Our experience of liver Epithelioid Hemangioendothelioma: from a misdiagnosis to liver transplantation with long term follow-up.

Malignant Hepatic Epithelioid hemangioendothelioma (HEHE) is an uncommon vascular tumor of intermediate malignant potential. HEHE is a rare tumor and it is difficult to diagnose for surgeons, hepatologists, radiologists and pathologists. So, misdiagnosis with a delay of the treatment is not uncommon. We describe a case of a young woman with a diagnosis of HEHE made 6 years after the first evidence of liver mass with a very long term follow-up after surgical treatment. She had two diagnoses of Hepatocellurar carcinoma (HCC) and a diagnosis of Cholangiocarcinoma after three different fine needle biopsies. After clinical observation, a new laparoscopic core biopsy was performed. In a first time approach, considering clinical and radiological patterns, a diagnosis of Budd-Chiari Syndrome was finally made. For that the patient underwent an orthotopicliver transplantation (OLTx). The surgical sample histological analysis allowed a definitive diagnosis of HEHE. At last, at follow up 7 years after three OLTx the patient is still alive and in good health with no evidence of recurrence.

Hepatic angiomyolipoma and intramural small intestinal schwannoma: a coincidence or a relationship?

We report a case of hepatic angiomyolipoma associated to a small bowel schwannoma in a 40-years old woman. Both lesions were asyntomatic. Histologically, hepatic angiomyolipoma showed oncocytic features and scanty adipose tissue, the tumor cells expressed desmin, smooth muscle actin, S-100 protein and HMB45. The tumor cells of intramural small intestinal mass were positive for S-100 protein and GFAP and negative for CD117, CD34 and desmin. To the best of our knowledge, no case of hepatic angiomyolipoma has been previously reported in association with intestinal schwannoma.

Clear cell "sugar" tumors of urethra: a previously undescribed occurrence and review of published data.

Clear cell "sugar" tumor is a rare benign tumor considered as a member of the family of perivascular epithelioid cell tumors. To the best of our knowledge, this is the first case of clear cell "sugar" tumor presented as a mass protruding from the urethra in a 15-year-old girl. Pathologic examination revealed a tumor composed of epithelioid cells with "water" clear cytoplasm that stained positively for melanocytic and smooth muscle-specific markers. Treatment of this tumor included a surgical excision and complete removal of the urethral mass, with examination of surgical margins. Three months after surgery, the patient remains clinically free of disease.

Sustained virological response to antiviral therapy reduces mortality in HCV reinfection after liver transplantation.

BACKGROUND/AIMS: HCV infection recurs almost in all HCV-positive patients receiving liver transplantation and carries a poor prognosis. Aim of this study was to analyze efficacy and effect on survival of antiviral therapy in this clinical setting. METHODS: Pegylated-interferon alpha-2b and ribavirin were administered at a dose of 1 microg/kg of bwt weekly and 600-800 mg/day. Planned duration of treatment was 24 or 48 weeks according to HCV genotype. Patients who failed to respond at week 24 were considered as non-responders. RESULTS: 61 patients were enrolled. According to intention-to-treat analysis, 44 (72%) patients were considered as treatment failure (31 non-responders, 4 relapsers, 9 dropout). Sustained virological response was achieved in 17 cases (28%). Genotype 2, higher doses of antivirals and absence of histological cirrhosis were predictors of sustained virological response. In the follow up, patients with sustained virological response had a significantly lower mortality compared to patients with treatment failure (chi2=6.9; P<0.01). CONCLUSIONS: Response rate to antiviral therapy in HCV reinfection after liver transplantation is higher if a full dose of antiviral drugs is administered and if treatment starts before histological cirrhosis has developed. Sustained virological response improves patient survival.

Isolated hepatic involvement of cat scratch disease in immunocompetent adults: Enhanced magnetic resonance imaging, pathological findings, and molecular analysis--two cases.

Visceral involvement in absence of lymphadenopathy is a rare manifestation in cat scratch disease; hepatic granulomas are rare, representing 0.3% of systemic manifestations of cat scratch disease, and gallbladder extension is a singular case. The present article refers to 2 rare cases of visceral cat scratch disease in immunocompetent adults with hepatic granulomatous inflammation, caused by Bartonella henselae infection, with gallbladder involvement in 1 case and no lymphadenopathy. Histological features demonstrated the presence of inflammatory necrotizing granulomatous nonneoplastic process. Molecular studies (polymerase chain reaction) were performed to confirm the infectious etiology.

Hepatic splenosis misinterpreted as hepatocellular carcinoma in cirrhotic patients referred for liver transplantation: report of two cases.

Liver transplantation is one of the main therapeutic options for hepatocellular carcinoma (HCC) occurring in cirrhotic patients; an accurate diagnosis and staging of this cancer is crucial to selecting the candidates for this treatment. Although the best diagnostic strategy is debated, the guidelines proposed by the European Association for the Study of the Liver (EASL) are used by many centers. We report 2 cases of cirrhotic patients with hepatic splenosis, a rare condition that may be misinterpreted as HCC. In conclusion, the application of the EASL guidelines in the first case would have led to an incorrect staging of the cancer and in the second case would have led to a false diagnosis of HCC.

Multicystic mesothelioma of the liver with secondary involvement of peritoneum and inguinal region.

A case of multicystic mesothelioma of the liver with secondary involvement of the pelvic peritoneum and the inguinal region is presented. The case is of interest because of its unusual location and peculiar biological behavior.