Inflammatory pseudotumor of the liver in Kostmann's disease.

A case in which inflammatory pseudotumor of the liver (IPTL) seemed to complicate severe congenital neutropenia (Kostmann's disease) is reported. IPTL is a rare entity, especially in childhood. The exact etiology of this lesion is unknown, but it is generally regarded as a benign, reactive inflammatory condition. Based on 15 reported pediatric cases in the literature, the causes, diagnosis, and treatment of IPTL are discussed.