• Seok Soo Lee and Jang Hoon Lee.
• Department of Thoracic and Cardiovascular Surgery, Yeungnam University Medical Center.
• Medicine (Baltimore). 2020 Apr 1; 99 (16): e19859.

RationaleEpithelioid hemangioendothelioma is a rare endothelial tumor with a low-grade malignancy. This tumor can be treated with complete resection.Patient ConcernsA 20-year-old Korean man visited our hospital due to an abnormal finding on standing chest PA X-ray. He did not have any past medical history.DiagnosisChest computed tomography shows a well-defined, oval-shaped tumor invading the brachiocephalic vein and superior vena cava. A malignant tumor of vascular origin was diagnosed by a percutaneous needle biopsy.InterventionsWe performed en-bloc resection including the great vessels for complete resection of the tumor. Histologic evaluation confirmed the lesion to be a hemangioendothelioma and the surgical margins were free from tumor invasion.OutcomesFourteen days later, the patient was discharged without any complication. Thirty months after surgery, recurrences, or metastasis were not detected.LessonsEpithelioid hemangioendothelioma is a rare malignant endothelial tumor in the central vein. Surgery is the treatment of choice and shows good results. We introduce and appropriate surgical method to ensure successful treatment for rare disease.

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