Tuesday 27 April 2004
Definition : Kaposiform hemangioendothelioma is a rare tumor of childhood often associated with Kasabach-Merritt phenomenon (50%) and occasionally lymphangiomatosis.
A vascular tumor of kaposiform hemangioendothelioma / tufted angioma spectrum in an infant. Note the characteristic podoplanin / D2-40 stain
Hybrid cystic lymphangioma and kaposiform hemangioendothelioma
KH is a lesion having both a vascular and lymphatic component. Its common association with Kasabach-Merritt phenomenon probably relates in part to unique architectural features that favor turbulent blood flow and platelet activation.
Kaposiform haemangioendothelioma was first recognized in the early 1990s. This is a very rare neoplasm affecting mainly infants and children and which arises mainly in the retroperitoneum or the skin.
These lesions may be large and associated with consumption coagulopathy (Kasabach–Merritt syndrome). They may be hard to excise due to their large size.
They are lobulated vascular tumors composed mainly of spindle-shaped endothelial cells, showing some morphological overlap with tufted angioma . These tumours are HHV-8-negative.
Immunochemistry (Endothelial cells in nodules)
sites of platelet consumption
- scattered "epithelioid" or glomeruloid islands featuring endothelium
- clusters of plump alpha-smooth muscle actin-positive pericytes
- stippled hemosiderin
- CD61+ fibrin thrombi
kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon (15793511)
- KHE presenting in the absence of Kasabach-Merritt phenomenon is rare, although tufted angioma frequently occurs without thrombocytopenia.
alive without residual disease (45%) (15105642)
alive with disease (36%) (15105642)
none distant metastases (O%) (15105642)
regional perinodal soft tissue involvement (10%) (15105642)
death from the disease (14%) (15105642)
- absence of HHV-8
Milroy disease (primary hereditary lymphedema)
Expression of prox1, lymphatic endothelial nuclear transcription factor, in Kaposiform hemangioendothelioma and tufted angioma. Le Huu AR, Jokinen CH, Ruben BP, Mihm MC, Weiss SW, North PE, Dadras SS. Am J Surg Pathol. 2010 Nov;34(11):1563-73. PMID: 20975337
Debelenko LV, Perez-Atayde AR, Mulliken JB, Liang MG, Archibald TH, Kozakewich HP. D2-40 immunohistochemical analysis of pediatric vascular tumors reveals positivity in kaposiform hemangioendothelioma. Mod Pathol. 2005 Nov;18(11):1454-60. PMID: 15920541
Gruman A, Liang MG, Mulliken JB, Fishman SJ, Burrows PE, Kozakewich HP, Blei F, Frieden IJ. Kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon. J Am Acad Dermatol. 2005 Apr;52(4):616-22. PMID: 15793511
Lyons LL, North PE, Mac-Moune Lai F, Stoler MH, Folpe AL, Weiss SW. Kaposiform Hemangioendothelioma: A Study of 33 Cases Emphasizing Its Pathologic, Immunophenotypic, and Biologic Uniqueness From Juvenile Hemangioma. Am J Surg Pathol. 2004 May;28(5):559-568. PMID: 15105642