Epithelioid Hemangio Endothelioma, an ultra-rare tumor.
Epithelioid hemangioendothelioma (EHE) is a very rare vascular tumor. It exhibits intermediate malignancy between hemangioma and angiosarcoma and originates from endothelial cells lining of the blood vessels. EHE is notable for its distinctive epithelioid endothelial cell morphology. Its mainly present in soft tissues or any organ.The disease commonly presents in young adults with a slight female predominance. Symptoms vary, mostly asymptomatic, but can present in aggressive forms with metastasis, often manifesting as pain or unexplained weight loss. WWTR1(TAZ)-CAMTA1 gene fusion is seen in over 90% of cases. Diagnosis is challenging due to its rarity and requires histological and immunohistochemical analysis, including markers like CD31, ERG, and CD34. There is no standardized treatment, with surgery, targeted therapy, chemotherapy, and radiation being used variably. Prognosis depends on disease spread, with survival ranging from months to decades. Though various chemo regimens have been tried post-surgery in literature, for her we are using VEGF inhibitor Bevacizumab as the tumor is of endothelial origin. A post-chemotherapy status with a one-year follow-up will reveal its efficacy.
Keywords: WWTR1(TAZ)-CAMTA1, Bevacizumab, VEGF INHIBITOR, CD31, CD34
