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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Diagnostic Checklist
TERMINOLOGY
Synonyms
- Langerhans cell histiocytosis (LCH)
- Several entities (eosinophilic granuloma, Hand-Schüller-Christian disease, Letterer-Siwe disease, and histiocytosis X now under single designation of LCH
- Langerhans cell histiocytosis (LCH)
Definitions
- LCH now best understood as neoplastic disease
- Activating somatic BRAF mutations, LCH cell clonality
- Inflammatory myeloid neoplasia
- Divided into 3 groups (based on number of lesions, systems involved)
- Unifocal localized form (a.k.a. eosinophilic granuloma)
- 70% of cases
- Limited to single or a few bones, may involve lung
- Multifocal unisystem
- 20% of cases
- Chronic, recurring
- Multiple bones, reticuloendothelial system, pituitary/hypothalamus
- Multifocal, multisystem
- 10% of cases
- Fulminant (often fatal)
- Unifocal localized form (a.k.a. eosinophilic granuloma)
- LCH now best understood as neoplastic disease
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- 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
- Zheng H et al: Pediatric Langerhans cell histiocytosis of the temporal bone: clinical and imaging studies of 27 cases. World J Surg Oncol. 16(1):72, 2018
- Chevallier KM et al: Differentiating pediatric rhabdomyosarcoma and Langerhans cell histiocytosis of the temporal bone by imaging appearance. AJNR Am J Neuroradiol. 37(6):1185-9, 2016
- Berres ML et al: Progress in understanding the pathogenesis of Langerhans cell histiocytosis: back to Histiocytosis X? Br J Haematol. 169(1):3-13, 2015
- De La Hoz Polo M et al: Neuroimaging of Langerhans cell histiocytosis in the central nervous system of children. Radiologia. 57(2):123-130, 2015
- Demellawy DE et al: Langerhans cell histiocytosis: a comprehensive review. Pathology. 47(4):294-301, 2015
- Esmaili N et al: Langerhans cll histiocytosis of the orbit: spectrum of disease and risk of central nervous system sequelae in unifocal cases. Ophthal Plast Reconstr Surg. ePub, 2015
- Modest MC et al: Langerhans cell histiocytosis of the temporal bone: A review of 29 cases at a single center. Laryngoscope. ePub, 2015
- Porto L et al: Central nervous system imaging in childhood Langerhans cell histiocytosis - a reference center analysis. Radiol Oncol. 49(3):242-9, 2015
- Nabavizadeh SA et al: CT and MRI of Pediatric Skull Lesions with Fluid-Fluid Levels. AJNR Am J Neuroradiol. 35(3):604-8, 2014
- 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
- Laurencikas E et al: Incidence and pattern of radiological central nervous system Langerhans cell histiocytosis in children: a population based study. Pediatr Blood Cancer. 56(2):250-7, 2011
- Grois N et al: Central nervous system disease in Langerhans cell histiocytosis. J Pediatr. 156(6):873-81, 881, 2010
- D'Ambrosio N et al: Craniofacial and intracranial manifestations of langerhans cell histiocytosis: report of findings in 100 patients. AJR Am J Roentgenol. 191(2):589-97, 2008
- Demaerel P et al: Paediatric neuroradiological aspects of Langerhans cell histiocytosis. Neuroradiology. 50(1):85-92, 2008
- Saliba I et al: Langerhans' cell histiocytosis of the temporal bone in children. Int J Pediatr Otorhinolaryngol. 72(6):775-86, 2008
- van der Knaap MS et al: Cerebellar leukoencephalopathy: most likely histiocytosis-related. Neurology. 71(17):1361-7, 2008
- Varan A et al: Radiological evaluation of patients with pituitary langerhans cell histiocytosis at diagnosis and at follow-up. Pediatr Hematol Oncol. 25(6):567-74, 2008
- Kaste SC et al: PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Radiol. 37(7):615-22, 2007
- Prosch H et al: Long-term MR imaging course of neurodegenerative Langerhans cell histiocytosis. AJNR Am J Neuroradiol. 28(6):1022-8, 2007
- Horn EM et al: Isolated Langerhans cell histiocytosis of the infundibulum presenting with fulminant diabetes insipidus. Childs Nerv Syst. 22(5):542-4, 2006
- Martin-Duverneuil N et al: MRI features of neurodegenerative Langerhans cell histiocytosis. Eur Radiol. 16(9):2074-82, 2006
