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Central Nervous System Langerhans Cell Histiocytosis

MedGen UID:
1381057
•Concept ID:
C4329634
•
Neoplastic Process

Definition

Rare involvement of the central nervous system by Langerhans cell histiocytosis. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCentral Nervous System Langerhans Cell Histiocytosis

Professional guidelines

PubMed

Central diabetes insipidus in children: Diagnosis and management.
Patti G, Ibba A, Morana G, Napoli F, Fava D, di Iorgi N, Maghnie M
Best Pract Res Clin Endocrinol Metab 2020 Sep;34(5):101440. Epub 2020 Jun 29 doi: 10.1016/j.beem.2020.101440. PMID: 32646670
MECHANISMS IN ENDOCRINOLOGY: Hypophysitis: diagnosis and treatment.
Joshi MN, Whitelaw BC, Carroll PV
Eur J Endocrinol 2018 Sep;179(3):R151-R163. Epub 2018 Jun 7 doi: 10.1530/EJE-17-0009. PMID: 29880706
Modern management of children with haemophagocytic lymphohistiocytosis.
Janka GE, Schneider EM
Br J Haematol 2004 Jan;124(1):4-14. doi: 10.1046/j.1365-2141.2003.04726.x. PMID: 14675403

Recent clinical studies

Etiology

CNS Langerhans cell histiocytosis: Common hematopoietic origin for LCH-associated neurodegeneration and mass lesions.
McClain KL, Picarsic J, Chakraborty R, Zinn D, Lin H, Abhyankar H, Scull B, Shih A, Lim KPH, Eckstein O, Lubega J, Peters TL, Olea W, Burke T, Ahmed N, Hicks MJ, Tran B, Jones J, Dauser R, Jeng M, Baiocchi R, Schiff D, Goldman S, Heym KM, Wilson H, Carcamo B, Kumar A, Rodriguez-Galindo C, Whipple NS, Campbell P, Murdoch G, Kofler J, Heales S, Malone M, Woltjer R, Quinn JF, Orchard P, Kruer MC, Jaffe R, Manz MG, Lira SA, Parsons DW, Merad M, Man TK, Allen CE
Cancer 2018 Jun 15;124(12):2607-2620. Epub 2018 Apr 6 doi: 10.1002/cncr.31348. PMID: 29624648Free PMC Article
Efficacy of vinblastine in central nervous system Langerhans cell histiocytosis: a nationwide retrospective study.
Ng Wing Tin S, Martin-Duverneuil N, Idbaih A, Garel C, Ribeiro M, Parker JL, Defachelles AS, Lambilliotte A, Barkaoui M, Munzer M, Gardembas M, Sibilia J, Lutz P, Fior R, Polak M, Robert A, Aumaitre O, Plantaz D, Armari-Alla C, Genereau T, Berard PM, Talom GN, Pennaforte JL, Le Pointe HD, Barthez MA, Couillault G, Haroche J, Mokhtari K, Donadieu J, Hoang-Xuan K; French LCH study group
Orphanet J Rare Dis 2011 Dec 12;6:83. doi: 10.1186/1750-1172-6-83. PMID: 22151964Free PMC Article
Fifteen years of treatment with intravenous immunoglobulin in central nervous system Langerhans cell histiocytosis.
Gavhed D, Laurencikas E, Akefeldt SO, Henter JI
Acta Paediatr 2011 Jul;100(7):e36-9. Epub 2011 Jan 11 doi: 10.1111/j.1651-2227.2010.02125.x. PMID: 21166862
Neurodegenerative central nervous system Langerhans cell histiocytosis and coincident hydrocephalus treated with vincristine/cytosine arabinoside.
Allen CE, Flores R, Rauch R, Dauser R, Murray JC, Puccetti D, Hsu DA, Sondel P, Hetherington M, Goldman S, McClain KL
Pediatr Blood Cancer 2010 Mar;54(3):416-23. doi: 10.1002/pbc.22326. PMID: 19908293Free PMC Article
Neurodegenerative central nervous system disease as late sequelae of Langerhans cell histiocytosis. Report from the Japan LCH Study Group.
Imashuku S, Shioda Y, Kobayashi R, Hosoi G, Fujino H, Seto S, Wakita H, Oka A, Okazaki N, Fujita N, Minato T, Koike K, Tsunematsu Y, Morimoto A; Japan LCH Study Group (JLSG)
Haematologica 2008 Apr;93(4):615-8. Epub 2008 Feb 20 doi: 10.3324/haematol.11827. PMID: 18287136

