Search

Undifferentiated small-cell hepatoblastoma (HB) is a rare malignant tumor of childhood. The cell of origin is supposed to be a pluripotential, probably entodermal, stem-cell. Differential diagnosis of this type of HB is difficult among the group of small round and blue cell malignant tumors of children. The immunohistochemically determined coexpression of cytokeratin 8, 18, and 19 and of vimentin and actin, regularly in the absence of alpha-fetoprotein expression may be diagnostically helpful. We present the case of an undifferentiated small-cell HB of a 15-month-old girl with agenesis of the right kidney. As morphological peculiarity the tumor presented disseminated histiocytic giant cells.

We report on a 43-year-old man with a primary sarcoma of the liver. The patient was admitted to the hospital for evaluation of dyspnea, abdominal pain in the right upper quadrant, diarrhea, and fever. Physical examination revealed hepatomegaly. Increased laboratory values were found for gamma-GT, LDH, CA 125, and NSE, but not for aspartate and alanine aminotransferase. Computed tomography presented a tumor in the right lobe of the liver. Venous cavography revealed a caval tumor thrombus reaching up to the right atrium. Major liver resection combined with replacement of the vena cava inferior was proposed, but before operation the patient complained about shortness of breath. Spontaneous fragmentation of the tumor thrombus with consecutive embolization of the lungs was suspected. Despite lysis therapy the patient died because of right ventricular failure. Autopsy revealed a tumor measuring 8 cm in diameter, which was located in the right lobe of the liver and invaded the inferior vena cava. Because of multiple tumor aggregates seen in the left and right main pulmonary arteries acute tumor embolization of the lungs was regarded as cause of death. Histologically the tumor was composed of bizarre giant cells surrounded by irregular spindle cells. The positive immunoreactivity pattern of the tumor cells for vimentin, lysozym, and CD68 justified the diagnosis of a malignant fibrous histocytoma (MFH) of the liver.

The Budd-Chiari syndrome is one cause of portal hypertension with multiple etiology and different therapeutic interventional strategies. We report on a 23-year-old female patient who developed de novo liver tumors 33 months after portocaval shunting for acute Budd-Chiari syndrome.

Durch Sonographie, Farb-Doppler-Technik, Computer- und Magnetresonanztomographie sowie szintigraphische Methoden gelingt in aller Regel eine gute Differenzierung der haufigsten benignen Lebertumoren. Erganzt wird diese naturlich durch klinische und humorale Untersuchung unter Einschlus von Tumormarkern, um die Verdachtsdiagnose maligne oder benigne zu untermauern. Die Biopsie sollte weitgehend vermieden werden, da die meistens sichere Diagnostik eindeutig nichtmaligner Strukturen durch die genannten Verfahren moglich ist und malignitatsverdachtige Strukturen ohnehin eine Operation notwendig machen, sofern der Zustand des Patienten dies erlaubt. In dieser Arbeit werden nur die benignen soliden Lebertumoren behandelt, nicht die (poly-)zystischen, parasitar zystischen Lebererkrankungen und Abszesse.

Background Ischemia-reperfusion injury (IRI) can result in severe organ dys- or nonfunction. Interaction of leukocytes and endothelial cells mediated by E-selectin appears to be a key step for disturbed microcirculation. Therefore we studied gene and protein expression as well as localization of E-selectin during intestinal IRI. Methods Intestinal tissue samples were obtained from extracorporeal perfused intestines (cold ischemia time [CIT] 2 or 20 hours, each n = 5) and additionally in intestinal transplanted pigs (CIT 2 or 20 hours, each n = 1). Mucosal damage was graded according to the Chiu classification. E-selectin mRNA was determined by PCR and quantitative RT-PCR. Localization of E-selectin mRNA was performed by in situ hybridization and of the protein by immunohistochemistry. Results Histologically, mucosal damage occurred during reperfusion and was earlier and more severe after 20 hours of CIT. E-selectin mRNA expression was detected by PCR already after laparotomy and was elevated after reperfusion. Interestingly, mRNA expression was already increased after 20 hours of CIT. E-selectin mRNA was localized to the luminal surface of muscular, submucosal, and mucosal endothelial cells and the protein was detected on submucosal arterial endothelium as early as 2 hours after reperfusion. Conclusion Prolongation of CIT results in more severe mucosal damage during reperfusion, which is associated with protein expression of E-selection that might be used as a marker for activated endothelial cells. Increased E-selectin mRNA at end of 20 hours of CIT might indicate a preactivated state of endothelial cells potentially triggered by bacterial translocation or products.

