A case of intrahepatic stones with an anomalous intrahepatic portal venous system

A 64-year-old woman who had undergone surgery for gallstones twice was admitted with epigastric pain and fever. Ultrasonography revealed gallstones in the dilated left lateral intrahepatic bile ducts. Abdominal computed tomography disclosed atrophy of the left lateral segment and the right lobe of the liver, and hypertrophy of the left medial segment and the caudate lobe. Percutaneous transhepatic cholangiography demonstrated that the stones were localized in the left lateral intrahepatic bile ducts, and that the choledochoduodenostomy previously established was patent. Percutaneous transhepatic portography revealed that the left portal vein was absent and the left medial segment was supplied by the right portal vein. Left lateral hepatic segmentectomy and release of the choledochoduodenostomy were performed. Postoperative recovery was good, and the patient is now well without any complaint. This report discusses the surgical treatment for intrahepatic stones under very complicated conditions and the relationship between intrahepatic stones and anomalous portal venous system.     

Tubercular epididymitis and orchitis: US patterns

BACKGROUND: We describe the case of a man with intrahepatic arterioportal fistulae located in the left lobe, whose left lateral segment was transplanted into his son who was suffering from severe acute hepatitis B. METHODS: A male infant with severe acute hepatitis B was considered to be a candidate for liver transplantation. The father was willing to be the donor. Preoperative evaluation of the donor revealed intrahepatic arterioportal fistulae, however, duplex ultrasonography showed normograde portal blood flow. A living-related liver transplantation was performed. RESULTS: The postoperative course for both the donor and recipient was uneventful. The recipient is free of recurrent hepatitis B and has normograde portal blood flow. CONCLUSIONS: The present case suggests that there may be a symptomless population with intrahepatic arterioportal fistulae, which cause various degrees of disruption of the portal blood flow. Duplex ultrasonography might be helpful in the evaluation of candidates for liver donation.     

Hepatic hilar inflammatory pseudotumor mimicking cholangiocarcinoma with cholangitis and phlebitis--a variant of primary sclerosing cholangitis?

Inflammatory pseudotumor (IPT) of the liver is rare. We present a case of hepatic IPT mimicking cholangiocarcinoma in which the tumor was located at the left porta hepatis. The patient was a 64-year-old man in whom abnormal liver function test results had been noted incidentally during an annual health checkup in 1993: the patient declined to go to the hospital for further examination. At the annual health checkup the following year, abnormal liver function test results were noticed again, and this time he did go to a hospital, where a hepatic mass was found. Laboratory test results were unremarkable. Based on the location of the lesion and the findings of a variety of imaging modalities, such as ultrasound and computed tomography examination, the lesion was preoperatively diagnosed as hilar cholangiocarcinoma and was surgically resected. Pathologic examination of the resected lesion, however, revealed that it was not a true tumor but an inflammatory pseudotumor with marked destructive and sclerosing cholangitis mimicking primary sclerosing cholangitis (PSC) and obliterative phlebitis. Since the location and features of the tumor in the present case are very pertinent to the relationship between IPT and PSC, we describe its clinical and histologic features and discuss the findings in relation to PSC in the context of our literature review.     

Splenic infarct as a late complication of liver transplantation

Splenic infarct is a rare complication of portal hypertension. It has been reported as an early complication after successful liver transplantation when portal pressure returns to normal and the splenic size progressively declines. It has not been reported as a late complication of liver transplantation. We describe the case of a 19-year-old patient with a splenic infarct which occurred 11 months after successful orthotopic liver transplantation for decompensated cryptogenic liver cirrhosis. Following transplantation, the patient was in excellent general health, liver function tests were normal, there was no clinical evidence of portal hypertension and the splenic size had decreased significantly compared to the pre-transplantation period, although it remained increased. The patient presented with high fever, left pleuritic pain and vomiting. The splenic size had not changed and left pleuritic exudate fluid collection was detected. A hypoechoic region of the spleen was demonstrated in the ultrasound examination corresponding to a hypodense lesion in the computerized tomography scanning. The patient recovered completely, with the disappearance of the infarct in the imaging studies in 2 months time. This case report indicates that a symptomatic splenic infarct can occur late following successful liver transplantation for liver cirrhosis despite lack of any evidence of residual portal hypertension at a time that splenomegaly has not yet regressed. The differential diagnosis from a splenic abscess in transplanted patients can be difficult but the final prognosis seems to be good.     

