Patient with liver epithelioid hemangioendothelioma treated by transplantation: 3 years' observation

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.