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+
 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

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

 juvenile hemangioma (#15105642#)

• GLUT-1-
• LeY-

 Kaposi sarcoma

• absence of HHV-8

Associations

 Milroy disease (primary hereditary lymphedema)

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Return to: vascular tumors

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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#

 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#