Diagnosis

CNS Langerhans cell histiocytosis: Common hematopoietic origin for LCH-associated neurodegeneration and mass lesions.
McClain KL, Picarsic J, Chakraborty R, Zinn D, Lin H, Abhyankar H, Scull B, Shih A, Lim KPH, Eckstein O, Lubega J, Peters TL, Olea W, Burke T, Ahmed N, Hicks MJ, Tran B, Jones J, Dauser R, Jeng M, Baiocchi R, Schiff D, Goldman S, Heym KM, Wilson H, Carcamo B, Kumar A, Rodriguez-Galindo C, Whipple NS, Campbell P, Murdoch G, Kofler J, Heales S, Malone M, Woltjer R, Quinn JF, Orchard P, Kruer MC, Jaffe R, Manz MG, Lira SA, Parsons DW, Merad M, Man TK, Allen CE
Cancer 2018 Jun 15;124(12):2607-2620. Epub 2018 Apr 6 doi: 10.1002/cncr.31348. PMID: 29624648Free PMC Article
Efficacy of vinblastine in central nervous system Langerhans cell histiocytosis: a nationwide retrospective study.
Ng Wing Tin S, Martin-Duverneuil N, Idbaih A, Garel C, Ribeiro M, Parker JL, Defachelles AS, Lambilliotte A, Barkaoui M, Munzer M, Gardembas M, Sibilia J, Lutz P, Fior R, Polak M, Robert A, Aumaitre O, Plantaz D, Armari-Alla C, Genereau T, Berard PM, Talom GN, Pennaforte JL, Le Pointe HD, Barthez MA, Couillault G, Haroche J, Mokhtari K, Donadieu J, Hoang-Xuan K; French LCH study group
Orphanet J Rare Dis 2011 Dec 12;6:83. doi: 10.1186/1750-1172-6-83. PMID: 22151964Free PMC Article
Incidence and pattern of radiological central nervous system Langerhans cell histiocytosis in children: a population based study.
Laurencikas E, Gavhed D, Stålemark H, van't Hooft I, Prayer D, Grois N, Henter JI
Pediatr Blood Cancer 2011 Feb;56(2):250-7. doi: 10.1002/pbc.22791. PMID: 21157893
Neurodegenerative central nervous system Langerhans cell histiocytosis and coincident hydrocephalus treated with vincristine/cytosine arabinoside.
Allen CE, Flores R, Rauch R, Dauser R, Murray JC, Puccetti D, Hsu DA, Sondel P, Hetherington M, Goldman S, McClain KL
Pediatr Blood Cancer 2010 Mar;54(3):416-23. doi: 10.1002/pbc.22326. PMID: 19908293Free PMC Article
Neurodegenerative central nervous system disease as late sequelae of Langerhans cell histiocytosis. Report from the Japan LCH Study Group.
Imashuku S, Shioda Y, Kobayashi R, Hosoi G, Fujino H, Seto S, Wakita H, Oka A, Okazaki N, Fujita N, Minato T, Koike K, Tsunematsu Y, Morimoto A; Japan LCH Study Group (JLSG)
Haematologica 2008 Apr;93(4):615-8. Epub 2008 Feb 20 doi: 10.3324/haematol.11827. PMID: 18287136

