Skin nonmelanocytic tumor

Vascular tumors


Last author update: 1 August 2012
Last staff update: 17 March 2022

Copyright: 2003-2023,, Inc.

PubMed search: kaposiform [title] hemangioendothelioma skin

Christopher S. Hale, M.D.
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Cite this page: Hale CS. Hemangioendothelioma-kaposiform. website. Accessed March 22nd, 2023.
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

Positive stains
Negative stains
  • GLUT1 (glucose transporter protein isoform 1), Lewis Y antigen, HHV8
Differential diagnosis
  • Juvenile hemangioma: spontaneously involutes, not associated with Kasabach-Merritt phenomenon; GLUT1+, Lewis Y+
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