Kaposiform hemangioendothelioma arising in the deltoid muscle without the Kasabach–Merritt phenomenon

Abstract

Kaposiform hemangioendothelioma (KHE) is a rare tumor that occurs nearly exclusively during infancy and childhood. It has features common to both capillary hemangioma and Kaposi sarcoma and for that reason many terms have been used for these tumors including "Kaposi-like infantile hemangioendothelioma" and "hemangioma with Kaposi sarcoma-like features." KHE typically presents as an ill-defined, red to purple, indurated plaque and is often complicated by the Kasabach-Merritt phenomenon (KMP), a condition of severe thrombocytopenia and consumptive coagulopathy. Knowledge of the radiological findings of this uncommon tumor might be helpful for diagnosis. We present the MRI features of a case of KHE with neither typical skin lesions nor the Kasabach-Merritt phenomenon.

Testicular Kaposiform Hemangioendothelioma Of An 80-Year-Old: A Case Report And Literature Review.

“…In order to properly identify KHE lesions and differentiate them from other similar vascular lesions, a combination of pathologic findings and biochemical markers can be used. A variety of endothelial and lymphatic markers have been found to be expressed on KHE cells including CD31, CD34, LYVE-1, podoplanin, Prox1 9 , VEGFR-2, VEGFR-3 10 , D2-40 11 , and vimentin 12 . Importantly, KHE is seen to lack HHV-8 DNA and PAS-positive globules (seen in Kaposi sarcoma) and GLUT-1 (seen in infantile hemangiomas).…”

A Review of the Current State and Future Directions for Management of Scalp and Facial Vascular Malformations

“…Imaging modalities such as MRI can be helpful in diagnosing associated syndromes such as Sturge-Weber [ 35 ]. A key distinction from a malignant vascular lesion, kaposiform hemangioendothelioma may be subcutaneous fat stranding on MRI, which is sometimes described as a sunburst pattern” [ 43 ]. The majority of facial capillary malformations (approximately 90%) are unilateral in a trigeminal dermatomal distribution [ 35 ].…”