Pediatric Histiocytic Disorders: Morphology, Immunophenotype and Genetics

Abstract

Histiocytic disorders are rare in childhood and often present with a wide spectrum of histological and clinical symptoms, making their diagnosis challenging. The pathological classification of histiocytic disorders has been evolving during the last few decades, and new diagnostic criteria and classifications have been recently updated. Herein, we review pediatric histiocytic disorders, focusing on the pathological features of morphology, immunophenotype, and newly discovered molecular data. These insights shed light on the pathogenesis of these disorders and may become therapeutic biomarkers.

Helping to Correct the Frequent Invisibility of Pediatric Pathology: A Rare and Much Needed Collection of Papers Highlighting Topics in a Dismissed Specialty

“…Cheng JJ, et al provide a comprehensive analysis of the histological, immunophenotypical and genetic characteristics of the major forms of histiocytic disorders in children, including Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG), Rosai-Dorfman-Destombes disease (RDD), hemophagocytic lymphohistiocytosis (HLH), histiocytic sarcoma (HS) and ALK-histiocytosis (ALK-H). 5 Histiocytic disorders are relatively common in children, and this review provides updates on the appropriate and current classification systems, immunohistochemical approach and genetic analysis. The paper covers hematopathology, dermatopathology and genetic/molecular pathology issues, outlining the most effective diagnostic approaches.…”