Kaposiform haemangioendothelioma of the head and neck

Crit Rev Oncol Hematol. 2016 Aug:104:156-68. doi: 10.1016/j.critrevonc.2016.06.005. Epub 2016 Jun 16.


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.

Materials and 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).

Conclusion: Recurrence and metastasis were seen in 35.8% and 20.0% patients respectively. Complete surgical excision with clear margins remains the treatment of choice.

Keywords: Head; Kaposiform haemangioendothelioma; Neck.

Publication types

  • Review

MeSH terms

  • Databases, Factual
  • Head and Neck Neoplasms* / diagnosis
  • Head and Neck Neoplasms* / epidemiology
  • Head and Neck Neoplasms* / pathology
  • Head and Neck Neoplasms* / therapy
  • Hemangioendothelioma* / diagnosis
  • Hemangioendothelioma* / epidemiology
  • Hemangioendothelioma* / pathology
  • Hemangioendothelioma* / therapy
  • Humans
  • Kasabach-Merritt Syndrome* / diagnosis
  • Kasabach-Merritt Syndrome* / epidemiology
  • Kasabach-Merritt Syndrome* / pathology
  • Kasabach-Merritt Syndrome* / therapy
  • Prognosis
  • Sarcoma, Kaposi* / diagnosis
  • Sarcoma, Kaposi* / epidemiology
  • Sarcoma, Kaposi* / pathology
  • Sarcoma, Kaposi* / therapy

Supplementary concepts

  • Kaposiform Hemangioendothelioma