title: Mediastinal and neck kaposiform hemangioendothelioma: report of three cases.
abstract: Kaposiform hemangioendothelioma is an aggressive vascular tumor, named for its striking histologic resemblance to Kaposi sarcoma and locally invasive growth. Mortality is high, and ranges from 10% to 24% for all kaposiform hemangioendothelioma lesions, with a significantly higher mortality for deep soft-tissue or visceral lesions occurring in infants less than 6 months. Mediastinal and neck kaposiform hemangioendothelioma in particular merit special discussion, as involvement of these critical anatomic locations results in significant site-specific therapeutic challenges due to invasion of vital structures, inherent delays in establishing histopathologic confirmation, and difficulties in monitoring disease status. We report our experience with three cases of mediastinal and neck kaposiform hemangioendothelioma, emphasizing the unique diagnostic and management challenges, variable response to treatment and outcome of this anatomic variant of kaposiform hemangioendothelioma.
authors.affiliation: Department of Paediatric Dermatology, National Maternity Hospital, Dublin, Ireland.
authors.forename: Grainne M
authors.initials: GM
authors.lastname: O'Regan
authors.forename: Alan D
authors.initials: AD
authors.lastname: Irvine
authors.forename: Nana
authors.initials: N
authors.lastname: Yao
authors.forename: Aengus
authors.initials: A
authors.lastname: O'Marcaigh
authors.forename: Margaret
authors.initials: M
authors.lastname: Sheridan-Pereira
authors.forename: Eithne
authors.initials: E
authors.lastname: Phelan
authors.forename: Michael B
authors.initials: MB
authors.lastname: McDermott
authors.forename: Anne
authors.initials: A
authors.lastname: Twomey
authors.forename: John
authors.initials: J
authors.lastname: Russell
authors.forename: Rosemarie
authors.initials: R
authors.lastname: Watson
mesh_terms: D005260:Female; D006258:Head and Neck Neoplasms / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D006390:Hemangioendothelioma / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008479:Mediastinal Neoplasms / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D012514:Sarcoma, Kaposi / Q000473:pathology