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Disseminated Juvenile Xanthogranuloma

MedGen UID:
414731
•Concept ID:
C2825742
•
Neoplastic Process
Synonym: Disseminated JXG

Definition

Juvenile xanthogranuloma disseminated to extracutaneous sites including mucosal surfaces, lung, central nervous system, pituitary, lymph nodes, eye, liver, and bone marrow. It is characterized by the presence of lipid-laden, foamy histiocytes and Touton-type giant cells forming nodules in the affected anatomic sites. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDisseminated Juvenile Xanthogranuloma

Professional guidelines

PubMed

Dendritic cell and histiocytic neoplasms: biology, diagnosis, and treatment.
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

Descriptive Analysis of Histiocytic and Dendritic Cell Neoplasms: A Single-Institution Experience.
Kim HM, Yang WI, Lyu CJ, Hahn SM, Yoon SO
Yonsei Med J 2020 Sep;61(9):774-779. doi: 10.3349/ymj.2020.61.9.774. PMID: 32882761Free PMC Article
Histiocytic and Dendritic Cell Neoplasms.
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
Histiocytic cell neoplasms involving the bone marrow: summary of the workshop cases submitted to the 18th Meeting of the European Association for Haematopathology (EAHP) organized by the European Bone Marrow Working Group, Basel 2016.
Tzankov A, Kremer M, Leguit R, Orazi A, van der Walt J, Gianelli U, Hebeda KM
Ann Hematol 2018 Nov;97(11):2117-2128. Epub 2018 Aug 6 doi: 10.1007/s00277-018-3436-0. PMID: 30084011Free PMC Article
Disseminated juvenile xanthogranuloma occurring after treatment of Langerhans cell histiocytosis: a case report.
Lee TK, Jung TY, Baek HJ, Kim SK, Lee KH, Yun SJ
Childs Nerv Syst 2018 Apr;34(4):765-770. Epub 2017 Dec 5 doi: 10.1007/s00381-017-3675-8. PMID: 29209883

Diagnosis

Pediatric liver failure with massive sinusoidal infiltration of histiocytes.
Irie R, Shioda Y, Osumi T, Sakamoto KI, Kasahara M, Matsumoto K, Nakazawa A
J Clin Exp Hematop 2022 Mar 9;62(1):25-34. Epub 2021 Nov 26 doi: 10.3960/jslrt.21022. PMID: 34840207Free PMC Article
Acute Liver Failure due to Disseminated Juvenile Xanthogranuloma.
Kakiuchi T, Nakayama A, Matsuo M
Indian J Pediatr 2021 Aug;88(8):841-842. Epub 2020 Jul 15 doi: 10.1007/s12098-020-03389-7. PMID: 32671637
Descriptive Analysis of Histiocytic and Dendritic Cell Neoplasms: A Single-Institution Experience.
Kim HM, Yang WI, Lyu CJ, Hahn SM, Yoon SO
Yonsei Med J 2020 Sep;61(9):774-779. doi: 10.3349/ymj.2020.61.9.774. PMID: 32882761Free PMC Article
Histiocytic and Dendritic Cell Neoplasms.
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
Dendritic cell and histiocytic neoplasms: biology, diagnosis, and treatment.
Dalia S, Shao H, Sagatys E, Cualing H, Sokol L
Cancer Control 2014 Oct;21(4):290-300. doi: 10.1177/107327481402100405. PMID: 25310210

Therapy

Successful Salvage Treosulfan-Based Megachemotherapy With Allogeneic Stem Cell Transplantation in Nonsyndromic, Therapy-Resistant Disseminated Juvenile Xanthogranuloma: A Case Report.
Janeczko-Czarnecka M, Raciborska A, Gos M, Kordowska O, Gajek K, Rybka B, Ryczan-Krawczyk R, Kałwak K, Ussowicz M
Transplant Proc 2020 Nov;52(9):2844-2848. Epub 2020 Jul 16 doi: 10.1016/j.transproceed.2020.05.025. PMID: 32684371
Disseminated juvenile xanthogranuloma occurring after treatment of Langerhans cell histiocytosis: a case report.
Lee TK, Jung TY, Baek HJ, Kim SK, Lee KH, Yun SJ
Childs Nerv Syst 2018 Apr;34(4):765-770. Epub 2017 Dec 5 doi: 10.1007/s00381-017-3675-8. PMID: 29209883
Disseminated juvenile xanthogranuloma associated with follicular lymphoma in an adult: successful treatment with chemotherapy and rituximab. A review of the literature.
Narváez-Moreno B, Pulpillo-Ruiz Á, De Zulueta-Dorado T, Conejo-Mir J
Actas Dermosifiliogr 2013 Apr;104(3):242-6. Epub 2012 Jun 6 doi: 10.1016/j.ad.2012.02.006. PMID: 22681714

Prognosis

Histiocytic and Dendritic Cell Neoplasms.
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
Disseminated juvenile xanthogranuloma occurring after treatment of Langerhans cell histiocytosis: a case report.
Lee TK, Jung TY, Baek HJ, Kim SK, Lee KH, Yun SJ
Childs Nerv Syst 2018 Apr;34(4):765-770. Epub 2017 Dec 5 doi: 10.1007/s00381-017-3675-8. PMID: 29209883
Dendritic cell and histiocytic neoplasms: biology, diagnosis, and treatment.
Dalia S, Shao H, Sagatys E, Cualing H, Sokol L
Cancer Control 2014 Oct;21(4):290-300. doi: 10.1177/107327481402100405. PMID: 25310210

Clinical prediction guides

Successful Salvage Treosulfan-Based Megachemotherapy With Allogeneic Stem Cell Transplantation in Nonsyndromic, Therapy-Resistant Disseminated Juvenile Xanthogranuloma: A Case Report.
Janeczko-Czarnecka M, Raciborska A, Gos M, Kordowska O, Gajek K, Rybka B, Ryczan-Krawczyk R, Kałwak K, Ussowicz M
Transplant Proc 2020 Nov;52(9):2844-2848. Epub 2020 Jul 16 doi: 10.1016/j.transproceed.2020.05.025. PMID: 32684371
Histiocytic cell neoplasms involving the bone marrow: summary of the workshop cases submitted to the 18th Meeting of the European Association for Haematopathology (EAHP) organized by the European Bone Marrow Working Group, Basel 2016.
Tzankov A, Kremer M, Leguit R, Orazi A, van der Walt J, Gianelli U, Hebeda KM
Ann Hematol 2018 Nov;97(11):2117-2128. Epub 2018 Aug 6 doi: 10.1007/s00277-018-3436-0. PMID: 30084011Free PMC Article

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