Multiple hepatic cysts along the intrahepatic bile duct--case report

A case of multiple hepatic cysts of the periductal gland located along the left intrahepatic bile duct is described. Ultrasonography and computed tomography disclosed many cystic lesions along the left portal vein in the left lateral segment of the liver. Percutaneous transhepatic cholangiography showed many compressed lesions. The resected specimen revealed multiple cysts of 2-7mm in diameter along the intrahepatic bile duct. Microscopically, cysts within the large Glisson's capsule were intermixed with lobuli of the periductal glands, thus suggesting periductal gland origin. Histopathological features of these cysts were similar to those of "multiple hilar cysts" reported by Nakanuma, but lack of portal hypertension and underlying chronic liver disease is the significant characteristics in this case which is different from "multiple hilar cysts".     

Decreased interleukin-7 and transforming growth factor-beta1 levels in blister fluids as compared to the respective serum levels in patients with bullous pemphigoid. Opposite behavior of TNF-alpha, interleukin-4 and interleukin-10

Prognosis of hepatocellular carcinoma (HCC) patients with tumor thrombi (TT) in the trunk of the portal vein (PV) has been extremely poor. There have been few reports of long-term survivors with such an advanced condition. In this article, the case of a 62-year-old woman of HCC, who survived for 6 years and 9 months after an operation, with TT in the trunk of the PV is described. The patient not only had a primary tumor of 4 cm in diameter with TT but also multiple intrahepatic metastases in the bilateral lobe of the liver. A palliative lateral segmentectomy with tumor thrombectomy through the incised left first branch of the PV was performed. Moreover, an intraoperative ethanol injection for residual intrahepatic metastatic tumors was performed subsequently. Hepatic arterial infusion of anti-cancer drug with Lipiodol, intraportal continuous infusion of 5-FU and percutaneous ethanol injection therapy were performed suitably during the follow-up periods. The patient survived for 6 years and 9 months after operation and died of hepatic insufficiency with cancer. In this case a patient who suffered from HCC with TT in the trunk of the PV was successfully treated by multimodality procedures including hepatic resection with tumor thrombectomy.     

Optic neuritis with residual tunnel vision in perchloroethylene toxicity

BACKGROUND: To identify and differentiate agenesis and severe atrophy of the right hepatic lobe on computed tomography (CT). METHODS: The CT examinations of three cases of agenesis and 11 cases of severe atrophy of the right hepatic lobe were reviewed. We evaluated visibility of the three hepatic veins, the two main portal veins (including their branches if necessary), the dilated intrahepatic ducts, enlargement of the medial and lateral segments of the left lobe and caudate lobe of the liver, presence of a retrohepatic gallbladder, hyperattenuation of the atrophic liver parenchyma, posterolateral interposition of the hepatic flexure of the colon, and upward migration of the right kidney. RESULTS: In the three cases of agenesis, no structure can be recognized as the right hepatic vein, right portal vein, or dilated right intrahepatic ducts. In the 11 cases of severe lobar atrophy, the right portal vein (or its branches) was recognized in eight cases, the right hepatic vein in four cases, and the dilated right intrahepatic ducts in 11 cases. The degree of enlargement of the lateral segment does not necessarily change inversely with the size of the medial segment and the caudate lobe. The retrohepatic gallbladder is present in eight cases (two in agenesis and six in atrophy). The phenomenon of hyperattenuation of the atrophic liver parenchyma was noted in six cases. CONCLUSION: Even though a retrohepatic gallbladder and a severely distorted hepatic morphology due to compensatory hypertrophy of the left and caudate lobes may raise a suspicion of agenesis of the right lobe of the liver, absence of visualization of all of the right hepatic vein, right portal vein and its branches, and dilated right intrahepatic ducts is a prerequisite of the diagnosis of agenesis of the right hepatic lobe on CT. In severe lobar atrophy, at least one of these structures is recognizable.     

CT and MRI in detection of intrahepatic portosystemic shunts in patients with liver cirrhosis

OBJECTIVE: Our goal was to determine the prevalence and anatomic location of intrahepatic portosystemic shunts (IPSs) in patients with hepatic cirrhosis as shown by CT and MRI. MATERIALS AND METHODS: We retrospectively reviewed CT and MR scans of 33 cirrhotic patients who had IPSs. In addition, two series of 100 consecutive CT or MR were reviewed to determine the prevalence of IPSs and the percentage of intrahepatic and extrahepatic paraumbilical veins. RESULTS: Intrahepatic portosystemic shunts were divided into three groups according to the intrahepatic course: paraumbilical shunt between the left portal vein and the paraumbilical vein anterior to the liver (n = 29); inferior vena caval shunt between the posterior branch of the right portal vein and the inferior vena cava (n = 2); and miscellaneous (n = 2). Shunts of the paraumbilical type ran through the medial (n = 23), lateral (n = 3), or both medial and lateral (n = 3) segments of the left lobe of the liver. Twenty-five patients had one shunt, and four had more than one. Six cases were also associated with extrahepatic paraumbilical veins. CONCLUSION: Intrahepatic portosystemic shunts, especially the paraumbilical type, were not infrequently visualized in patients with hepatic cirrhosis.     

