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This neoplasm is not reportable.
Name
Juvenile xanthogranuloma (JXG)
ICD-O-3 Morphology
9749/1
Effective
2023 and later
Reportability
This neoplasm is not reportable
Primary Site(s)
No primary site specified
Cutaneous primary sites only (C440-C449, C510-C512, C518-C519, C600-C602, C608-C609, C632)
This hematopoietic disease is not reportable for the U.S.
Canada, refer to your specific province for reportability requirements.
This hematopoietic disease is not reportable for the U.S.
Canada, refer to your specific province for reportability requirements.
Abstractor Notes
This is a new reportable neoplasm for 2023 for primary sites C700-C729, C751-C753 only. If the diagnosis is prior to 1/1/2023, it is not reportable.
Juvenile xanthogranuloma (JXG) is part of the Histiocyte/macrophage neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B12)
JXG is generally confined to the skin, with a predilection for head and neck, upper trunk, and proximal extremities. Ocular involvement can rarely occur, and is usually solitary.
JXG can also occur in the CNS (brain, intradural extramedullary spin, nerve roots, and meninges.) CNS involvement usually occurs in children and young adults. CNS JXG can cause seizures, hydrocephalus, or diabetes insipidus.
JXG may occur in patients with neurofibromatosis type 1.
Juvenile xanthogranuloma (JXG) is part of the Histiocyte/macrophage neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B12)
JXG is generally confined to the skin, with a predilection for head and neck, upper trunk, and proximal extremities. Ocular involvement can rarely occur, and is usually solitary.
JXG can also occur in the CNS (brain, intradural extramedullary spin, nerve roots, and meninges.) CNS involvement usually occurs in children and young adults. CNS JXG can cause seizures, hydrocephalus, or diabetes insipidus.
JXG may occur in patients with neurofibromatosis type 1.
Diagnostic Confirmation
This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.
Alternate Names
Benign cephalic histiocytosis (clinical subtype of JXG)
Disseminated juvenile xanthogranuloma
Definition
Juvenile xanthogranuloma (JXG) is a clonal expansion of non–Langerhans cell histiocytes that share a dermal macrophage phenotype. (WHO 5th edition)
Definitive Diagnostic Methods
This data item does not apply
Genetics Data
This data item does not apply
Immunophenotyping
This data item does not apply
Corresponding ICD-10 Codes (Cause of Death codes only)
D76.3 Other histiocytosis syndromes
Corresponding ICD-10-CM Codes (U.S. only)
D76.3 Other histiocytosis syndromes (effective October 01, 2015)
Epidemiology and Mortality
Sources
WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Histiocytic/dendritic cell neoplasms
Pages: Part A: 242-244
Section: Histiocytic/dendritic cell neoplasms
Pages: Part A: 242-244
International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
PDQ® Pediatric Treatment Editorial Board. PDQ Langerhans Cell Histiocytosis Treatment. Bethesda, MD: National Cancer Institute. Updated <01/06/2025>. Available at: https://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq. Accessed <02/28/2025>. [PMID: 26389240]
Section: Langerhans Cell Histiocytosis Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq#section/_178%20or%20http://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq
Section: Langerhans Cell Histiocytosis Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq#section/_178%20or%20http://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq
