Hepatic hemangioendothelioma - A rare case report

Abstract

Introduction and importance: Hepatic hemangioendothelioma (HHE) is a very rare mild-moderate malignant tumor of the hepatic vascular endothelium. The etiology is yet not fully understood. Patients can be asymptomatic or may present with non-specific symptoms or hepatic insufficiency. CT and MRI scans show various radiographic features but a definitive diagnosis can be made with histological analysis and immunohistochemistry. Here we report a case of a patient who presented with a non-resectable disease for which living donor liver transplantation was done.

Case presentation: A 19-year-old female patient presented with intermittent moderate dull pain in the right hypochondrium for 9 months. Examination revealed mild tender hepatomegaly. On abdominal ultrasound, two hypoechoic lesions were noted in both lobes of the liver that were confirmed on CT scan. Histologic examination of the ultrasound-guided fine-needle aspiration cytology of the voluminous right lobe lesion clinched the diagnosis of HHE. The patient underwent successful living donor liver transplantation due to the multifocal bi-lobar nature of the lesions. At 8 months follow up, she is fine and doing well.

Conclusion: HHE is a very rare mild-moderate malignant tumor of hepatic vascular origin. Resection is the preferable treatment option in patients with resectable disease. However, liver transplantation has become the treatment of choice for patients with non-resectable multifocal and bi-lobar lesions. The long-term outcome of this malignancy is not fully known and there is a need for long-term follow-up studies to determine the actual recurrence rate of this disease.

Keywords: Diagnostic imaging; Hepatic hemangioendothelioma (HHE); Liver transplantation (LT); Resection; Vascular tumor.

Fig. 1

CT scan liver showing two hypodense lesions in the arterial phase.

Fig. 2

a Low power b Medium power.