title: Kaposiform haemangioendothelioma of the head and neck.
abstract: BACKGROUND
  Kaposiform haemangioendothelioma (KHE) is a tumor of intermediate malignant potential derived from vascular endothelial cells. Due to rarity of head neck KHE (HN-KHE) this comprehensive review aims to compile, analyze and present details to develop a consensus and augment available literature on HN-KHE.
  
  
  METHODS
  A comprehensive literature search was performed on PUBMED/MEDLINE, EMBASE, CINAHL and Science Citation Index for HN-KHE using MeSH words. Statistical analysis was performed using a variety of tests.
  
  
  RESULTS
  Common sites of involvement were neck 41.5%, face and scalp 32.0% and tympanomastoid region in 13.2% patients. Kasabach-Merritt phenomenon was seen in 58.5% patients. Surgical excision was performed in 37.7% patients while 39.6% patients underwent medical management/chemotherapy (CT). Significantly better disease free survival (DFS) was seen in patients undergoing surgical excision vs. CT (p=0.001), without recurrence vs. with recurrence (p=0.001) and those presenting within 0-1year of life vs. 1-5 years (p=0.021).
  
  
  CONCLUSIONS
  Recurrence and metastasis were seen in 35.8% and 20.0% patients respectively. Complete surgical excision with clear margins remains the treatment of choice.
authors.affiliation: Department of Otolaryngology, Head and Neck Surgery, Addenbrookes Hospital, Cambridge University Hospitals NHS Trust, Cambridge CB2 0QQ, UK. Electronic address: bwonglk@gmail.com.
authors.forename: Billy L K
authors.initials: BL
authors.lastname: Wong
authors.affiliation: School of Medicine, Kings College, London WC2R 2LS, UK.
authors.forename: Vivian N Y
authors.initials: VN
authors.lastname: Lee
authors.affiliation: Department of Otolaryngology, Head and Neck Surgery, New Cross Hospital, Royal Wolverhampton Hospitals NHS Trust, Wolverhampton WV10 0QP, UK.
authors.forename: Theofano
authors.initials: T
authors.lastname: Tikka
authors.affiliation: Department of Otolaryngology, Head and Neck Surgery, St. Georges University Hospital, London SW17 0QT, UK.
authors.forename: Dae
authors.initials: D
authors.lastname: Kim
authors.affiliation: Department of Otolaryngology, Head and Neck Surgery, Addenbrookes Hospital, Cambridge University Hospitals NHS Trust, Cambridge CB2 0QQ, UK.
authors.forename: Raghav C
authors.initials: RC
authors.lastname: Dwivedi
mesh_terms: D016208:Databases, Factual; D006258:Head and Neck Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D006390:Hemangioendothelioma / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D006801:Humans; D059885:Kasabach-Merritt Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D012514:Sarcoma, Kaposi / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy
keywords: Head; Kaposiform haemangioendothelioma; Neck