Kaposiform Hemangioendothelioma



Kaposiform Hemangioendothelioma


Steven D. Billings, MD









Clinical photograph shows a large inguinal tumor in an infant. More superficial tumors typically present as an erythematous or violaceous mass.






Hematoxylin & eosin shows proliferation of spindled endothelial cells. The spindled cells are arranged in short fascicles and form slit-like vascular lumens similar to Kaposi sarcoma.


CLINICAL ISSUES


Epidemiology



  • Age



    • Majority present in childhood to teen years


    • Approximately 1/2 of kaposiform hemangioendothelioma cases present in 1st year of life


Presentation



  • Painful or painless mass



    • Presents as superficial or deep mass


    • Cutaneous lesions present as violaceous plaques


    • Deep tumors often multiple nodules


    • Associated with Kasabach-Merritt syndrome (especially retroperitoneal tumors)



      • Consumptive coagulopathy


      • Thrombocytopenia


      • Majority of all cases of Kasabach-Merritt syndrome associated with kaposiform hemangioendothelioma

Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Kaposiform Hemangioendothelioma
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