Introduction: Epithelioid hemangioendothelioma (EHE) of liver is a very rare primary tumor with a variable course of disease. A case is presented of a 70-years-old male patient with large size hepatic EHE (20.1 x 14.7 x 20.7 cm) with rupture.
Methods: The patient received a living donor liver transplantation as modified right liver from his son.
Results: Preoperative biopsy revealed no definite diagnosis, and tumor markers were not elevated before surgery. Preoperative PET CT showed no distant metastasis and no malignant tumors in other organs. Hepatic resection could not be considered because the size of the tumor was very large and hepatic failure was progressed. Model for end-stage liver disease score was 25 points. There was no intra- and post-operative complication. There were no abnormal findings in liver dynamic computed tomography performed 1 week and 3 weeks after surgery. However, three months later, multiple peritoneal nodules were observed on liver dynamic computed tomography, and distant metastasis were observed on vertebrae T3, T10, left 7th rib and iliac bone on positron emission tomography.
Conclusion: EHE of the liver is characterized by very huge and ruptured, which excludes resection; thus, liver transplantation is the method of choice. However, in the case of EHE with clinically malignant features, recurrence and distant metastasis may occur very early in immune suppressed patients, so evaluation of accurate diagnosis through tissue examination before surgery and short term follow-up after surgery are very important.