Hepatobiliary cystadenoma. A study of five cases with reference to histogenesis

Hepatobiliary cystadenoma is a rare hepatic lesion characterized by a multiloculated cyst lined by cuboidal or columnar epithelial cells. Four cases of hepatobiliary cystadenoma with mesenchymal stroma (HCMS) and one case of hepatobiliary cystadenoma with intracystic epithelial component were studied by light microscopy, immunohistochemical methods, and electron microscopy. Similar studies were conducted on six fetal gallbladder tissues, representing the biliary tree, and two adult ovarian tissues. By light microscopy, the columnar epithelium of the five cases of hepatobiliary cystadenoma was similar to the epithelium of the developing gallbladder. The spindle cell stroma of the HCMS and the subepithelial spindle cells of the developing gallbladders showed similar reactivity to smooth-muscle actin. Vimentin reactivity was strongly positive in the stroma of the HCMS, and in the fetal gallbladders it was only noted in the subepithelial spindle cells of the 15-week gestation fetal gallbladder tissues. By electron microscopy, the epithelium lining the hepatic lesions showed characteristic gastrointestinal features and was identical to the epithelia lining the embryonic gallbladders. Furthermore, the mesenchymal stroma of the HCMS recapitulated the features found in subepithelial tissues in developing gallbladders. Although the ovarian stroma resembled the stroma of the HCMS by light microscopy, the immunohistochemical reactions and the electron microscopic studies showed dissimilarities. This study supports the hypothesis that the hepatobiliary cystadenomas arise from ectopic embryonic tissues destined to form the adult gallbladder.     

Hepatic vein stenting for Budd-Chiari syndrome

This report describes two patients with Budd-Chiari syndrome with intractable ascites due to a tight hepatic vein stenosis while the other hepatic veins were occluded. Percutaneous transluminal angioplasty of the hepatic vein stenosis followed by insertion of expandable metallic stents reduced the pressure gradient across the stenosis to almost zero. In both patients, ascites disappeared and diuretic therapy could be reduced significantly. This treatment has remained effective for more than 1 yr in one case and 2 yr in the other. These cases demonstrate the feasibility of hepatic vein stenting as a therapy for hepatic venous outflow obstruction. This therapy may be used in selected patients to defer and perhaps avoid shunt-surgery or liver transplantation.     

Food intake increases the relative oral bioavailability of vanoxerine

Hepatobiliary cystadenoma, characterised by the interposition of a mesenchymal stroma beneath the epithelium and the connective tissue layer, is a tumor described exclusively in women. These tumors have a potential for malignant transformation. Complete surgical excision is mandatory. We report a case cystadenoma with mesenchymal stroma (CMS), with mural nodules in the cyst wall, similar to those described in ovarian mucinous cystadenoma.     

A case of biliary cystadenoma with obstructive jaundice

Biliary cystadenoma is a rare cause of obstructive jaundice. We report a case of a 78-year-old Japanese man with biliary cystadenoma presenting repetitive abdominal pain and jaundice. Ultrasound sonography revealed a hyperechoic mass in the left lateral lobe of the liver. Histological examination revealed a biliary cystadenoma. Intracystic hemorrhage was assumed to be the cause of obstruction of the bile ducts.     

Cystadenoma of the pancreas: report of a case diagnosed by endoscopic retrograde pancreatography

The first case of a cystadenoma of the pancreas occurring in an African patient is reported. cystadenoma of the pancreas is a rare tumour. It should be suspected when a patient presents with a pancreatic cyst but without a history of pancreatitis, alcoholism or abdominal trauma. Estimation of the serum amylase level, selective coeliac and superior mesenteric arteriograms, and endoscopic retrograde pancreatography (ERP) are helpful in making the preoperative diagnosis. The ERP appearances in cystadenoma of the pancreas are described.     

