Kaposiform hemangioendothelioma (with Kasabach Merritt syndrome) of the head and neck: case report and review of the literature

Int J Pediatr Otorhinolaryngol. 2009 Oct;73(10):1474-6. doi: 10.1016/j.ijporl.2009.06.019. Epub 2009 Jul 29.

Abstract

An infant initially diagnosed with a parotid hemangioma presented with stridor and thrombocytopenia. Diagnosis of Kaposiform hemangioendothelioma was confirmed with biopsy. The child succumbed to multi-system organ failure related to consumptive coagulopathy despite aggressive medical management. Kaposiform hemangioendothelioma is a rare head and neck tumor that may be mistaken for a hemangioma on preliminary diagnosis, which may lead to increased morbidity and mortality especially in the setting of Kasabach-Merritt phenomenon. A platelet count may provide an early and important clue to the possibility of coagulopathy; prompting physicians to look for a diagnosis other than a simple hemangioma.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Disease Progression
  • Disseminated Intravascular Coagulation / diagnosis
  • Disseminated Intravascular Coagulation / therapy
  • Fatal Outcome
  • Female
  • Hemangioendothelioma / diagnosis
  • Hemangioendothelioma / therapy
  • Hemangioma, Capillary / diagnosis
  • Hemangioma, Capillary / therapy
  • Hemangioma, Cavernous / diagnosis
  • Hemangioma, Cavernous / therapy
  • Humans
  • Infant, Newborn
  • Kasabach-Merritt Syndrome
  • Laryngeal Neoplasms / diagnosis*
  • Laryngeal Neoplasms / therapy
  • Laryngoscopy / methods
  • Magnetic Resonance Imaging / methods
  • Multiple Organ Failure / diagnosis*
  • Multiple Organ Failure / etiology
  • Risk Assessment
  • Sarcoma, Kaposi / diagnosis
  • Sarcoma, Kaposi / therapy
  • Severity of Illness Index
  • Skin Neoplasms / diagnosis
  • Skin Neoplasms / therapy
  • Thrombocytopenia / diagnosis
  • Thrombocytopenia / therapy
  • Tracheostomy / methods
  • Vascular Neoplasms / diagnosis
  • Vascular Neoplasms / therapy

Supplementary concepts

  • Kaposiform Hemangioendothelioma