High serum alpha-fetoprotein concentration associated with pseudoinfarction of a cirrhotic liver: report of a case

We report herein the case of a 65-year-old man with cirrhosis of the liver in whom a portal vein thrombus was found to be the cause of a marked elevation in serum alpha-fetoprotein (AFP). The patient presented with fever and abdominal pain, and a diagnostic work-up revealed a liver mass and an increased serum AFP concentration of 91,000 ng/ml. The mass gradually regressed, and the AFP concentration simultaneously decreased to 163 ng/ml. However, because hepatocellular carcinoma (HCC) could not be ruled out, a partial hepatectomy was performed. Histological examination of the resected specimen revealed a thrombus of the portal vein surrounded by the fibrosis associated with liver cirrhosis, but no neoplastic lesion was found. Thus, portal thrombus associated with liver cirrhosis might induce an extremely high level of AFP production.     

CA 19-9 in evaluating the response to chemotherapy in advanced pancreatic cancer

BACKGROUND/AIMS: The determination of serum carbohydrate antigen 19-9 (CA 19-9) level is useful in managing pancreatic cancer. However, the usefulness of this marker in evaluating the response to chemotherapy has not been fully established. MATERIALS AND METHODS: Serial changes of serum CA 19-9 levels were studied during chemotherapy in 66 pancreatic cancer patients who showed CA 19-9 level of 100 U/ml or greater before treatment. We investigated the relationship between patient survival and reduction in serum CA 19-9 level after treatment. RESULTS: When a responder was defined as a patient whose serum CA 19-9 level was reduced by more than 50% of the pre-treatment level within 2 months after treatment, CA 19-9 response was observed in 9 (13%) of the 66 patients. Median survival times of CA 19-9 responders and non-responders were 141 and 88 days, respectively. Based on Cox regression analysis, the relative risk of cancer death in CA 19-9 responders for non-responders was 0.47 (95% confidence interval, 0.21 to 1.05). CONCLUSIONS: CA 19-9 reduction may be useful for assessing the efficacy of chemotherapy for advanced pancreatic cancer.     

Sinusitis in the immunocompromised host

Using retrospective studies, we have investigated the possibility of obtaining characteristic findings of inflammatory pseudotumor of the liver by magnetic resonance (MR) imaging. We examined 8 patients (involving 8 masses) who had been histologically diagnosed as having an inflammatory pseudotumor in the liver. The histological studies were performed on an excised specimen of 1 mass, and on aspiration needle biopsy specimens and the clinical courses of the other 7 masses. T1 weighted images (T1WI) and T2 weighted images (T2WI) were obtained on MR imaging. MR imagings were analyzed for visualized patterns, patterns of internal structure and patterns of contrast enhancement of dynamic MR imaging. The 8 masses were visualized as hypointense on T1WI and hyperintense on T2WI by MR imaging. Dynamic MR imaging revealed that 1 mass was markedly enhanced peripherally while another mass was homogeneously enhanced, and that enhancement was most marked immediately after injection of contrast medium and then gradually disappeared. Vessels were observed in 4 masses (the portal vein in 2 masses, the hepatic vein in 1 mass, and portal and hepatic veins in 1 mass), and these vessels were clearly visualized on T1WI. The MR imaging findings from the early stage of an inflammatory pseudotumor showed a pattern similar to that of hepatic tumors with rich blood flow. The portal vein or hepatic vein was found in the tumor in half the patients, suggesting that this characteristic was useful for diagnosis of an inflammatory pseudotumor in the liver.     