Hepatobiliary cystadenocarcinoma without ovarian stroma and normal CA 19-9 levels. Unusually prolonged evolution

Hepatobiliary cystadenoma and cystadenocarcinoma are rare tumors of the liver. The differential diagnosis of cystadenoma includes other cystic neoplasms, nonneoplastic cysts, and even on some occasions parasitic liver involvement. It has been suggested that elevated serum level of the tumor marker CA 19-9 may be of value in distinguishing between cystadenoma with ovarian stroma and hydatid disease. We report the unique case of a male patient with a hepatobiliary cystadenocarcinoma without ovarian stroma of prolonged evolution characterized by a normal CA 19-9 serum level, low cyst fluid CA 19-9, and a very weak CA 19-9 expression on cyst epithelium.     

Serous cystadenoma of the pancreas with invasive growth: benign or malignant?

We describe a case of serous cystadenoma, that invaded a lymph node and adipose tissue. Preoperatively, the cystic lesion of the pancreas was diagnosed as a serous cystadenoma and subsequently the patient, a 71-yr-old woman, underwent distal pancreatectomy with splenectomy. Macroscopically, a greyish white, externally lobulated and partly ovoid tumor, measuring 12 x 8.5 x 5 cm, occupied the pancreatic body and tail extensively. In cross-section, multiple nodules were observed, which measured from 0.5 to 3 cm in diameter, were separated by hyalinized fibrous septa and were filled with numerous microcysts. Light microscopic findings were consistent with those for serous cystadenoma. At the splenic hilus, the tumor was found to have invaded the lymph node and adipose tissue. Based on the clinicopathological features of the six reported cases, including the present case (which behaved in a malignant fashion in terms of pathological findings of invasion or metastasis), serous cystadenoma should be regarded as having the potential for malignant growth.     

Intrahepatic biliary cystadenoma demonstrated by intraoperative cholangiography

Biliary cystadenomas are rare neoplasms usually found in the liver. These neoplasms have a strong tendency to recur and undergo malignant transformation, and so differentiating between cystadenomas and other cystic lesions of the liver is very important. We describe herein the characteristics of these neoplasms and report the first case of an intrahepatic biliary cystadenoma being demonstrated by intraoperative cholangiography. In our case, intraoperative cholangiography was very useful in differentiating a cystadenoma or cystadeno-carcinoma from other cystic mass lesions of the liver. Not only did it reveal a communication between the intrahepatic bile duct and the cystadenoma, but it also allowed obtain fluid for cytology.     

Hepatobiliary cystadenoma can protrude and grow into the bile ducts

BACKGROUND/AIMS: To evaluate the phenomenon and the potential reasons for protrusion and growth of hepatobiliary cystadenoma into the extrahepatic bile ducts in our patients, accomplished by a review of the data regarding hepatobiliary cystadenomas published elsewhere. METHODOLOGY: In a retrospective open study conducted over the last eight years, five patients with hepatobiliary cystadenoma and one patient with hepatobiliary cystadenocarcinoma were operated on. All the patients were females aged between 25 to 61 years. Diagnostic procedures, laboratory, operative and histopathological findings and treatment were evaluated. RESULTS: Most of our patients were found to have hepatobiliary cystadenoma located in the left surgical liver. In three out of five patients with HBC mesenchymal stroma was histologically detected. In two of the three, protrusion and growth into the extrahepatic bile ducts was found. CONCLUSION: Considering the pathogenesis, location and the morphology of HBC, the mesenchymal stroma may present the competent potential for intraductal progression of the tumor. Radical excision should be performed for successful treatment of hepatobiliary cystadenomas, because of the potential for reoccurrence.     

Lymphoepithelioma-like carcinoma of the ureter

We reported a case of the biliary cystadenoma of the liver. The cystic mass had lobulation and septation and showed marked hyperintensity on T1-weighted images and hypointensity on T2-weighted images; MR findings were very unusual for cystadenoma. The content of the cystic mass was jelly-like, thick mucinous fluid without intracystic hemorrhage. We concluded that these unusual signal intensities of the cyst were due to hyperproteinous mucinous fluid.     

