Abstract

Hepatic epithelioid hemangioendothelioma (HEH) is extremely rare, occurring in 1 to 2 per 100,000, with chemotherapy options not well defined. Our case involved a 49-year-old female who had hepatic masses and metastasis to the lungs with a liver biopsy revealing HEH. After developing a rash from sorafenib, thalidomide was started with the progression of disease stabilized. Resection is only an option in 10% of the cases; therefore, chemotherapy is the only line of treatment. Newer chemotherapy alternatives are targeting angiogenesis via the vascular endothelial growth factor. Thalidomide was first used as an antiemetic, but, sadly, soon linked to phocomelia birth defects. Given the mechanism of action against angiogenesis, thalidomide has a valid role in vascular tumors. In conclusion, the use of thalidomide as chemotherapy is novel and promising, especially in the setting of a rare vascular liver tumor such as HEH.

Highlights

  • Vascular in origin, hepatic epithelioid hemangioendothelioma (HEH) is an extremely rare diagnosis; treatment for this malignancy has not been well defined

  • We present a female diagnosed with metastatic HEH who has been successfully treated with thalidomide chemotherapy

  • The term epithelioid hemangioma was coined by Weiss and Enzinger in 1982 to describe a distinct entity, a soft tissue vascular tumor of endothelial origin with a clinical course intermediate between benign hemangioma and malignant angiosarcoma [1]

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Summary

Introduction

Hepatic epithelioid hemangioendothelioma (HEH) is an extremely rare diagnosis; treatment for this malignancy has not been well defined. In the mid-twentieth century, thalidomide was used for morning sickness in pregnant women, and their babies developed well-documented phocomelia birth defects. From those horrific malformations, continued investigation into the explanation of thalidomide involvement has led to a better understanding of its harmful effects on angiogenesis. With this discovery, the careful use of the medication has been employed as chemotherapy in cancers such as HEH. The careful use of the medication has been employed as chemotherapy in cancers such as HEH In this case report, we present a female diagnosed with metastatic HEH who has been successfully treated with thalidomide chemotherapy

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