Sorafenib Monotherapy in a Patient with Unresectable Hepatic Epithelioid Hemangioendothelioma

Abstract

A 49-year-old Japanese man had multiple huge masses (max. size 60 mm diameter) in his liver. These tumors were pathologically diagnosed by tumor biopsy as epithelioid hemangioendotheliomas of the liver. In this case, multiple liver tumors existed in both lobes. Also this patient did not agree to receive surgical resection including liver transplantation. Chemotherapy with sorafenib at a dose of 400 mg/body twice a day was started. About 6 months later, CT findings revealed that these tumors were shrinking slightly; 33 months later, the tumors obviously showed a partial response in accordance with the Response Evaluation Criteria in Solid Tumors (RECIST). Also 60 months later, the partial response continued with sorafenib monotherapy.

Hepatic epithelioid hemangioendothelioma successfully treated with living donor liver transplantation: A case report and literature review

“…Additional treatment after LT may be necessary for advanced HEH, such as the cases with vascular invasion. Successful treatment with chemotherapy has been reported, using sorafenib and thalidomide, doxorubicin, cyclophosphamide, and interferon . However, all reports have a small number of cases, and the effect as an established adjuvant therapy after surgery is uncertain.…”

“…Microvascular or combined macro-microvascular invasion have been reported as prognostic factors, and the pre-LT waiting time (120 days or less) and hilar lymph node invasion were reported as risk factors for recurrence after LT. 16 Additional treatment after LT may be necessary for advanced HEH, such as the cases with vascular invasion. Successful treatment with chemotherapy has been reported, using sorafenib 17,18 and thalidomide, 19 doxorubicin, 20 cyclophosphamide, 21 and interferon. 22 However, all reports have a small number of cases, and the effect as an established adjuvant therapy after surgery is uncertain.…”

Diffuse hepatic epithelioid hemangioendothelioma with multiple splenic metastasis and delayed multifocal bone metastasis after liver transplantation on FDG PET/CT images

“…Some studies reported that the use of sorafenib took more advantages over other anti-angiogenic agents because of its dual anti-tumor activity. [20] In a case report, Kobayashi et al [21] used sorafenib monotherapy in a patient with mutiple unresectable lesions, who did not agree to receive surgical resection including liver transplantation. About 6 months later, CT findings indicated that these tumors were shrinking slightly; even 60 months later, the partial response was still observed with sorafenib monotherapy.…”

Apatinib for the treatment of pulmonary epithelioid hemangioendothelioma

“…Durable stabilization was also observed in sorafenib therapy, with partial response for 5 years and stable disease in almost 2 years being observed in previous reports. [ 68 , 69 ] In a phase 2 study of progressive EHE patients treated with sorafenib, the 2-, 4-, 6-, and 9-month progression-free survival was 84.6% (11 of 13), 46.4% (6 of 13), 38.4% (5 of 13), and 30.7% (4 of 13), respectively. [ 70 ] In contrast, only 3 EHE patients who were treated with sunitinib have been previously reported, with stabilization lasting about 10 months, 2 years, and 6 years, respectively.…”