Sarcomatous Transformation of Unicentric Calcaneal Epithelioid Hemangioendothelioma: A Case Report with Review of Literature

Abstract

Vascular origin tumors of bone are rare entities. Epithelioid hemangioendothelioma (EHE) is a rare tumor representing <1% of primary bone tumors and is considered a low to intermediate grade malignancy. Literature describes cases which were either a part of multicentric disease or were metastatic. In this study, we reported a case of a 23-year-old female with biopsy-proven EHE with prior history of resection, presenting with recurrence of disease. Radiographs showed a lytic lesion involving the left calceneum and other tarsal bones with extensive soft-tissue component. Magnetic resonance imaging revealed an ill-defined, enhancing, lobular, and lytic lesion with adjacent soft-tissues component. Computed tomography confirmed extensive destruction of the involved tarsal bones. EHE is treated with wide surgical excision and recurrence is rare; however, our case presented with recurrence as well as thoracic metastasis which posed a great clinical challenge. The patient was treated with below knee amputation and was given subsequent radiotherapy. The main aim of this study is to revisit the clinicopathological aspects as well as the spectral behavior of vascular bone tumors.