Therapy

Efficacy of vinblastine in central nervous system Langerhans cell histiocytosis: a nationwide retrospective study.
Ng Wing Tin S, Martin-Duverneuil N, Idbaih A, Garel C, Ribeiro M, Parker JL, Defachelles AS, Lambilliotte A, Barkaoui M, Munzer M, Gardembas M, Sibilia J, Lutz P, Fior R, Polak M, Robert A, Aumaitre O, Plantaz D, Armari-Alla C, Genereau T, Berard PM, Talom GN, Pennaforte JL, Le Pointe HD, Barthez MA, Couillault G, Haroche J, Mokhtari K, Donadieu J, Hoang-Xuan K; French LCH study group
Orphanet J Rare Dis 2011 Dec 12;6:83. doi: 10.1186/1750-1172-6-83. PMID: 22151964Free PMC Article
Fifteen years of treatment with intravenous immunoglobulin in central nervous system Langerhans cell histiocytosis.
Gavhed D, Laurencikas E, Akefeldt SO, Henter JI
Acta Paediatr 2011 Jul;100(7):e36-9. Epub 2011 Jan 11 doi: 10.1111/j.1651-2227.2010.02125.x. PMID: 21166862
Neurodegenerative central nervous system Langerhans cell histiocytosis and coincident hydrocephalus treated with vincristine/cytosine arabinoside.
Allen CE, Flores R, Rauch R, Dauser R, Murray JC, Puccetti D, Hsu DA, Sondel P, Hetherington M, Goldman S, McClain KL
Pediatr Blood Cancer 2010 Mar;54(3):416-23. doi: 10.1002/pbc.22326. PMID: 19908293Free PMC Article

Prognosis

Fifteen years of treatment with intravenous immunoglobulin in central nervous system Langerhans cell histiocytosis.
Gavhed D, Laurencikas E, Akefeldt SO, Henter JI
Acta Paediatr 2011 Jul;100(7):e36-9. Epub 2011 Jan 11 doi: 10.1111/j.1651-2227.2010.02125.x. PMID: 21166862
Incidence and pattern of radiological central nervous system Langerhans cell histiocytosis in children: a population based study.
Laurencikas E, Gavhed D, Stålemark H, van't Hooft I, Prayer D, Grois N, Henter JI
Pediatr Blood Cancer 2011 Feb;56(2):250-7. doi: 10.1002/pbc.22791. PMID: 21157893
Neurodegenerative central nervous system disease as late sequelae of Langerhans cell histiocytosis. Report from the Japan LCH Study Group.
Imashuku S, Shioda Y, Kobayashi R, Hosoi G, Fujino H, Seto S, Wakita H, Oka A, Okazaki N, Fujita N, Minato T, Koike K, Tsunematsu Y, Morimoto A; Japan LCH Study Group (JLSG)
Haematologica 2008 Apr;93(4):615-8. Epub 2008 Feb 20 doi: 10.3324/haematol.11827. PMID: 18287136

Clinical prediction guides

Neurodegenerative central nervous system Langerhans cell histiocytosis and coincident hydrocephalus treated with vincristine/cytosine arabinoside.
Allen CE, Flores R, Rauch R, Dauser R, Murray JC, Puccetti D, Hsu DA, Sondel P, Hetherington M, Goldman S, McClain KL
Pediatr Blood Cancer 2010 Mar;54(3):416-23. doi: 10.1002/pbc.22326. PMID: 19908293Free PMC Article
Neurodegenerative central nervous system disease as late sequelae of Langerhans cell histiocytosis. Report from the Japan LCH Study Group.
Imashuku S, Shioda Y, Kobayashi R, Hosoi G, Fujino H, Seto S, Wakita H, Oka A, Okazaki N, Fujita N, Minato T, Koike K, Tsunematsu Y, Morimoto A; Japan LCH Study Group (JLSG)
Haematologica 2008 Apr;93(4):615-8. Epub 2008 Feb 20 doi: 10.3324/haematol.11827. PMID: 18287136

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