Kaposiform hemangioendothelioma with associated Kasabach-Merritt phenomenon

Benign Cutaneous Vascular Tumors of Infancy

“…53 Systemic corticosteroids alone have been beneficial in some patients, though most reports cite steroid use in combination with other therapeutic modalities. 94,95 Interferon alfa is useful for treating both the tumor and associated KMS, and is effective in about 50% of children. [96][97][98] Multimodal intervention has also been successful using embolization, interferon, cyclophosphamide, ⑀-aminocaproic acid, and compression therapy.…”

Successful Surgical Excision of a Complex Vascular Lesion in an Infant with Kasabach–Merritt Syndrome

“…A recent study revealed that the KM syndrome is a distinctive disease of infancy and the underlying vascular lesion is not a classic involuting hemangioma of infancy, but rather a kaposiform hemangioendothelioma (3). Other studies have also shown an association of the KM syndrome with tufted angioma (4)(5)(6). Clinical features distinguish infantile vascular tumors associated with the KM syndrome from involuting hemangiomas of infancy.…”

Refractory Kaposiform Hemangioendothelioma That Expressed Vascular Endothelial Growth Factor Receptor (VEGFR)-2 and VEGFR-3

“…Nineteen children with coagulopathy owing to KHE of deep soft tissue have been reported. [1][2][3][4][5][6][7][8][9][10] These reports indicate that the most favorable response is expected with surgical procedures, if the KHE is localized. However, most patients are not eligible for surgical therapy owing to extended diffuse lesions and the risk of massive bleeding, as observed in the present case.…”

“…A surgical resection, steroid, interferon (IFN)-a, chemotherapy, and radiation therapy have been reported to be effective for the treatment of KHE. [1][2][3][4][5][6][7][8][9][10] However, KHE is often associated with lifethreatening coagulopathy, designated as Kasabach-Merritt syndrome (KMS), which has a mortality rate as high as 24%. 11 This report describes the case of a KHE patient who only responded transiently to the standard therapy and died from an intracranial hemorrhage and an opportunistic infection.…”