NMDA receptors play an anti-aggregating role in human platelets

We Describe a case of ovarian serous cystadenoma having Sertoli-Leydig cell tumor, well differentiated, in the cystic septum. Well differentiated Sertoli-Leydig cell tumor coexisting with other tumor, including serous tumor, has not yet been described. In all cases of Sertoli-Leydig cell tumor with heterologous components or other tumors, the androblastomatous components are intermediately or poorly differentiated. The present case revealed a well differentiated Sertoli-Leydig cell tumor arising in a septum of serous cystadenoma, as a circumscribed nodule. With these findings, we discuss the possibility of this Sertoli-Leydig cell tumor, considered a mural nodule, which is well established in cystic common epithelial tumors of the ovary.     

In vitro experimental study of adriamycin-loaded chitosan drug delivery system

Tumors of the epididymis are very rare. They are benign tumors in 75 per cent of the cases. Papillary cystadenoma represents 4-9 per cent of epididymal benign tumors. Often associated with the syndrome of von Hippel Lindau and infertility, histologically it can be confused with metastatic renal cell carcinoma. We report two cases of papillary cystadenoma located in the head of the right epididymis, with no concomitance with the syndrome of von Hippel Lindau, cured by the removal of the neoplastic nodule. There was no recidivation, in confirmation of the neoplastic benignity.     

Fibrolamellar hepatocellular carcinoma: results of partial liver resection

AIM: To assess the results of partial hepatic resection in the treatment of fibrolamellar hepatocellular carcinoma. PATIENTS AND RESULTS: We present six cases of Fibrolamellar hepatocellular carcinoma treated by partial hepatic resection. There were five females and one male with a mean age of 20 (2.6) yr. Five patients were stage IVA and 1 IVB based on the Union International Against Cancer Classification. Two patients, due to recurrent disease, needed further pulmonary resection and a hilar lymphadenectomy. All six cases underwent major resection, three with vascular reconstruction. No operative mortality was recorded; half of the patients displayed some degree of morbidity. One patient died six months after the operation and the rest are alive at 78, 41, 24, 12 and 9 months. We believe that even in advanced cases, an aggressive surgical policy even with vascular reconstruction is justified in patients with fibrolamellar hepatocellular carcinoma.     

Oncocytic cystadenoma of the parotid gland with prominent signet-ring cell features

A case of distinctive benign cystadenoma of the parotid gland composed of several different morphological components is presented. The most conspicuous morphological component and the largest part of the neoplasm was represented by solid sheets of oncocytic cells surrounded by myoepithelial cell layer. Most oncocytic cells possessed large intracytoplasmic vacuoles with the nuclei displaced towards the periphery, imparting them with a striking signet-ring cell appearance. The size of the intracytoplasmic vacuoles ranged from 4 to 50 microm. Immunohistochemically these signet-ring cells lacked immunoreactivity for S-100 protein and cytokeratin but they strongly stained for antimitochondrial antibody 113-1. The present case illustrates an unusual, hitherto undescribed, morphological feature of benign oncocytic cystadenoma of the parotid gland.     

Somatic von Hippel-Lindau mutation in clear cell papillary cystadenoma of the epididymis

Papillary cystadenoma of the epididymis is an uncommon benign lesion that may occur sporadically or as a manifestation of von Hippel-Lindau (VHL) disease. Neither immunohistochemical studies nor molecular genetic analyses of the VHL gene have been reported previously for this lesion. The authors describe two cases of clear cell papillary cystadenoma of the epididymis, both of which were initially confused with metastatic renal cell carcinoma. Both lesions showed positive immunohistochemical staining for low and intermediate molecular weight keratins (Cam 5.2 and AE1/AE3), EMA, vimentin, alpha 1-antitrypsin, and alpha 1-antichymotrypsin. Each was negative for CEA. Because clear cell papillary cystadenoma is similar to renal cell carcinoma histologically, and because both occur as components of the von Hippel-Lindau disease complex, the authors analyzed both cases for the presence of mutations in the VHL gene. A somatic VHL gene mutation was detected in one of the two tumors by polymerase chain reaction followed by single-strand conformation polymorphism analysis. Direct sequencing revealed a cytosine to thymine transition at nucleotide 694, resulting in the replacement of an arginine with a stop codon after the sixth amino acid of exon 3. As the VHL gene is believed to function as a tumor suppressor gene, VHL gene mutations may play a role in the initiation of tumorigenesis in sporadic cystadenomas of the epididymis.     

