Skin nonmelanocytic tumor
Vascular tumors
Hemangioendothelioma-kaposiform
Author: Christopher S. Hale, M.D.
Last author update: 1 August 2012
Last staff update: 17 March 2022
Copyright: 2003-2023, PathologyOutlines.com, Inc.
PubMed search: kaposiform [title] hemangioendothelioma skin
Table of Contents
Definition / general | Case reports | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Differential diagnosisCite this page: Hale CS. Hemangioendothelioma-kaposiform. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocytichemangioendotheliomakaposiform.html. 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
- 24 days old male with ill defined arm mass (J Res Med Sci 2009;14:389)
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’s 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
Differential diagnosis
- Juvenile hemangioma: spontaneously involutes, not associated with Kasabach-Merritt phenomenon; GLUT1+, Lewis Y+
