Kaposiform hemangioendothelioma. An aggressive, locally invasive vascular tumor that can mimic hemangioma of infancy

D2-40 immunohistochemical analysis of pediatric vascular tumors reveals positivity in kaposiform hemangioendothelioma

“…The term was proposed in 1993 to delineate the entity from the infantile hemangioma. 1 The lesion was originally described in deep soft tissues; however, it frequently affects dermis and subcutis, [2][3][4] where it forms characteristic violaceous plaques. It is associated with profound thrombocytopenia (KasabachMerritt phenomenon) in more than a half of cases.…”

Overexpression of VEGF 121 in Immortalized Endothelial Cells Causes Conversion to Slowly Growing Angiosarcoma and High Level Expression of the VEGF Receptors VEGFR-1 and VEGFR-2 in Vivo

“…1 Hemangiomas constitute the most common tumor of childhood and, though benign, may require systemic therapy to alleviate complications, such as compression of vital structures, coagulopathy (Kasabach-Merritt syndrome), and high output heart failure. [2][3][4] Endothelial malignancies are difficult to treat surgically, because of multifocal growth and potential for distant spread, the latter often arising after successful resection of a primary tumor. 5,6 Thus, increased understanding of the pathogenesis of angiosarcoma is required and may lead to improved therapy.…”

Massive Congenital Vascular Tumor of Lower Limb: Kaposiform Hemangioendothelioma

“…[4] Pre-operative diagnostic biopsy is advised in newly-onset proliferative vascular mass in children older than 3 months of age. [5] Investigative modalities include doppler ultrasonography and MRI. On doppler study, KHE shows an ill-defined heterogeneous lesion with high internal vascularity and high systolic flow.…”

“…[1] Other useful microscopy findings to differentiate KHE from Kaposi sarcoma are a lobular pattern, a greater tendency to form complete vascular spaces, and the presence of scattered fibrin thrombi. [5] Treatment of KHE is decided by its size, location and expected side effects of various available therapies. Sirolimus is now been used as first line therapy or as part of multidisciplinary approach for treatment of KHE.…”