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Epithelioid Hemangioendothelioma: A Rare Case of an Aggressive Vascular Malignancy

Challenging differential diagnosis, Unusual or unexpected effect of treatment, Rare disease

Meghan Lytle, Sunil Daniel Bali, Yehuda Galili, Brittany Bednov, Rodrigo M. Murillo Alvarez, Stephen J. Carlan, Mario Madruga

Department of Internal Medicine, Orlando Regional Healthcare, Orlando, FL, USA

Am J Case Rep 2019; 20:864-867

DOI: 10.12659/AJCR.915874

Available online:

Published: 2019-06-18

BACKGROUND: Epithelioid hemangioendothelioma (EHE) is a rare vascular malignancy that occurs in multiple organs and tissues with a predilection for the extremities, bone, liver, and lung. It is often characterized by a clinically indolent course, delayed diagnosis, and unestablished standardized treatment options.
CASE REPORT: A 46-year-old female presented with a 2-month history of right shoulder and arm pain. Imaging revealed involvement of the humerus, lung, liver; and brain and biopsies of both the lung and humerus were performed. A diagnosis of epithelioid hemangioendothelioma was confirmed and the patient received radiation therapy to the right humerus and brain stereotactic radiosurgery. She was scheduled to begin palliative chemotherapy with doxorubicin but developed complications and never received chemotherapeutic agents. She died 5.5 months from her first admission with widespread metastasis.
CONCLUSIONS: Epithelioid hemangioendothelioma (EHE) is typically a low-to-intermediate grade vascular malignancy, but, as seen in this case, can be aggressive. In the future, diagnosis, prognosis, and treatment may improve using genetic or immune therapy considering a structural chromosomal translocation has been identified.

Keywords: Bone Diseases, Lung Neoplasms, Vascular Diseases