Skin - Nonmelanocytic tumors
Lymphoma and related disorders
Kaposiform hemangioendothelioma

Author: Christopher Hale, M.D. (see Authors page)

Revised: 12 October 2016, last major update August 2012

Copyright: (c) 2003-2016,, Inc.

PubMed search: kaposiform [title] hemangioendothelioma skin

Cite this page: Kaposiform hemangioendothelioma. website. Accessed October 20th, 2018.
Definition / general
  • Rare tumor of childhood (mean age 4 years, range 2 weeks to 20 years)
  • Some cases may previously have been called acquired tufted angioma
  • Usually on extremities or head and neck; may affect skin or deep soft tissue
  • Intermediate malignancy: does not regress; metastases limited to regional perinodal soft tissue
  • 50% are associated with Kasabach-Merritt phenomenon (profound thrombocytopenia and life threatening hemorrhage); occasionally associated with lymphangiomatosis; deaths are due to phenomenon, not tumor (Am J Surg Pathol 2004;28:559, Mod Pathol 2001;14;1087)
Case reports
Clinical images
Images hosted on other servers:

Bulky red mass on arm of neonate

Gross description
  • Skin lesions are slightly raised, blue red
Microscopic (histologic) description
  • Biphasic with vascular and lymphatic component
  • Irregular, infiltrating nodules of compressed vessels, evoking a dense hyaline stromal response
  • Vessels are tightly coiled and highly convoluted, and budded off larger vessels, resembling either capillary hemangioma or Kaposi‚Äôs sarcoma
  • Scattered epithelioid or glomeruloid islands are associated with pericytes, hemosiderin and fibrin thrombi
  • Also has lymphatic component with thin walled vessels
Microscopic (histologic) images
Images hosted on other servers:

Various images

Various images

Positive stains
Negative stains
Differential diagnosis
  • Juvenile hemangioma: spontaneously involutes, not associated with Kasabach-Merritt phenomenon; GLUT1+, Lewis Y+