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Juvenile xanthogranuloma

MedGen UID:
12179
Concept ID:
C0043324
Disease or Syndrome
Synonyms: Juvenile Xanthogranuloma; Juvenile Xanthogranulomas; Juvenile Xanthoma; Juvenile Xanthomas; Nevoxanthoendothelioma; Nevoxanthoendotheliomas; Xanthogranuloma, Juvenile; Xanthogranulomas, Juvenile; Xanthoma, Juvenile; Xanthomas, Juvenile
SNOMED CT: Juvenile xanthogranuloma (400031009); Multiple eruptive juvenile xanthogranuloma (400204000); Nevoxanthoendothelioma (400031009); Xanthoma neviforme (400031009); Xanthoma multiplex (400031009); Juvenile xanthoma (400031009); Congenital xanthoma tuberosum (400031009); Juvenile giant cell granuloma (400031009); Multiple eruptive xanthoma in infancy (400204000); JXG - Juvenile xanthogranuloma (400031009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0015534
Orphanet: ORPHA158000

Definition

Juvenile xanthogranuloma is the most common type of non-Langerhans cell histiocytosis (see this term) characterized by the occurrence of one or more reddish or yellowish self-limiting and benign papules or nodules of several millimeters in diameter, usually appearing on the head and neck (but sometimes on the extremities and trunk) during the first year of life (or rarely in adulthood) and usually regressing spontaneously. Extracutaneous involvement has also been reported, involving most commonly the eye (uveal tract) but with other locations including the central nervous system, lung, liver, bones and endocrine glands, and may be associated with considerable morbidity. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVJuvenile xanthogranuloma
Follow this link to review classifications for Juvenile xanthogranuloma in Orphanet.

Professional guidelines

PubMed

Yamada R, Komohara Y
J Clin Exp Hematop 2024;64(3):156-165. doi: 10.3960/jslrt.24031. PMID: 39343608Free PMC Article
Geerlinks AV, Abla O
Paediatr Drugs 2023 Jul;25(4):399-409. Epub 2023 May 19 doi: 10.1007/s40272-023-00569-8. PMID: 37204611
Dalia S, Shao H, Sagatys E, Cualing H, Sokol L
Cancer Control 2014 Oct;21(4):290-300. doi: 10.1177/107327481402100405. PMID: 25310210

Recent clinical studies

Etiology

Cournoyer E, Ferrell J, Sharp S, Ray A, Jordan M, Dandoy C, Grimley M, Roy S, Lorsbach R, Merrow AC, Nelson A, Bartlett A, Picarsic J, Kumar A
Haematologica 2024 Apr 1;109(4):1137-1148. doi: 10.3324/haematol.2023.283295. PMID: 37731389Free PMC Article
Fraitag S, Emile JF
Histopathology 2022 Jan;80(1):196-215. doi: 10.1111/his.14569. PMID: 34958507
Gulati N, Allen CE
Hematol Oncol 2021 Jun;39 Suppl 1(Suppl 1):15-23. doi: 10.1002/hon.2857. PMID: 34105821Free PMC Article
Hernández-San Martín MJ, Vargas-Mora P, Aranibar L
Actas Dermosifiliogr (Engl Ed) 2020 Nov;111(9):725-733. Epub 2020 Jul 25 doi: 10.1016/j.ad.2020.07.004. PMID: 32721389
Pan Z, Xu ML
Surg Pathol Clin 2019 Sep;12(3):805-829. Epub 2019 Jun 8 doi: 10.1016/j.path.2019.03.013. PMID: 31352989

Diagnosis

Garcia-Sirvent L, Espineira-Sicre J, Ruiz-Sanchez J, Cuesta-Montero L
Dermatol Online J 2024 Mar 15;30(1) doi: 10.5070/D330163291. PMID: 38762861
Pediatr Dermatol 2022 Jan;39(1):119-120. doi: 10.1111/pde.14920. PMID: 35106819
Hernández-San Martín MJ, Vargas-Mora P, Aranibar L
Actas Dermosifiliogr (Engl Ed) 2020 Nov;111(9):725-733. Epub 2020 Jul 25 doi: 10.1016/j.ad.2020.07.004. PMID: 32721389
Hernandez-Martin A, Baselga E, Drolet BA, Esterly NB
J Am Acad Dermatol 1997 Mar;36(3 Pt 1):355-67; quiz 368-9. doi: 10.1016/s0190-9622(97)80207-1. PMID: 9091465
Ringel E, Moschella S
Arch Dermatol 1985 Dec;121(12):1531-41. PMID: 2998286

