It has been used successfully in the management of several vascular anomalies such as
kaposiform hemangioendothelioma, tufted angioma, and lymphatic malformations [1].
Variable response to propranolol treatment of
kaposiform hemangioendothelioma, tufted angioma and Kasabach-Merritt syndrome.
Tumours with features of tufted angioblastoma and
kaposiform hemangioendothelioma have been described.
Vincristine and corticosteroids as first-line treatment of Kasabach-Merritt syndrome in
kaposiform hemangioendothelioma. J Cutan Med Surg.
Kasabach-Merritt phenomenon is a rare and life-threatening condition in which specific types of vascular tumors (either tufted angioma or
Kaposiform hemangioendothelioma) trap and destroy platelets.
Vascular anomalies are classified as follows: * Tumors Juvenile hemangioma Rapidly involuting congenital hemangioma Noninvoluting congenital hemangioma
Kaposiform hemangioendothelioma Tufted angioma * Vascular malformations High-flow Arteriovenous malformation Low-flow Venous malformation Lymphatic malformation Lymphatic-venous malformation Capillary (or venular) malformation (portwine stain)
Unexpectedly, the histologic result was a
kaposiform hemangioendothelioma, a proliferation of endothelial fusiform cells, considered to be nearly exclusive of children and teenagers.
Kozakewich et al., "
Kaposiform hemangioendothelioma: atypical features and risks of kasabach-merritt phenomenon in 107 referrals," Journal of Pediatrics, vol.
Other entities in the differential diagnosis include Wilm's tumor, hepatoblastoma, solid and cystic papillary tumor of the pancreas, non-Hodgkin lymphoma, and a
Kaposiform hemangioendothelioma.12 The age and gender of the child should help to narrow down the differential diagnosis.
Immunohistochemistry shows strong positivity for Ubx European I lectin and EN4 and unlike infantile haemangioma, negative staining for GLUT-1.7 TA and
Kaposiform hemangioendothelioma (KH) sometimes show overlapping histopathological features.
The histologic differential diagnosis of KS includes other vascular tumors such as benign lymphangioendothelioma, hobnail hemangioma, spindle cell hemangioma,
kaposiform hemangioendothelioma, cutaneous angiosarcoma, and acroangiodermatitis.
Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma.