Surgical treatment of asymptomatic epithelioid hemangioendothelioma originating from the superior vena cava

Abstract

Rationale: Epithelioid hemangioendothelioma is a rare endothelial tumor with a low-grade malignancy. This tumor can be treated with complete resection. Patient concerns: A 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. Diagnosis: Chest 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. Interventions: We 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. Outcomes: Fourteen days later, the patient was discharged without any complication. Thirty months after surgery, recurrences, or metastasis were not detected. Lessons: Epithelioid 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.

Case report: Surgical treatment of a primary giant epithelioid hemangioendothelioma of the spine with total en-bloc spondylectomy

“…The extent of surgical can range from wide margin to marginal or intralesional resection. Several clinical studies suggest that wide resection (i.e., TES), should be performed to achieve favorable outcomes ( 14 ). Luzzati et al.…”

Hemangioendotelioma cardiaco primario recurrente: reporte de caso y revisión de la literatura

“…La piedra angular del tratamiento es la resección quirúrgica a nivel del corazón y la vena cava; cuando se realiza de forma oportuna tiene un excelente desenlace. [30][31][32][33][34] Por otro lado, la efectividad de las terapias adyuvantes posoperatorias de rutina sigue siendo muy controversial, por lo cual estas representan un desafío en la práctica clínica actual. Aunque se ha visto que la terapia adyuvante agresiva mejora la supervivencia a largo plazo después del tratamiento quirúrgico de los sarcomas de la arteria pulmonar y el corazón.…”

Rare epithelioid hemangioendothelioma in the brachiocephalic vein for long-term survival after surgery: A case report

“…1 EHE usually originates from the cells of the blood vessels; however, EHE rarely originates in the large arteries and veins, with only a few cases reported. 4–7 Although it is asymptomatic, edema and thrombophlebitis occur depending on the site of occurrence. Diagnosis requires pathological examination of the tumor and histological findings that include nests and cords of epithelioid endothelial cells distributed in a myxohyaline stroma.…”

“…Several reports suggest that reconstruction was performed with an artificial blood vessel after tumor resection, but in our case, since it was a bifurcation lesion and there was a risk of thrombosis in the artificial blood vessel, reconstruction with a patch was performed. 7,11 The tumor invaded the microscopic surgical margin; however, complete resection was considered possible. Six years after the surgery, no recurrence has been observed.…”