Sirolimus combined with interferon-alpha 2b therapy for giant hepatic epithelioid hemangioendothelioma: a case report

Abstract

Hepatic epithelioid hemangioendothelioma (HEH) is a very rare tumor originated from vascular endothelial cells, with unpredictable malignancy. No standard treatment has been established yet. Although surgical resection and liver transplantation have been reported to be effective treatments with favorable long-term outcomes, the multiple intrahepatic lesions or extrahepatic metastasis makes these procedures unsuitable to most patients. Sirolimus was reported to be an effective drug for epithelioid hemangioendothelioma but only about 10% achieved partial response. Interferon-alpha 2b (IFN-a 2b) has also been used for the treatment of HEH, and the rate of tumor regression was more than 50%. Here, we report a HEH patient with giant intrahepatic tumor (>15cm), who achieved partial response after the combined therapy of sirolimus and IFN-a 2b. The giant intrahepatic lesion (>15 cm) regressed obviously after 8 months treatment and no severe adverse event was reported. The good response and safety of combined therapy with sirolimus and IFN-a 2b provide a promising guidance for future clinical study.

Keywords: case report; epithelioid hemangioendothelioma; interferon; liver; sirolimus.

Figure 1

T2-weighted images of liver MRI (TR/TE: 7059/85 ms; field of view: 44 × 40 cm; matrix: 320 × 224; thickness: 8 mm), which showed a giant heterogenous high signal intensity tumor mainly located in the left lobe (A, B, marked with arrows) and multiple small high signal intensity lesions in the right lobe (C, D, marked with arrows).

Figure 2

Contrast-enhance images of liver MRI (dose: 0.1 mmol/kg; injection rate: 2.0 ml/s; TR/TE: 3.6/1.7 ms; slice thickness: 5 mm; image matrix: 256 × 192; field of view: 40 × 44 cm; the scanning delay times were 20s, 60s and 180s for the arterial, portal and delay phases, respectively), which showed the involvement of right branch of portal vein (A, B, marked with arrows) and right hepatic artery (C, marked with arrow).

Figure 3

Hematoxylin & eosin and immunohistochemical staining of the liver biopsy, which showed spindle-shaped tumor cells and epithelioid tumor cells (A, hematoxylin & eosin, ×200), and positive for CD 31, CD34, ERG and Fli-1 (B-E, respectively, ×200). The index rate of Ki-67 was 20% (F, ×200).

Figure 4

Comparison of the largest lesion on MRI before (A, D) and after the combined therapy of sirolimus and IFN-a 2b (B, C, E, F). The tumor (marked with black arrows) gradually regressed and the involved right branch of portal vein (marked with white arrows) got released from the tumor.