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Year : 2016  |  Volume : 6  |  Issue : 1  |  Page : 60-62

Epithelioid hemangioendothelioma of the femur

Department of Onco Surgery, Cancer Research Institute, Swami Rama Himalayan University, Dehradun, Uttarakhand, India

Correspondence Address:
Dr. Neha Jindal
Department of Onco Surgery, Cancer Research Institute, Swami Rama Himalayan University, Jollygrant, PO - Doiwala, Dehradun - 248 140, Uttarakhand
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2278-9596.187195

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Epithelioid hemangioendothelioma (EHE) of the bone is extremely uncommon and only 0.01% of the cancer population has it. It is characterized by epithelioid endothelial cells and has variable biological behavior. Because the behavior of these tumors is intermediate between angiosarcoma and hemangioma, it is important to not misdiagnose EHE as an angiosarcoma. EHE typically occurs in patients of 20-40 years of age with no sex predilection. Approximately, half of EHE is present with multifocal disease. Here, we describe the case of a 60-year-old female who presented with pain and swelling of the right thigh. Contrast Enhanced Computed Tomography (CECT) of the right thigh revealed multiple lytic lesions in the right femur with cortical destruction with multiple peripherally enhancing lesions in the muscular plane along the shaft of the right femur. Histopathology report from bone tissue showed connective tissue tumor with CK, Vimentin, CD31, and Fil-1 positivity on immunohistochemistry (IHC). In view of multiple lytic lesions of bone, right hip disarticulation was done instead of salvage surgery of the limb. The tumor is locally aggressive and has high rates of local recurrence. Thereby, necessitating aggressive local treatment which is mostly surgical. These tumors are not generally chemo and radiosensitive, hence these modalities cannot be used as adjuvant to incomplete surgery or local recurrence. IHC plays a valuable role in characterizing these tumors due to its variable biological behavior.

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