Key result
Epithelioid hemangioendothelioma expressing CAMTA-1 arises within a mediastinal myelolipoma in a 51-year-old woman.
Case Report (n=1)
This is the first report of a malignant vascular neoplasm (epithelioid hemangioendothelioma) arising in association with a mediastinal myelolipoma.
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Documents an unprecedented EHE-myelolipoma association; leaves open diagnostic implications pending additional cases.
In this article, we describe a case of conventional epithelioid hemangioendothelioma (EHE) arising within an extra-adrenal myelolipoma. This composite neoplasm arose in the mediastinum of a 51-year-old female. The tumor was composed of a large myelolipoma that contained nodules of EHE consisting of CD31-positive epithelioid endothelial cells that grew in solid cords and were enmeshed in a basophilic hyalinized stroma. Both EHE and myelolipoma are characterized genetically by alterations of WWTR1. We demonstrated the expression of CAMTA-1 chimeric protein by immunohistochemistry both in the neoplastic endothelial cells of EHE and some of the endothelial cells lining the blood vessels in the myelolipoma. To the best of our knowledge, this is the first report of a malignant vascular neoplasm arising in association with myelolipoma.
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Díaz-Pérez et al. (2019) conducted a case report in Epithelioid hemangioendothelioma arising within a mediastinal myelolipoma (n=1). A conventional epithelioid hemangioendothelioma arising within a mediastinal myelolipoma was identified in a 51-year-old female, demonstrating CAMTA-1 chimeric protein expression.
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