Several entities (eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, and "histiocytosis X" now under single designation of LCH
Definitions
LCH = dendritic cell disorder
LCH now best understood as neoplastic disease
Activating somatic BRAF V600Emutations
Associated with more severe disease
Divided into 2 groups (based on number of lesions, systems involved)
Single-system LCH
70-80% of cases (usually skin, bones, or lymph nodes)
Leung AKC et al: Childhood Langerhans cell histiocytosis: a disease with many faces. World J Pediatr. 15(6):536-45, 2019
Pan Z et al: Histiocytic and dendritic cell neoplasms. Surg Pathol Clin. 12(3):805-29, 2019
Papo M et al: Systemic histiocytosis (langerhans cell histiocytosis, Erdheim-Chester disease, Destombes-Rosai-Dorfman disease): from oncogenic mutations to inflammatory disorders. Curr Oncol Rep. 21(7):62, 2019
Park H et al: Imaging of histiocytosis in the era of genomic medicine. Radiographics. 39(1):95-114, 2019
Thacker NH et al: Pediatric Langerhans cell histiocytosis: state of the science and future directions. Clin Adv Hematol Oncol. 17(2):122-31, 2019
Wang Y et al: Neuroimaging features of CNS histiocytosis syndromes. Clin Imaging. 60(1):131-40, 2019
Kim JH et al: Magnetic resonance imaging features in solitary cerebral Langerhans cell histiocytosis: case report and review of literature. World Neurosurg. 116:333-6, 2018
Kobayashi M et al: Langerhans cell histiocytosis in adults: advances in pathophysiology and treatment. Cancer Sci. 109(12):3707-13, 2018
Krooks J et al: Langerhans cell histiocytosis in children: diagnosis, differential diagnosis, treatment, sequelae, and standardized follow-up. J Am Acad Dermatol. 78(6):1047-56, 2018
Tran G et al: Langerhans cell histiocytosis: a neoplastic disorder driven by Ras-ERK pathway mutations. J Am Acad Dermatol. 78(3):579-90.e4, 2018
Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
Badalian-Very G et al: Pathogenesis of Langerhans cell histiocytosis. Annu Rev Pathol. 8:1-20, 2013
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Langerhans cell histiocytosis (LCH)
Several entities (eosinophilic granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, and "histiocytosis X" now under single designation of LCH
Definitions
LCH = dendritic cell disorder
LCH now best understood as neoplastic disease
Activating somatic BRAF V600Emutations
Associated with more severe disease
Divided into 2 groups (based on number of lesions, systems involved)
Single-system LCH
70-80% of cases (usually skin, bones, or lymph nodes)
Leung AKC et al: Childhood Langerhans cell histiocytosis: a disease with many faces. World J Pediatr. 15(6):536-45, 2019
Pan Z et al: Histiocytic and dendritic cell neoplasms. Surg Pathol Clin. 12(3):805-29, 2019
Papo M et al: Systemic histiocytosis (langerhans cell histiocytosis, Erdheim-Chester disease, Destombes-Rosai-Dorfman disease): from oncogenic mutations to inflammatory disorders. Curr Oncol Rep. 21(7):62, 2019
Park H et al: Imaging of histiocytosis in the era of genomic medicine. Radiographics. 39(1):95-114, 2019
Thacker NH et al: Pediatric Langerhans cell histiocytosis: state of the science and future directions. Clin Adv Hematol Oncol. 17(2):122-31, 2019
Wang Y et al: Neuroimaging features of CNS histiocytosis syndromes. Clin Imaging. 60(1):131-40, 2019
Kim JH et al: Magnetic resonance imaging features in solitary cerebral Langerhans cell histiocytosis: case report and review of literature. World Neurosurg. 116:333-6, 2018
Kobayashi M et al: Langerhans cell histiocytosis in adults: advances in pathophysiology and treatment. Cancer Sci. 109(12):3707-13, 2018
Krooks J et al: Langerhans cell histiocytosis in children: diagnosis, differential diagnosis, treatment, sequelae, and standardized follow-up. J Am Acad Dermatol. 78(6):1047-56, 2018
Tran G et al: Langerhans cell histiocytosis: a neoplastic disorder driven by Ras-ERK pathway mutations. J Am Acad Dermatol. 78(3):579-90.e4, 2018
Zaveri J et al: More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. Radiographics. 34(7):2008-24, 2014
Badalian-Very G et al: Pathogenesis of Langerhans cell histiocytosis. Annu Rev Pathol. 8:1-20, 2013
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