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Purpose: Perivascular epitheloid cell tumour [PEComa] is a rare neoplasm entity, characterized by perivascular epitheloid cells with a coexpression of smooth muscle and melanocytic markers. PEComas are found in a variety of localizations, though lesions within the liver are still scarcely found. Although the majority of these tumours are recognized as benign, there are some reports about advanced and aggressive tumours even with fatal outcome. By means of this case report and literary review including other 21 published cases, potential treatment modalities concerning clinical diagnostics, therapy and the follow-up care should be discussed., Methods: The following report presents the case of a 53-year old woman with a known liver lesion, since four years under regularly sonographic controls. Finally, after a haemorrhage episode, the lesion was resected and the diagnosis found. For the literary review a systematic search for case reports published between January 1, 1999 and May 1, 2012 was performed on Pubmed., Results: The only way, till now, of confirming the diagnosis is through immunohistochemical examinations. The already published Malignancy criteria by Folpe et al. must be taken carefully in question, as there are cases of malignant behaviour, that do not exactly coincide with these., Conclusion: Primary PEComa of the liver must be treated as potential malignant and therefore a close follow-up is demanded., (© Georg Thieme Verlag KG Stuttgart · New York.)

Here we report on a patient with a primary hepatocellular carcinoma in a non-cirrhotic liver, in whom heterozygosity for an AAT-deficiency allele was found (PiMZ). Based on this observation and the current literature, the possible mechanisms for an eventual contribution of a heterozygosity of a heterozygous AAT-deficiency for a hepatocellular carcinoma are discussed. Alpha-1-antitrypsin (AAT)-deficiency (Laurell-Eriksson syndrome) is a genetic disorder, in which individuals who are homozygous for a deficiency allele are at an increased lifetime risk for pulmonary emphysema, liver cirrhosis, and primary hepatocellular carcinoma. It has been controversially discussed whether the heterozygous form (PiMZ) is also associated with an increased risk for liver diseases. Hepatocarcinogenesis for AAT-deficiency is probably based on a series of toxic events. Precipitation of AAT aggregates in hepatocytes is the initial step. These accumulate in the endoplasmic reticulum and cannot be eliminated from all hepatocytes by proteasomal and non-proteasomal mechanisms. AAT aggregates induce proinflammatory pathways and may be a stimulus for hepatocarcinogenesis. This hypothesis is based mostly on studies of individuals homozygous for a deficiency allele (PiZZ). The mechanism may also play a role in heterozygous patients. Since not all patients with precipitates of AAT-aggregates are develop a hepatocellular carcinoma related comorbidities such as chronic hepatitis B, C, chronic alcohol abuse, or so far unknown genetic and environmental factors may be crucial., (© Georg Thieme Verlag KG Stuttgart · New York.)

Nodular regenerative hyperplasia (NRH) is an uncommon cause of portal hypertension. The disease is often associated with rheumatological or lymphoproliferative disorders. We report the case of a 19-year old patient with celiac disease and portal hypertension. Biopsy of the liver revealed NRH as the underlying cause. There were no signs of autoimmune diseases or defects in the coagulation system that might cause NRH. Celiac disease is often associated with liver abnormalities, but the association with NRH has rarely been described. We discuss the possible relationship of celiac disease and NRH.

The liver is essential for metabolism of drugs and exogenous toxins. Therefore, liver toxicity due to natural, domestic and industrial toxins or drugs is common but rarely recognised. Although thorough epidemiological data are missing, the number of cases with drug-induced liver toxicity is increasing, parallel to the growing number of drugs. Adverse hepatic reactions may present as acute or chronic liver damage. Independent from the cause and mechanism of liver damage, the clinical presentation may be either cytolytic or cholestatic. In addition, liver damage may be accompanied by a systemic hypersensitivity reaction against the applied agent. Drug- or toxin-induced liver damage includes vascular damage, induction of liver tumours and development of liver cirrhosis. The prerequisite for specialised treatment of drug-induced adverse hepatic reactions is establishing the diagnosis which is obtained by a thorough medical history taken by an experienced physician with a special emphasis on drug or toxin exposure. The diagnosis may be confirmed by additional diagnostic measures (e. g. liver biopsy) or the clinical course after exposure to the causing agent has been stopped. Only in a minority of cases a definite treatment may be available. Therefore, the main aim is to prevent chronic liver damage through early and correct diagnosis. Due to the extensive variety of possibly liver toxic drugs and chemical agents this aspect is a major challenge to physicians.

Benign hepatic tumours include a broad spectrum of regenerative and true neoplastic processes. Due to advances in imaging procedures like MRI, Cf-scan and ultrasound as well as progress in immunohistochemistry, the appropriate diagnosis is made ina high percentage of patients without laparotomy and resection. Most important in clinical practice is the differential diagnosis of focal nodular hyperplasia and hepatocellular adenoma because of the risk of rupture and bleeding in the latter. Cavernous haemangioma, the most common benign hepatic tumour, rarely needs treatment. The diagnosis of nodular regenerative hyperplasia is often missed and patients present with secondary complications and signs of portal hypertension that necessitate treatment. The main problem in angiomyolipoma is to distinguish it from malignant processes which do require treatment. Because of its clinical presentation, inflammatory pseudotumour is also sometimes confused with a malignant tumour. Therapeutic options are drug therapy or surgical resection. Benign haemangioendothelioma of the infant is rare but may cause life-threatening complications. Bile duct adenoma is an incidental finding that is not known to cause any symptoms whereas biliary cystadenoma is often symptomatic and may progress to cystadenocarcinoma and therefore needs resection.

A 22-year-old Libyan patient suffering from chronic diarrhea presented with an alpha-heavy chain paraprotein and a lympho-plasmacellular lymphoma infiltration of the duodenal mucosa. These findings supported the diagnosis of "immunoproliferative small intestinal disease" (IPSID). In this disease, that occurs almost solely in countries with low socioeconomic status, a diffuse infiltration of small intestinal mucosa by neoplastic lymphoid cells causes chronic malabsorption. About 65% of patients exhibit a paraprotein in serum, urine or jejunal juice that consists of the heavy chain of immunoglobulin A (alpha-heavy chain). In advanced stages, IPSID resembles histologically and clinically high grade lymphoma: some patients develop masses in the gut wall, an abdominal lymphadenopathy and involvement of other organs including bone marrow. The disease is believed to be triggered by a chronic infectious antigenic stimulus. Thus, in early stages in some patients cure may be achieved by antibiotic therapy alone. In advanced disease, chemotherapy including anthracyclins is necessary.