Contrast-enhanced endoscopic ultrasonography in pancreatic diseases: a preliminary study

OBJECTIVE: The purpose of this study was to clarify the usefulness of contrast-enhanced endoscopic ultrasonography in pancreatic diseases. METHODS: The subjects comprised 37 patients with pancreatic diseases: 11 with ductal cell carcinoma, 10 with mucin-producing tumor, five with pseudo-cyst, four with islet cell tumor, four with chronic pancreatitis, and three with serous cystadenoma. After endoscopic ultrasonography, Albunex (0.22 ml/kg) was injected intravenously at a rate of 1 ml/s into the right median vein, and observation was continued for 10 min. The presence or absence of enhancement of the lesion was determined in each disease. Because all the patients with ductal cell carcinoma, islet cell tumor, chronic pancreatitis, and serous cystadenoma, as well as five with mucin-producing tumor and three with pseudo-cyst, underwent angiography, vascularity was compared between angiographic images and those of contrast-enhanced ultrasonography. RESULTS: Enhancement of the lesion was observed in all patients with islet cell tumor and serous cystadenoma, in eight with mucin-producing tumor, and in three with chronic pancreatitis. However, no enhancement effect was observed in the patients with ductal cell carcinoma and those with pseudo-cyst. Comparison between the images of contrast-enhanced endoscopic ultrasonography and angiographic images showed three patients in whom angiograms were hypovascular, but enhancement effect was observed on ultrasonographic images. CONCLUSION: The combined evaluation of plain and enhanced images of endoscopic ultrasonography may be useful for the diagnosis of pancreatic diseases.     

Outcome of 100 patients after transjugular intrahepatic portosystemic shunt for variceal hemorrhage

OBJECTIVES: One hundred consecutive patients with recurrent or refractory acute variceal hemorrhage treated with a transjugular intrahepatic portosystemic shunt (TIPS) from June 1990 to June 1993 at Oregon Health Sciences University or the Portland Veterans Affairs Medical Center were evaluated to assess shunt patency and clinical outcome, including complications of TIPS, rebleeding, and survival. METHODS: Success of shunt placement, reduction in portal pressure, complications, survival, recurrent hemorrhage, severity of ascites, hepatic encephalopathy before and after TIPS, and shunt patency were assessed in each patient. RESULTS: The mean follow-up period was 17.7 months (range, 0.1-56.7 months). TIPS was successfully completed in all patients, with a mean reduction in portosystemic gradient from 24 to 11 mm Hg. Major complications occurred in 11 patients, including one death. Survival after TIPS was 85% at 30 days, 71% at 1 yr, and 56% at 2 yr. Variceal bleeding stopped within 24 hours after TIPS in all eight patients with active hemorrhage. Recurrent variceal hemorrhage occurred in 18 patients at a mean of 4.3 months (range, 1-713 days) after TIPS. The cumulative rate of recurrent variceal bleeding was 20% at 1 yr and 25% at 2 yr after TIPS. Recurrent variceal bleeding was associated with shunt stenosis or occlusion in all patients with endoscopically documented variceal hemorrhage, which was successfully managed by reopening obstructed shunts and performing variceal embolization. The prevalence of ascites was significantly reduced among surviving patients evaluated 3 months after TIPS (67 vs 25%, p < 0.005). Three months after TIPS, the incidence of new or worsening hepatic encephalopathy was 20%, but encephalopathy improved in an equal proportion of patients. Seventy-three of 77 (95%) shunts examined for patency were open at the last follow-up examination. However, most shunts required intervention to maintain patency, and only 48% (37 of 77) were primarily patent at a mean of 168 days (range, 2-538 days) of follow-up. Shunt stenosis or occlusion, as determined by venography, became increasingly frequent with longer follow-up (52% at 3-9 months and 70% at 9-15 months). CONCLUSIONS: TIPS is effective in lowering elevated portal pressures in patients with refractory variceal hemorrhage, has acceptable postprocedure complication and mortality rates, ameliorates ascites, and in, a minority of patients, worsens encephalopathy. Shunt stenosis occurs in the majority of patients but can be effectively treated by interventional techniques to maintain patency. The incidence of recurrent variceal hemorrhage is low and is associated with shunt stenosis or occlusion.     

