Primary Renal Epithelioid Hemangioendothelioma

Abstract

Background: Epithelioid hemangioendothelioma (EH) is a rare vascular neoplasm with an unpredictable malignant potential that has been described mainly in soft tissue, liver, and lung. Case Report: We report a case of a primary renal EH in a 54-year-old woman who presented with a 3-month history of abdominal pain. Abdominal ultrasound and computed tomography scan revealed a solitary mass of 4.0 ×3.2 cm in the outer surface of the right kidney. Nephron-sparing surgery was performed. On the basis of the histological and immunohistochemical findings, the diagnosis of EH was made. The patient was free of disease 6 months after surgery. Conclusions: Although EH may have a histologically benign appearance and can be treated using a nephron-sparing approach, this neoplasm is potentially malignant urging the need for a long-term follow-up in these patients.

Epithelioid Hemangioendothelioma of the Kidney Treated with Sunitinib

“…Epithelioidhemangioendotheliomaisararemalignantvasculartumor,arisingintheendothelialliningofvessels.Themost commonlocationofaprimaryepithelioidhemangioendotheliomaistheliver,andinmanycasesthetumorismultifocalat the time of diagnosis so that liver resection or hepatectomy andtransplantationcouldbetheonlycurativeprocedureand theprocedureofchoiceevenincasesofextrahepaticdisease [4,5].Aprimarylocationinthekidneywithsurgicalexcision ofalocalizedtumorandanuneventfulfollow-uphasbeendescribedinonly1publication [1].Thalidomide,paclitaxeland doxorubicin,adriamycin,andinterferon-2-alphahaveallbeen reportedincasereportsassystemictherapiesforpatientswith epithelioid hemangioendothelioma [6][7][8][9], but with uncertain resultsgiventhesmallnumberofreports.…”

“…However, primary location of the tumor in the kidney has been shown.Sofar,noeffectivesystemictreatmentformetastatic diseasehasbeendescribed [1].Wereportacaseofmetastatic epithelioid hemangioendothelioma primarily located in the kidneyhighlysensitivetotreatmentwithsunitinib.…”

Primary Renal Epithelioid Hemangioendothelioma

“…This has led to its inclusion under angiosarcomas in the current WHO classification [1]. This tumor has been reported in various sites; however, to the best of our knowledge, there is only one well-documented case of primary epithelioid hemangioendothelioma of kidney in the literature to date [2]. Epithelioid hemangioendothelioma tends to occur over a wide range of age groups excluding childhood and affects both sexes equally [1–3].…”

“…It was initially grouped along with other hemangioendotheliomas—hobnail and kaposiform; however due to the aggressive biological nature, it is now grouped with angiosarcomas in the current WHO classification [1]. Hemangioendotheliomas have been reported in various sites; most commonly in soft tissues followed by liver, lung, bone, peritoneum, stomach, thyroid, and central nervous system [2–6]. Kidney is an extremely rare site, especially for the epithelioid variant.…”

“…Kidney is an extremely rare site, especially for the epithelioid variant. There are very few case reports documenting hemangioendothelioma in the kidney [2, 6–8]. Karasavvidou et al [2] described the first and only case of primary renal epithelioid hemangioendothelioma in a 54-year-old woman.…”