title: Long-term outcome for kaposiform hemangioendothelioma: A report of two cases.
abstract: Kaposiform hemangioendothelioma (KHE) is a rare aggressive vascular tumor of skin and deep soft tissues that typically presents in infancy and may be associated with a potentially life-threatening coagulopathy known as Kasabach-Merrit phenomenon (KMP). Recent advances in medical therapy have successfully treated many patients. However, our knowledge regarding the natural history of these lesions and optimum surveillance strategies remains rudimentary. We report two young women who had KHE with KMP treated in infancy and presented in adolescence with comorbidities related to their KHE tumor. This presentation supports the need for long-term surveillance in these patients.
authors.affiliation: Cancer and Blood Disease Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Beverly A
authors.initials: BA
authors.lastname: Schaefer
authors.affiliation: Department of Pathology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Dehua
authors.initials: D
authors.lastname: Wang
authors.affiliation: Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Arnold C
authors.initials: AC
authors.lastname: Merrow
authors.affiliation: Department of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Belinda H
authors.initials: BH
authors.lastname: Dickie
authors.affiliation: Department of Pediatric Hematology/Oncology, Boston Children's Hospital, Boston, Massachusetts.
authors.forename: Denise M
authors.initials: DM
authors.lastname: Adams
mesh_terms: D003131:Combined Modality Therapy; D005260:Female; D006390:Hemangioendothelioma / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007223:Infant; D059885:Kasabach-Merritt Syndrome / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D011379:Prognosis; D012514:Sarcoma, Kaposi / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D015996:Survival Rate
keywords: chronic pain; kaposiform hemangioendothelioma; sirolimus