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Diagnostic Checklist
TERMINOLOGY
Synonyms
- Langerhans cell histiocytosis (LCH)
- Several entities (eosinophilic granuloma, Hand-Schüller-Christian disease, Letterer-Siwe disease, and histiocytosis X now under single designation of LCH
- Langerhans cell histiocytosis (LCH)
Definitions
- LCH now best understood as neoplastic disease
- Activating somatic BRAF mutations, LCH cell clonality
- Inflammatory myeloid neoplasia
- Divided into 3 groups (based on number of lesions, systems involved)
- Unifocal localized form (a.k.a. eosinophilic granuloma)
- 70% of cases
- Limited to single or a few bones, may involve lung
- Multifocal unisystem
- 20% of cases
- Chronic, recurring
- Multiple bones, reticuloendothelial system, pituitary/hypothalamus
- Multifocal, multisystem
- 10% of cases
- Fulminant (often fatal)
- Unifocal localized form (a.k.a. eosinophilic granuloma)
- LCH now best understood as neoplastic disease
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- 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
- Zheng H et al: Pediatric Langerhans cell histiocytosis of the temporal bone: clinical and imaging studies of 27 cases. World J Surg Oncol. 16(1):72, 2018
- Chevallier KM et al: Differentiating pediatric rhabdomyosarcoma and Langerhans cell histiocytosis of the temporal bone by imaging appearance. AJNR Am J Neuroradiol. 37(6):1185-9, 2016
- Berres ML et al: Progress in understanding the pathogenesis of Langerhans cell histiocytosis: back to Histiocytosis X? Br J Haematol. 169(1):3-13, 2015
- De La Hoz Polo M et al: Neuroimaging of Langerhans cell histiocytosis in the central nervous system of children. Radiologia. 57(2):123-130, 2015
- Demellawy DE et al: Langerhans cell histiocytosis: a comprehensive review. Pathology. 47(4):294-301, 2015
- Esmaili N et al: Langerhans cll histiocytosis of the orbit: spectrum of disease and risk of central nervous system sequelae in unifocal cases. Ophthal Plast Reconstr Surg. ePub, 2015
- Modest MC et al: Langerhans cell histiocytosis of the temporal bone: A review of 29 cases at a single center. Laryngoscope. ePub, 2015
- Porto L et al: Central nervous system imaging in childhood Langerhans cell histiocytosis - a reference center analysis. Radiol Oncol. 49(3):242-9, 2015
- Nabavizadeh SA et al: CT and MRI of Pediatric Skull Lesions with Fluid-Fluid Levels. AJNR Am J Neuroradiol. 35(3):604-8, 2014
- 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
- Laurencikas E et al: Incidence and pattern of radiological central nervous system Langerhans cell histiocytosis in children: a population based study. Pediatr Blood Cancer. 56(2):250-7, 2011
- Grois N et al: Central nervous system disease in Langerhans cell histiocytosis. J Pediatr. 156(6):873-81, 881, 2010
- D'Ambrosio N et al: Craniofacial and intracranial manifestations of langerhans cell histiocytosis: report of findings in 100 patients. AJR Am J Roentgenol. 191(2):589-97, 2008
- Demaerel P et al: Paediatric neuroradiological aspects of Langerhans cell histiocytosis. Neuroradiology. 50(1):85-92, 2008
- Saliba I et al: Langerhans' cell histiocytosis of the temporal bone in children. Int J Pediatr Otorhinolaryngol. 72(6):775-86, 2008
- van der Knaap MS et al: Cerebellar leukoencephalopathy: most likely histiocytosis-related. Neurology. 71(17):1361-7, 2008
- Varan A et al: Radiological evaluation of patients with pituitary langerhans cell histiocytosis at diagnosis and at follow-up. Pediatr Hematol Oncol. 25(6):567-74, 2008
- Kaste SC et al: PET-CT in pediatric Langerhans cell histiocytosis. Pediatr Radiol. 37(7):615-22, 2007
- Prosch H et al: Long-term MR imaging course of neurodegenerative Langerhans cell histiocytosis. AJNR Am J Neuroradiol. 28(6):1022-8, 2007
- Horn EM et al: Isolated Langerhans cell histiocytosis of the infundibulum presenting with fulminant diabetes insipidus. Childs Nerv Syst. 22(5):542-4, 2006
- Martin-Duverneuil N et al: MRI features of neurodegenerative Langerhans cell histiocytosis. Eur Radiol. 16(9):2074-82, 2006