Mucinous cystadenoma of the pancreas as a cause of acute pancreatitis

Acute pancreatitis is only rarely the first presentation of a cystic neoplasm of the pancreas. Mucinous cystadenomas have not been reported to be a cause of acute pancreatitis; however, we present two cases of mucinous cystadenoma of the pancreas which have caused acute pancreatitis. Both patients (female) presented acute abdominal pain, with serum amylase elevation and ultrasound scan (US) and computed tomography (CT) evidence of moderate pancreatitis, which resolved with medical treatment; fluid collection in the distal pancreas had been misinterpreted as a pseudocyst. There was no history of alcohol abuse or gallstone disease. After distal pancreatectomy the diagnosis of mucinous cystadenoma was confirmed; in one case a large pseudocyst was associated with this diagnosis. Pre-operative differential diagnosis between inflammatory and neoplastic cysts is difficult, especially when the patient's first presentation is due to an episode of acute pancreatitis. A neoplastic cyst should be considered when acute pancreatitis attacks occur in non-alcoholic women, who do not have gallstone disease.     

Incidence, consequences, and risk factors of early reocclusion after primary and/or rescue percutaneous transluminal coronary angioplasty for acute myocardial infarction

We recently experienced a 43-year-old man with a large, multiloculated, cystic tumor that appeared on the pelvis. The tumor was composed of glands and cysts lined by prostatic-type epithelium and attached microscopically to the prostate by a pedicle. The prostatic nature of the lesions was confirmed by immunohistochemical staining of epithelium for prostate specific antigen (PSA). Our review of literature disclosed nine similar cases in men of various ages, originated from the prostate and grew to massive proportions. The lesions in these reported cases did not invade contiguous structures but they can adhere to viscera in their proximity. The multilocular prostatic cystadenoma is a pathologically benign entity, and they can be definitively treated by a carefully planned complete surgical excision. This lesion should be included in the differential diagnosis of retroperitoneal cystic tumors in man. We report a rare case of multilocular prostatic cystadenoma that did not invade adjacent organs and showed no evidence of recurrence after complete surgical excision.     

Papillary cystadenoma of the epididymis. An ultrastructural and immunohistochemical study

Papillary cystadenoma of the epididymis is a rare neoplasm that is sometimes associated with von Hippel-Lindau's syndrome. Electron microscopic study of the present case revealed that neoplastic cells contained abundant glycogen granules and large lipid droplets, but a few organelles. On the apical surface there were numerous microvilli and a few single cilia, but no ciliated cells. Subepithelial basal lamina was noted, but it was occasionally disrupted. Furthermore, microvilli sprang from the circumference of the small tumor-cell nest and became associated with matrix components (microvillus-matrix associations). On immunohistochemical study, neoplastic cells showed epithelial characteristics, but positive reactivity for S-100 protein. These findings resembled those of the epithelial cells of the efferent ductules of the epididymis. In the stroma, prominent vasculature was characteristic and fenestrated-type capillaries were found in the peripheral portion of the tumor. Papillary cystadenoma of the epididymis may originate from non-ciliated epithelial cells of the efferent ductules.