An official website of the United States government
Home
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)
C700-C729,
C751-C753
This neoplasm is reportable for primary sites C700-C729, C751-C753 only.
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.
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.
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.
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
Disseminated juvenile xanthogranuloma
Definition
Histology that presents in infants as a single or several cutaneous papulo-nodular lesions. Lesions are general asymptomatic and evolve from raised, pink to dark brown lesion with progressive flattening and can disappear within months or years with scarring or anetodema.
Benign cephalic histiocytosis is a clinical subtype presenting as an asymptomatic papular eruption predominantly in the head and neck of young children with self-healing properties.
In adults, lesions are often large, solitary and persistent and it is important to exclude Erdheim-Chester disease (9749/3)
Benign cephalic histiocytosis is a clinical subtype presenting as an asymptomatic papular eruption predominantly in the head and neck of young children with self-healing properties.
In adults, lesions are often large, solitary and persistent and it is important to exclude Erdheim-Chester disease (9749/3)
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-9 Codes
238.5 Histiocytic and mast cells
Corresponding ICD-10 Codes
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. Hematopoietic Neoplasms [Internet]. Lyon (France): International Agency for Research on Cancer; 2021 [cited 2022/08/22 WHO classification of tumours series, 5th ed.). Available from: https://tumourclassification.iarc.who.int/chapters/63
Section: Section: Histiocytic/Dendritic cell neoplasms
Section: Section: Histiocytic/Dendritic cell neoplasms
WHO Classification of Tumours Editorial Board. Central nervous system tumours [Internet]. Lyon (France): International Agency for Research on Cancer; 2021 [cited 2022/08/22 WHO classification of tumours series, 5th ed.; vol. 6). Available from: https://tumourclassification.iarc.who.int/chapters/45.
Section: Haematolymphoid tumors involving the CNS
Section: Haematolymphoid tumors involving the CNS
