Langerhans cell histiocytosis: current insights in a molecular age with emphasis on clinical oral and maxillofacial pathology practice

Abstract

Langerhans cell histiocytosis (LCH) commonly involves the oral and maxillofacial region, and comes to the attention of dental practitioners when a patient presents with orofacial pain and a bony or soft tissue lesion. This is a relatively rare entity, which has made it difficult to investigate the clinical, biologic, and molecular aspects of the disease. Treatment protocols are not well defined, particularly in adults. During the past decade, the Histiocyte Society has formulated various LCH categories, based on risk stratification, and treatment protocols for the pediatric population. Adult trials are currently available through the Histiocyte Society. Although there has been considerable controversy, the neoplastic nature of LCH has been established by demonstrating clonality. LCH symptoms and the development and persistence of LCH lesions have been ascribed to a "chemokine/cytokine storm" due to autocrine and paracrine mechanisms. Discovery of biologic, cytogenetic, and molecular abnormalities in LCH have already affected treatment by providing novel therapeutic targets.