KAPOSIFORM HEMANGIOENDOTHELIOMA: 2 RARE CASES REPORT OF THE VIETNAM NATIONAL CHILDREN’S HOSPITAL

Hoang Ngoc Thach1, Le Thi Xuan Lai2, Le Thi Ha1, Dang Hoang Thom1, Bui Ngoc Lan1
1 Vietnam National Children’s Hospital
2 Hue Central Hospital

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Abstract

Kaposiform hemangioendothelioma (KHE) is a rare and locally aggressive endothelial neoplasm in children, progresses rapidly, able to cause severe anemia. The tumours can occur in soft subcutaneous tissue and noncutaneous sites such as the liver and pancreas. We report two cases of a male and a female infant with a large tumour in the right arm - forearm area, both prenting with thrombocytopenic anemia. Needle biopsies were performed, combined histology and IHC to confirm the diagnosis of KHE. Medical treatment with Vincristine, Aspirin and Propranolol has led to good result, demonstrated by the tumours stopping progression and reducing volume soon after the first treatment course.

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