Hepatic vascular neoplasms are a growing problem in gastroenterology practice due to the widespread use of new imaging techniques. Hepatic epithelioid hemangioendothelioma (HEH) is a rare vascular tumor of endothelial origin with an incidence of < 1 case per million.1 It was first described by Weiss and Enzinger in 1982.2 The clinical presentation is nonspecific, and right upper quadrant pain is the most common symptom. This tumor is most common in women between the second and fourth decades of life.3 Liver function tests are normal in most patients. In advanced cases, mild elevation of alkaline phosphatase and gamma-glutamyl transferase levels may be observed. Levels of other tumor markers such as alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9 usually are normal. The HEH usually is multifocal with variable nodule size. In advanced cases, the tumor can spread diffusely into the entire liver parenchyma. Definitive diagnosis requires histopathologic examination.4 The prognosis of HEH can vary, although it is known for its low malignant potential, and primary treatment usually is surgical.5,6
We report a case of HEH that was treated successfully with orthotopic liver transplant. A 48-year-old man was referred to our transplant unit because of multifocal hepatic lesions that were found incidentally on an abdominal ultrasonogram. He had right upper quadrant pain. Laboratory investigation revealed levels of hemoglobin 14.4 g/dL, white blood cell count 15.9 × 109/L, platelet count 428 × 109/L, aspartate aminotransferase 44 U/L (reference, 5-34 U/L), alanine aminotransferase 42 U/L (0-55 U/L), total bilirubin 0.5 mg/dL (0.2-1.2 mg/dL), alkaline phosphatase 119 U/L (35-150 U/L), gamma-glutamyl transferase 116 U/L (10-66 U/L), prothrombin time 16.1 seconds, and international normalized ratio 1.27. Levels of the tumor markers alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9 were normal. Abdominal computed tomography scan revealed multiple confluent hypodense liver masses (Figures 1 and 2). A percutaneous liver biopsy was performed, and histopathologic examination showed HEH. Living-donor liver transplant was performed successfully. The patient was discharged home on postoperative day 15 with tacrolimus and prednisone as maintenance therapy. Prednisone was stopped 12 months after transplant. He has not had an episode of acute rejection, and allograft function remains normal. The patient has been followed in our outpatient clinic for > 30 months, and he has not had recurrence.
Epithelioid hemangioendothelioma is a rare, low-grade malignant vascular tumor. It may involve the lung, spleen, bone, brain, meninges, breast, heart, stomach, and lymph nodes.7-9 Although imaging studies such as computed tomography or magnetic resonance imaging may show highly vascular lesions, no imaging features are pathognomonic. Histo-pathologic examination is required for definitive diagnosis.
There is no standard treatment protocol for HEH. Surgery, either resection or transplant, has been recommended as curative treatment. However, resection cannot be performed in 87% patients. Most patients have bilobar involvement of the liver, and extrahepatic disease is present in 36.6% patients. Due to its low malignant potential, extrahepatic disease and lymph node involvement are not cont-raindications for transplant. The 3-year survival rate after liver transplant is > 80%. Chemotherapy with or without radiotherapy and transarterial chemo-embolization are other treatment options, but HEH may be nonsensitive to radiotherapy and chemotherapy.3,10
In conclusion, HEH is an appropriate indication for liver transplant, even in patients with advanced disease.
Volume : 13
Issue : 3
Pages : 298 - 299
DOI : 10.6002/ect.2014.0235
From the Departments of 1Gastroenterology and 2Transplantation,
Medicana International Hospital, Ankara, Turkey
Acknowledgements: The authors have no conflicts of interest to declare. No funding was received for this study.
Corresponding author: Reskan Altun, MD, Medicana International Ankara Hospital, Department of Gastroenterology, Söğütözü Mah. 2165, Sok No. 6, 06520 Söğütözü, Ankara, Turkey
Phone: +90 312 292 92 92
Fax: +90 312 220 31 26
Figure 1. Abdominal CT Scan Revealed Multiple Confluent Hypodense Liver Masses
Figure 2. Abdominal CT Scan Revealed Multiple Confluent Hypodense Liver Masses