Therapy

Cournoyer E, Ferrell J, Sharp S, Ray A, Jordan M, Dandoy C, Grimley M, Roy S, Lorsbach R, Merrow AC, Nelson A, Bartlett A, Picarsic J, Kumar A
Haematologica 2024 Apr 1;109(4):1137-1148. doi: 10.3324/haematol.2023.283295. PMID: 37731389Free PMC Article
Geerlinks AV, Abla O
Paediatr Drugs 2023 Jul;25(4):399-409. Epub 2023 May 19 doi: 10.1007/s40272-023-00569-8. PMID: 37204611
Irmola T, Laitinen MK, Parkkinen J, Engellau J, Neva MH
Eur Spine J 2018 Jul;27(Suppl 3):555-560. Epub 2018 Jun 6 doi: 10.1007/s00586-018-5651-8. PMID: 29876657
Haroun F, Millado K, Tabbara I
Anticancer Res 2017 Jun;37(6):2777-2783. doi: 10.21873/anticanres.11629. PMID: 28551613
Walton W, Von Hagen S, Grigorian R, Zarbin M
Surv Ophthalmol 2002 Jul-Aug;47(4):297-334. doi: 10.1016/s0039-6257(02)00317-x. PMID: 12161209

Prognosis

Pan Z, Xu ML
Surg Pathol Clin 2019 Sep;12(3):805-829. Epub 2019 Jun 8 doi: 10.1016/j.path.2019.03.013. PMID: 31352989
Ranganathan S
Semin Diagn Pathol 2016 Nov;33(6):396-409. Epub 2016 Sep 1 doi: 10.1053/j.semdp.2016.08.009. PMID: 27720561
Read RW, Zamir E, Rao NA
Surv Ophthalmol 2002 Mar-Apr;47(2):81-124. doi: 10.1016/s0039-6257(01)00305-8. PMID: 11918892
Hernandez-Martin A, Baselga E, Drolet BA, Esterly NB
J Am Acad Dermatol 1997 Mar;36(3 Pt 1):355-67; quiz 368-9. doi: 10.1016/s0190-9622(97)80207-1. PMID: 9091465
Ringel E, Moschella S
Arch Dermatol 1985 Dec;121(12):1531-41. PMID: 2998286

Clinical prediction guides

Zhou EY, Yu F, Wang Y, Bi Y, Tuan H, Huang Y, Wang B, Li J, Yin H, Li L, Zhao Y
Eur J Dermatol 2023 Jun 1;33(3):270-279. doi: 10.1684/ejd.2023.4502. PMID: 37594335
García-Martínez FJ, Hernández-Martín A
Actas Dermosifiliogr 2023 Mar;114(3):187-193. Epub 2022 Nov 9 doi: 10.1016/j.ad.2022.10.036. PMID: 36370836
Peruilh-Bagolini L, Silva-Astorga M, Hernández San Martín MJ, Manoli MS, Papageorgiou C, Apalla Z, Lallas A
Dermatology 2021;237(6):946-951. Epub 2020 Oct 19 doi: 10.1159/000510265. PMID: 33075787
Ferrari F, Masurel A, Olivier-Faivre L, Vabres P
JAMA Dermatol 2014 Jan;150(1):42-6. doi: 10.1001/jamadermatol.2013.6434. PMID: 24258576
Zvulunov A, Barak Y, Metzker A
Arch Dermatol 1995 Aug;131(8):904-8. PMID: 7632061

Recent systematic reviews

Zou T, Wei A, Ma H, Lian H, Liu Y, Wang D, Zhao Y, Cui L, Li Z, Zhang R, Wang T
Pediatr Blood Cancer 2023 May;70(5):e30232. Epub 2023 Feb 13 doi: 10.1002/pbc.30232. PMID: 36779547
Gioco G, Rupe C, Basco A, Contaldo M, Gallenzi P, Lajolo C
Oral Surg Oral Med Oral Pathol Oral Radiol 2022 Jan;133(1):42-49. Epub 2021 Oct 8 doi: 10.1016/j.oooo.2021.09.015. PMID: 34736900
Murphrey M, Huy Nguyen A, White KP, Krol A, Bernert R, Yarbrough K
Pediatr Dermatol 2020 Mar;37(2):320-325. Epub 2020 Jan 12 doi: 10.1111/pde.14043. PMID: 31930561
Zvulunov A, Barak Y, Metzker A
Arch Dermatol 1995 Aug;131(8):904-8. PMID: 7632061

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