We present a 40-year-old female patient with epigastric pain, ascites, and progressive liver failure, caused by Budd-Chiari syndrome (BCS) with thrombotic occlusion of the right and middle hepatic veins. As underlying diseases, essential thrombocythemia and resistance to activated protein C (APC) due to heterozygote factor V Leiden were found. Initial therapy with heparin caused thrombocytopenia (HIT) type II culminating in thrombosis of the last patent left hepatic vein and further deterioration of liver function. The decision against a surgical shunt and liver transplantation by our surgeons on the basis of the risks involved, prompted us to insert a transjugular intrahepatic portosystemic stent-shunt (TIPS). There was no measurable flow signal in the doppler sonography of the portal vein presumably due to thrombosis. A further evaluation with magnetic resonance tomography and angiography was impossible due to movement artefacts. TIPS initially served as a diagnostic tool allowing direct angiography-diagnosed thrombosis of the portal vein, the superior mesenteric and the splenic vein respectively. However, insertion of the TIPS shunt and subsequent fragmentation led to an effective hepatic decompression and full recanalisation of the portal vein. In the present case TIPS simultaneously allowed the diagnosis of portal vein thrombosis and served as rescue therapy of complicated Budd-Chiari syndrome. The potential development of HIT type II should be kept in mind when heparin is given, especially to patients with thrombophilia.

Objective: Renal-transplant patients who are immunosuppressed with cyclosporin A (CyA) are often treated with proton-pump inhibitors to prevent ulcer disease. No data are available on the effect of the novel proton-pump inhibitor pantoprazole on CyA levels. Methods: In a controlled treatment, we investigated the effect of pantoprazole, which was administered in a pragmatic schedule for acid suppression (40 mg as single oral dose at 2200 hours) in six renal-transplant patients who received CyA (Sandimmun optoral, 50–175 mg twice daily) and prednisolone (5–7.5 mg/24 h). CyA trough levels (0730–0800 hours) were measured by immunoassay. Results: In the absence of pantoprazole, mean CyA trough levels measured on three consecutive days were between 164 ng/ml and 173 ng/ml (therapeutic range 120–200 ng/ml). Pantoprazole did not affect CyA trough levels during an observation period up to 3 months long. Conclusions: Pantoprazole seems to be a safe drug in combination with CyA.

We investigated the prophylactical administration of liposomal amphotericin B (Ambisome®) in the early phase after liver transplantation (LTx). Fifty-eight patients received Ambisome® prophylactically after LTx. Ambisome® (1 mg kg -1 day -1 ) was given intravenously for 7 days after LTx. Immunosuppressive prophylaxis was cyclosporin A (CsA) based in 11 patients. Forty-seven patients had a tacrolimus-based immunosuppressive regimen. CsA and tacrolimus dosages were adjusted to trough levels of 150-250 ng ml - (EMIT) and 5-15 ng ml - 1 (MEIA II) respectively. Three patients died from sepsis due to Aspergillus fumigatus infection. Reasons for a fatal outcome were foudroyant Aspergillus pneumonia in a patient transplanted for fulminant hepatic failure on post-operative day (pod) 8; Aspergillus sepsis with severe endocardidtis in a patient with two retransplantations for graft non/dysfunction on pod 24; and disseminated aspergillosis due to Aspergillus fumigatus in a patient retransplanted for primary non-function (pod 19). All three patients underwent haemofiltration for renal failure. One patient with Candida albicans sepsis (pod 4) recovered under increased dosage of Ambisome® (3 mg kg - 1 per day). Ambisome® (1 mg kg - 1 per day) seems to be beneficial against systemic Candida infections. However, the onset of systemic Aspergillus infections could not be prevented. Obviously, higher Ambisome® doses appear to be necessary against Aspergillus. We recommend the use of Ambisome® (3 mg kg -1 per day) for patients with risk factors such as graft dys-/non-function, retransplantation, haemofiltration and complicated acute liver failure to prevent invasive aspergillosis.

Due to posterior location and the close relationship to vascular and biliary structures, resection of tumors within the caudate lobe of the liver may be a surgical challenge as well as an oncological hazard. Various approaches and techniques of isolated tumorectomy and combined liver resections are available and must be tailored to the individual situation. Prerequisite for a low operative risk is control of bleeding which can be achieved by sequential inflow and outflow occlusion of the liver.