It has been used successfully in the management of several vascular anomalies such as kaposiform hemangioendothelioma
, tufted angioma, and lymphatic malformations .
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.
: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma.