Case Number : Case 4054 - 04 August 2022 Posted By: Saleem Taibjee

Tufted angioma developing Kasabach-Merrit syndrome (cosumptive coagulopathy). 

Epithelioid hemangioendothelioma

How can I differentiate acquired tufted angioma from kaposiform hemangioendothelioma? This is always difficult for me. In this case favor KHE because of many areas with spindled and quasi-fascicular pattern. 

tufted angioma

Tufted Angioma

Yes, very well done. This is a tufted angioma.

Paediatric vascular lesions are not my forte, but Dr Isabel Colmenero kindly reviewed this case and confirmed my impression. Tufted angioma and Kaposiform haemangioendothelioma are likely to be on a spectrum, hence the difficulty of firmly distinguishing in all cases. However, in my limited experience, the clinical scenario tends to be different, with KHE often presenting within the first few days of life/congenital, often very large and dramatic tumours with a high association with Kasabach-Merritt syndrome, compared to a more insidious clinical story with tufted angioma, and perhaps a low grade coagulopathy (Kasabach-Merritt less common).

Both may express lymphatic markers. The D2-40 in this case is shown below.

There has been some initial literature to suggest WT-1 may be helpful in distinguishing vascular tumours from malformations. However, this was negative in this case, and Isabel informs me that this marker is not very robust. Glut-1 was also negative, excluding infantile haemangioma.

BW Saleem