KAPOSIFORM HEMANGIOENDOTHELIOMA OF BONE WITH NO CUTANEOUS LESION IN CHILDREN - A CASE REPORT

Abstract

Kaposiform hemangioendothelioma (KHE) particularly of the bone is a rare malignant vascular tumor. Though a few case reports have reported imaging findings of KHE, but no particular local data has been documented to best of our knowledge in our region. We present 2 years old patient, who presented with pain in the right shoulder, clinically diagnosed with Erb’s palsy. Before presenting to us he has had a biopsy from outside our hospital for a lytic lesions in the right proximal humerus, which was reported to be fibro-osseous tissue with florid vascular proliferation. MRI was performed again and possible differentials included were malignant neoplastic osseous lesion such as Ewing sarcoma and osteomyelitis. Tissue sampling was advised again. Therefore, re-biopsy via and open biopsy was performed and specimen was submitted for pathologic evaluation which revealed this to epithelioid hemangioendothelioma (EH). The patient went through chemotherapy followed by excision of tumor. Plastic surgery team was taken on board for the graft and aesthetic reasons. Kaposi hemangioendothelioma occurs in variable location and affects mostly infant. We discussed the features of KHE which are essential to know for a prompt diagnosis by radiologists. It can cause morbidity and mortality in children and adults. Consequently, prompt diagnosis and appropriate management are crucial to improving the long-term prognosis of patients.