title: Pleural epithelioid hemangioendothelioma: literature summary and novel case report.
abstract: 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.
authors.affiliation: Imperial College Healthcare NHS Trust, Charing Cross Hospital, Fulham Palace Road, London W6 8RF, UK.
authors.forename: Julita
authors.initials: J
authors.lastname: Salijevska
authors.affiliation: Imperial College Healthcare NHS Trust, Charing Cross Hospital, Fulham Palace Road, London W6 8RF, UK.
authors.forename: Robert
authors.initials: R
authors.lastname: Watson
authors.affiliation: The North West London Hospitals NHS Trust, Northwick Park Hospital, Watford Road, Harrow, Middlesex, HA1 3UJ, UK.
authors.forename: Amy
authors.initials: A
authors.lastname: Clifford
authors.affiliation: The North West London Hospitals NHS Trust, Northwick Park Hospital, Watford Road, Harrow, Middlesex, HA1 3UJ, UK.
authors.forename: Andrew I
authors.initials: AI
authors.lastname: Ritchie
authors.affiliation: Imperial College Healthcare NHS Trust, The Hammersmith Hospital, Du Cane Road, London W12 0HS, UK.
authors.forename: Francisco
authors.initials: F
authors.lastname: Mauri
authors.affiliation: The North West London Hospitals NHS Trust, Northwick Park Hospital, Watford Road, Harrow, Middlesex, HA1 3UJ, UK.
authors.forename: David
authors.initials: D
authors.lastname: Adeboyeku
keywords: Epithelioid hemangioendothelioma; Intravascular bronchoalveolar tumor; Pleura; Pulmonary hemangioendothelioma