ABSTRACT
The clinical manifestations of HEHE are unspecific, varying from totally asymptomatic to hepatic failure ( Table 26.1 ). The malignant potential of HEHE often remains unclear in each individual patient. Based on the analysis of the ELITA-ELTR study, the most frequent symptoms are non-specific upper abdominal or epigastric discomfort or pain, weakness, general malaise, and jaundice. About 20% of patients are asymptomatic and 10% present with pulmonary symptoms ( Table 26.1 ). Hepato-splenomegaly (50%) and weight loss (20%) are the most frequent clinical signs; portal hypertension may be caused by tumor compression or venous infiltration ( 3-6 ).
