kaposiform hemangioendothelioma
Definition : 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.
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 are lobulated vascular tumors composed mainly of spindle-shaped endothelial cells, showing some morphological overlap with tufted angioma. These tumours are HHV-8-negative. They may be hard to excise due to their large size.
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-
Kaposi sarcoma
- absence of HHV-8
Associations
Milroy disease (primary hereditary lymphedema)
See also
vascular tumors
vascular dysplasias
References
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#