Functional hyposplenism due to a primary epithelioid hemangioendothelioma of the spleen.

We present a unique case of functional hyposplenism due to massive involvement of the spleen by a rare tumor, an epithelioid hemangioendothelioma, in a 9-year-old girl. To our knowledge, three previous cases of this disorder involving the spleen have been reported, but this is the first associated with functional hyposplenism.