Histiocytic and Dendritic Cell Disorders in the Liver and Spleen

Abstract

Histiocytic and dendritic cell proliferations are rare disorders that make up less than 1% of all neoplasms arising in lymph nodes or soft tissues. Isolated spleen or liver involvement of these disorders without lymph node disease is extremely rare. Only inflammatory pseudotumor-like variant of the follicular dendritic cell sarcoma is almost exclusively arising from the liver and spleen. Their clinical behavior ranges from mild to disseminate and, sometimes, life-threatening forms. The pathology of histiocytic and dendritic cell disorders is heterogeneous and may have overlapping features. The morphologic features in conjunction with immunophenotype and pattern of involvement should always be taken together with the clinical and radiographic findings to make a unifying diagnosis. Recently, strong emphasis on the molecular signature of these disorders gives an opportunity to better describe, subclassify, and ultimately treat these rare disorders/neoplasms.