kaposiform hemangioendothelioma
Kaposiform hemangioendothelioma is a rare tumor of childhood often associated with Kasabach-Merritt phenomenon (50%) and occasionally lymphangiomatosis.
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.
Immunochemistry (Endothelial cells in nodules)
CD31+
CD34+
D2-40 (15920541)
FLI1+
GLUT1 -
LeY-
Synopsis
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
Variants
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.
Prognosis
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)
Differential diagnosis
infantile hemangioma (15105642)
- GLUT1+
- LeY-
- absence of HHV-8
Associations
Milroy disease (primary hereditary lymphedema)
Return to: vascular tumors and vascular dysplasias
References
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