Birthmarks to worry about. Cutaneous markers of dysraphism

CYFRA 21-1 is a fragment of cytokeratin 19 (CK 19). Four patients with large intrahepatic (or peripheral) cholangiocarcinoma (CC) and high serum levels of CYFRA 21-1 (normal, < or = 2 ng/ml) are reported. CYFRA 21-1 levels exceeded 9 ng/ml in all 4 patients. Carcinoembryonic antigen (CEA), was high in 1 (CEA; normal range, < or = 5.0 ng/ml) and carbohydrate antigen 19-9 (CA 19-9) was high in 3 (CA19-9; normal range, < or = 36 U/ml). We also measured serum levels of CYFRA 21-1 in 13 patients with hepatocellular carcinoma (HCC) more than 5 cm in diameter. Levels of CYFRA 21-1 exceeded 2 ng/ml in 9 of the HCC patients and were higher than 9 ng/ml in 2 of the HCC patients. Levels of alpha fetoprotein (AFP) and/or protein induced by vitamin K absence or antagonist II (PIVKA II) were elevated in all HCC patients (AFP, PIVKA II, respectively; normal range, < or = 10.0 ng/ml and < or = 0.1 AU/ml) CYFRA 21-1 levels were measured twice or three times during the clinical course in 2 CC patients and in 6 HCC patients, and increased gradually with tumor growth in the 2 CC patients and in 3 of the 6 HCC patients. Marked increases in serum CYFRA 21-1 levels in patients with large liver cancers, particularly in those with normal levels of AFP and PIVKA II, would suggest the existence of intrahepatic CC rather than HCC.     

A case of advanced gastric cancer resembling submucosal tumor of the stomach

We report a case of primary gastric carcinoma with a macroscopic appearance indistinguishable from that of a submucosal tumor. A 48-year-old man visited our hospital with a chief complaint of epigastric discomfort. Endoscopic examination revealed a protruding lesion with a well defined margin on the anterior wall of the gastric antrum. Most of the tumor surface was covered with apparently normal gastric mucosa and a shallow recess with mild erosion was observed on the top. Abdominal ultrasonography showed a hypoechoic lesion with an irregular margin under the gastric mucosa. Laboratory examination revealed an elevated CA19-9 level of 106.9 U/ml. In spite of repeated bouling biopsies, no histological diagnosis could be obtained before surgery. However, gastrectomy with regional lymph node dissection was performed because of the high likelihood of gastric cancer, in view of the markedly elevated CA19-9 level and irregular tumor margin demonstrated by abdominal ultrasonography. The tumor was diagnosed histologically as papillo-tubular adenocarcinoma invasive to the serosa with marked vessel infiltration. No metastasis was found in the regional lymph nodes. Gastric cancer resembling submucosal tumor is rare and often difficult to diagnose. Careful estimation of the possibility of gastric cancer and the informed consent of the patient are critically important, in cases of suspected primary gastric cancer resembling submucosal tumor, in order to decide the form of treatment.     

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.     

Successful treatment of hepatocellular carcinoma with All-trans-retinoic acid

A 44-year-old female patient was admitted to our department with diagnosis of malignant lymphoma. The abdominal USG and CT showed multiple liver lesions with partial portal vein thrombosis, moderately increased alfa-fetoprotein (AFP), ASAT, ALAT (2x normal value), serology was negative for HBV and HCV. Liver transplantation was suggested but refused because of portal vein thrombosis. ATRA (45 mg/m2/day orally) was given on the basis of the assumption that HCC and acute promyelocytic leukaemia share similar oncogenic pathway (alter the RAR alpha and beta receptors). She was gained 15 kg-s and has resumed her work as a teacher for the last 20 months. Abdominal CT showed a complete regression of the intrahepatic tumour.     

Preoperative differential diagnosis of benign and malignant pancreatic lesions--the value of pancreatic secretory trypsin inhibitor, procarboxypeptidase B, CA19-9 and CEA

BACKGROUND/AIMS: Pancreatic secretory trypsin inhibitor (PSTI). Procarboxypeptidase B (PCPB or Human pancreas-specific protein HPASP), CA19-9 and CEA were evaluated for their performance in preoperative differential diagnosis of benign and malignant pancreatic lesions. METHODOLOGY: Our prospective study included 92 patients with pancreatic lesions diagnosed by imaging techniques. In 45 of them (group I), the lesions turned out to be malignant tumors (35 pancreatic cancer, 10 other carcinoma of the pancreatic region); 47 patients (group II) had benign lesions (38 inflammatory disease of the pancreas, 9 other benign lesions). RESULTS: Statistical analysis showed significant differences between these two groups for PSTI, PCPB and CA19-9, but not for CEA. When only pancreatitis versus pancreatic cancer was analyzed, differences were more significant for PSTI and PCPB, but less significant for CA19-9. Because of a strong trend toward false positive values in patients with pancreatic inflammation, the specificity of CA19-9 in our selected patient group was only 67%, but in combination with normal PSTI (< 13.5 ng/ml), it reached 96%. CONCLUSION: In our study, PSTI and PCPB were useful markers for pancreatitis. PSTI also showed good correlation with the severity of the inflammation and provided additional preoperative information, in combination with CA19-9.     

