Epithelioid hemangioendothelioma in the thorax: Clinicopathologic, CT, PET, and prognostic features

Rare Pleural Tumors

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare, often misdiagnosed vascular neoplasm with clinical behaviors that range from indolent to highly aggressive. Even when the appropriate diagnosis is achieved, the best treatment for HEHE has not been defined or standardized, further complicating the care of these patients. We present a diagnostically challenging case of HEHE where we utilized capecitabine and bevacizumab as another novel treatment option.

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Combination capecitabine and bevacizumab in the treatment of metastatic hepatic epithelioid hemangioendothelioma.

Pleural epithelioid hemangioendothelioma harboring CAMTA1 rearrangement

Epithelioid hemangioendothelioma (EHE) is a rare malignant cancer of vascular origin that can affect multiple and varied tissue sites. A subtype of EHE, pulmonary epithelioid hemangioendothelioma (PHE), is more unusual with only 200 reported cases. Of these, only 27 have been classified as pleural in origin. Based on available literature, the average age of presentation of pleural PHE is 45.7 years with a male preponderance of 2.375. A summary of all published case reports reveals significant heterogeneity both in presentation and management. Here we add to this knowledge-base with a report of an unusual case of pleural PHE in a 36-year-old female who presented with a 6-week history of chest pain and breathlessness. Significant challenges in the diagnosis and management of patients with pleural PHE exist, including a wide initial differential diagnosis and difficulties in obtaining tissue specimens, coupled with relatively limited treatment options. Early referral to a cardiothoracic center for video-assisted thoracoscopic biopsy is crucial in facilitating a diagnosis and allowing adequate pleural drainage for symptomatic relief. J Clin Med Res. 2015;7(7):566-570 doi: http://dx.doi.org/10.14740/jocmr2174w

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Angeles Montero-Fernández

Papers

Pleural epithelioid hemangioendothelioma in an elderly patient. A case report and review of the literature.