Epithelioid hemangioendothelioma (EHE) is a rare
neoplasm of vascular origin, but unknown etiology that occurs in the liver, lungs and other organs. Its hepatic form (HEHE) generally behaves as a low-grade malignant
tumor with a slowly progressive phenotype. Surgical resection or
liver transplantation (OLT) has been recommended after diagnosis. We present a 30-year-old woman with primary HEHE of the liver treated by OLT in 2002. Her medical history started 3 years prior when an abdominal ultrasound examination revealed multiple focal changes in the liver. The histopathological diagnosis from a needle biopsy was
carcinoma cholangiogenes desmoplasticum. For 2 years the patient was treated with
chemotherapy combinations. To explain the lack of efficacy of
chemotherapy, a laparoscopic biopsy was performed and HEHE diagnosed. Immunohistochemistry revealed positive staining for the
factor VIII-related
antigens, CD34 and CD31, which have been previously described as HEHE markers. The patient underwent OLT in March 2002. In the first month after OLT, the
thyroid stimulating hormone concentration was elevated but they continuously decreased from 11.4 to 2.4 uIU/mL in May 2002 and thereafter remains normal. After 3 years observation the patient presented with good liver function and no signs of
tumor recurrence. We concluded that immunohistochemical staining for characteristic endothelial cell markers may facilitate the correct diagnosis of HEHE. After diagnosis, OLT followed by immunosuppressive therapy, consisting of
basiliximab,
corticoids, low doses of
tacrolimus and temporary administration of
rapamycin, may be safe and effective. Monitoring of
thyroid-stimulating hormone concentrations should be performed in patients with HEHE.