Nested Stromal-Epithelial Tumor of the Liver: A Review

Abstract

Background: Nested stromal-epithelial tumor (NSET) is a rare liver tumor, which is most commonly seen in the pediatric age group. To the best of our knowledge, there has been no published review on this rare tumor in the English literature so far. Summary: In this review, we will discuss all the reported details of the published cases, including demography, clinical presentation, molecular histogenesis, imaging, gross pathology and histopathology, immunohistochemical findings, treatment modalities, and outcome of NSET of the liver. Key Message: Thirty-eight cases of NSET have been reported in the last 20 years in the English literature. This tumor produces a very large and calcified mass in the liver and characteristically can present as Cushing syndrome. NSET is a nonbiliary and nonhepatocytic tumor with biphasic differentiation into 2 components of epithelial and stromal cells. The epithelial cells are arranged as nests of mildly atypical epithelial cells with a few mitotic figures. The stromal component is composed of myofibroblasts and desmoplastic stroma which often shows ossification and calcification. Immunohistochemically, this tumor is positive for both epithelial and mesenchymal markers. The majority of the reported cases in the literature had benign behavior with an indolent course.