location of strictures in 58 patients with strictures in the upper portion of the biliary tract including 38 patients with intrahepatic bile duct stones and 9 with intrahepatic bile duct carcinoma. The cell cycle of epithelial cells from the intrahepatic bile duct were analyzed with using proliferating cell nuclear antigen, which is a immunohistochemical staining method. RESULTS: Fifty six of 58 patients had congenital cystic dilatation of the common bile duct (two infant type and 54 adult type). Thirty eight patients had intrahepatic bile duct stones proximal to the strictures at the hepatic hilum. The location of the strictures were classified into four types. Nine patients had intrahepatic bile duct carcinoma and eight of the 9 carcinomas coexisted with intrahepatic bile duct stones. In the nine patients with intrahepatic bile duct carcinoma, the expression of proliferating cellular nuclear antigen (PCNA) in the carcinoma and the normal bile duct epithelium adjacent to the carcinoma was higher than that of patients with hepatocellular carcinoma without anomaly of the biliary duct. CONCLUSION: Considering the location of the strictures and clinical features, the strictures may have been formed congenitally. Furthermore, adult type cysts of the common bile duct with strictures in the upper portion of the biliary tract are thought to be the basis for the formation of primary intrahepatic bile duct stones. The most appropriate treatment for intrahepatic bile duct stones is thus suggested to be removal of the affected hepatic segment including the region of strictures, combined eventually with hepaticoenterostomy.     

Hepatoid adenocarcinoma of the stomach: a case report

A patient with primary gastric carcinoma showing a high level of serum a-fetoprotein (AFP) (368 ng/ml) is described. Subtotal gastrectomy was performed, and a month after surgery the level of serum AFP fell rapidly to within normal limits. Histologically, two types of cells coexisted in the tumor: medullary-type cells resembling trabecular-type hepatocellular carcinoma, and moderately differentiated adenocarcinoma cells. The cells of the former type were arranged mostly in a trabecular pattern with bile granules, but also showed a scirrhous pattern in a restricted area. Immunohistochemistry demonstrated that both types of tumor cells stained positively for AFP. This tumor, which should be classified as a hepatoid adenocarcinoma of the stomach, is of interest because of its rarity, bile secretion (indicating marked differentiation toward a hepatocyte form), and two cellular arrangements-trabecular and scirrhous.     

Pancreatic mucin-producing adenocarcinoma associated with a pancreatic stone: report of a case

We present an autopsy case of an 83-year-old Japanese man with a mucin-producing adenocarcinoma accompanied by pancreatolithiasis in the head of the pancreas. He suffered from obstructive jaundice and died of disseminated intravascular coagulation. He did not normally drink alcohol and had no history of chronic pancreatitis. The autopsy findings revealed a mucinous cystic tumor, composed of multiple dilatated branches, in the head of the pancreas. Histological examinations showed papillary adenocarcinoma, which scirrhously infiltrated the distal common bile duct with perineural invasion and lymph node involvement. He was thus diagnosed to have mucin-producing branch-type cancer in the head of the pancreas. The main pancreatic duct was dilated, and the residual pancreatic tissue showed moderate fibrosis and parenchymal atrophy. A stone was observed in a dilated branch of the primary lesion. To the best of our knowledge, there have only been five previously reported cases of mucin-producing tumor associated with pancreatolithiasis. Intraductal calcification is a major characteristic of chronic pancreatitis, but it is clinically important not to misdiagnose cancers associated with pancreatolithiasis such as chronic pancreatitis.     

Long-term palliative treatment of hepatocellular carcinoma extending into the portal vein and bile duct by chemoembolization and metallic stenting

We present a patient with hepatocellular carcinoma accompanied by portal vein and bile duct tumor thrombi. The patient was treated with a spiral Z-stent covered by a polyethylene sheet placed in the bile duct, a Wallstent placed in the portal vein, chemoembolization, and external radiation therapy. The patient is alive with patency of both endoprostheses 18 months later.     

Tools for evaluating disasters: preliminary results of some hundreds of disasters

We report a rare case of Bellini duct carcinoma, which is an unusual variant of renal cell carcinoma. The patient, a 56-year-old man, was admitted to our hospital for detailed examination of a renal mass on the left side. He had no clinical symptoms such as gross hematuria or flank pain. Abdominal ultrasonography, computed tomography and magnetic resonance imaging revealed a tumor 4 cm in diameter at the lower pole of the left kidney. Selective renal angiography showed an avascular mass lesion. We performed left transperitoneal radical nephrectomy with a preoperative diagnosis of left renal tumor, T2N0M0. The histopathological diagnosis was Bellini duct carcinoma of papillary tubular type. Lectin histochemistry demonstrated positive staining with soyabean agglutinin and peanut agglutinin. These findings supported our conclusion that the tumor might have originated from the Bellini duct epithelium. The patient currently remains disease-free. The pathogenesis and management of this rare condition are discussed.     

Familial periventricular nodular heterotopia

Twenty patients with carcinoma of the ampulla of Vater were studied with sonography (N = 9) or both sonography and CT (N = 11). The tumor was shown by sonography in 16 patients (80%) as a small, round or oval, fairly well delineated mass in between the dilated distal common bile duct and duodenum which was delineated owing to luminal fluid or gas (N = 13); or as a polypoid mass within the dilated distal common bile duct resulting in abrupt obstruction (N = 3). In the remaining four patients, the mass was not delineated. Bile ducts were dilated down to the level of mass or ampullary region in all cases (100%), while the pancreatic duct was dilated in five cases (45%). We believe that sonography is the technique of initial choice in the diagnosis of carcinoma of the ampulla of Vater by identifying the mass at the distal end of the dilated common bile duct and/or pancreatic duct.     

Carcinoma of Bellini's ducts: apropos of a case

OBJECTIVE: To report an additional case of collecting duct carcinoma of the kidney, known as carcinoma of Bellini. METHODS/RESULTS: A 65-year-old male patient was admitted for left renal pain. An ultrasound scan showed a solid right renal mass. The patient underwent extended radical nephrectomy. Pathological analysis of the surgical specimen disclosed carcinoma of Bellini. The patient is asymptomatic 18 months postoperatively. CONCLUSIONS: Carcinoma of Bellini is an uncommon tumor type arising from the collecting duct cells and accounts for 0.5%-2% of all renal tumors.