Clinical outcomes for systemic corticosteroids versus vincristine in treating kaposiform hemangioendothelioma
and tufted angioma.
In the borderline category, kaposiform hemangioendothelioma
is a childhood tumor that may be associated with thrombocytopenia and consumptive coagulopathy, whereas Kaposi sarcoma is most commonly encountered in the immunocompromised adult.
The clinical differential diagnosis of subcutaneous LCH includes a wide variety of subcutaneous pathologies, including but not limited to vascular malformation, Kaposiform hemangioendothelioma
, and infantile hemangioma.
Vascular malformations include slow-flow malformations (with venous, capillary and/or lymphatic components) and fast-flow malformations (with arterial components); while vascular neoplasms undergo mitosis and include such lesions as infantile hemangioma, congenital hemangioma, kaposiform hemangioendothelioma
, tufted angioma, hemangiopericytoma, and angiosarcoma .
The diagnosis of littoral cell angioma (LCA) was finally made, which is a different vascular tumor with a resemblance pathologically to either tufted angioma (TA) or kaposiform hemangioendothelioma
(KHE) in association with lymphatic-like vessels .
of the spleen in an adult: an initial case report.
Prox1, a transcription factor important in the regulation and maintenance of the lymphatic endothelial phenotype, is expressed in lymphangiomas, Kaposi sarcoma, tufted angiomas, and Kaposiform hemangioendothelioma
. (262) The monoclonal antibody D2-40 binds to podoplanin, a glycoprotein highly expressed in lymphatic endothelium.
Hannah, now 19 months, suffers from Kaposiform hemangioendothelioma
with Kasabach-Merritt syndrome - which means tumours make platelet level drop dangerously low.
In the case of KMP, it is speculated that the unique vessel architecture of Kaposiform hemangioendothelioma
(KHE) and tufted angiomas (TA) promotes platelet trapping and the resultant consumptive coagulopathy; in contrast to the ordered tree-like vasculature of infantile hemangioma, convoluted capillaries arise directly off large vessels in KHE and TA resulting in turbulent flow-promoting platelet activation and aggregation .
or tufted angiomas are also characterized by rapid growth but can be associated with a low platelet count in the case of the Kasabach-Merritt phenomenon.
The differential diagnosis of angioblastoma are pyogenic granuloma, kaposiform hemangioendothelioma
(KHE), hemangiopericytoma, strawberry angioma etc.
Successful management of Kaposiform hemangioendothelioma
with Kasabach-Merritt phenomenon using vincristine and ticlopidine.