Resection for multiple metastatic liver tumors after portal embolization

BACKGROUND: Five patients with bilateral multiple liver metastases (3 to 12 lesions) from colorectal cancer who underwent extensive liver resection after portal embolization are described. METHODS: Portal embolization of the right portal branch was performed 9 days to 8 months before hepatic resection. The location and number of metastases were determined by intraoperative ultrasonography at the time of liver resection to accomplish complete resection of the tumors. Extended right lobectomy was carried out in four patients, two of whom underwent additional wedge resection of nodules located in the left lateral segment. The other patient underwent right lobectomy associated with local resection of the tumor in the left lobe. RESULTS: The postoperative course in the five patients was uneventful, with no serious complication or liver dysfunction. Although one patient died of recurrence 28 months after liver resection, the remaining four patients were alive and free of cancer between 36 and 74 months after hepatectomy. CONCLUSIONS: The presence of bilateral multiple (four or more) metastatic liver lesions from colorectal cancer is not considered a contraindication for hepatic resection if thorough examination of the liver is performed with intraoperative ultrasonography and the surgical risk is minimal. Portal embolization appears effective for increasing the safety of hepatectomy for patients with small metastases who require major right-sided resection combined with wedge resection of the left lobe.     

Inflammatory pseudotumor of the ileum. A report of a multifocal, transmural lesion with regional lymph node involvement

Inflammatory pseudotumor (inflammatory fibroid polyp) of the ileum is a rare, usually solitary and polypoid lesion that frequently presents clinically as small-intestinal intussusception and obstruction. Regional lymph nodes are usually not involved. We describe an inflammatory pseudotumor of the ileum that was multifocal, not polypoid, and involved one regional lymph node. Grossly, two circumferential transmural nodules were separated by 8.5 cm of normal ileum. Microscopically, the lesion extended through the muscularis propria into peri-intestinal adipose tissue and involved one noncontiguous regional lymph node. The pseudotumor was composed of highly vascularized stroma with a mixture of spindle cells and chronic inflammatory cells including numerous eosinophils, lymphocytes, plasma cells, histiocytes, neutrophils, and multinucleated giant cells forming small granulomas. Immunohistochemically, the majority of spindle cells reacted with vimentin but not smooth-muscle, endothelial, or lymphoid markers. Ultrastructurally, the spindle cells had abundant rough endoplasmic reticulum, cytoplasmic filaments, and dense bodies consistent with myofibroblasts, plump endothelial cells (some with Weibel-Palade bodies), and chronic inflammatory cells. We prefer the term inflammatory pseudotumor to inflammatory fibroid polyp for the lesion in this case, since it was not polypoid and shared many histologic features with inflammatory pseudotumors arising at sites other than the gastrointestinal tract.     

Paradoxical cerebral emboli after transjugular intrahepatic portosystemic shunt and coil embolization for treatment of duodenal varices

The use of the transjugular intrahepatic portosystemic shunt to treat portal hypertension has resulted in increased recognition of its associated complications. We report a patient with refractory duodenal variceal bleeding treated with transjugular intrahepatic portosystemic shunt, as well as coil embolization, who subsequently developed bilateral cerebral and cerebellar infarcts consistent with arterial emboli. This complication has not been previously described. The patient was found to have a patent foramen ovale and a right to left intracardiac shunt leading to paradoxical embolization of clots traveling from portal to systemic venous circulation, then to the left atrium. With the relative frequency of patent foramen ovale in the population, our observation has potential importance for patients with right to left cardiac shunts who are being considered for portosystemic shunt procedures, or who are undergoing embolization of bleeding varices.     

Hepatic schistosomiasis. X-ray computed tomographic aspects. Apropos of 97 cases

Ninety seven patients with liver cirrhosis of schistosomiasis were collected. Its CT findings and features were analysed on the clinicopathological basis including (1) the high percentage of the hepatomegaly and of the enlargement of the left lobe; (2) occurrence of deposition and calcification of eggs in large bowel wall (5/5), the portal vein system (22/97), the root of the mesentery (9/97) and the liver (93/97); (3) the variety and characteristics of the appearances of the intrahepatic calcification; (4) widened and fibrotic changes in the portal canal region. It was also noted that high percentage of hepatocellular carcinoma associated with this disease in the present series (25/97).