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. 2023 May;70(5):e30232.
doi: 10.1002/pbc.30232. Epub 2023 Feb 13.

Systemic juvenile xanthogranuloma: A systematic review

Tong Zou  1 , Ang Wei  1 , Honghao Ma  1 , Hongyun Lian  1 , Yali Liu  2 , Dong Wang  1 , Yunze Zhao  1 , Lei Cui  3 , Zhigang Li  3 , Rui Zhang  1 , Tianyou Wang  1
Affiliations

Systemic juvenile xanthogranuloma: A systematic review

Tong Zou et al. Pediatr Blood Cancer. 2023 May.

Abstract

Objective: To perform a systematic review to investigate the available literature regarding systemic juvenile xanthogranuloma (SJXG) and report the population characteristics, clinical manifestation, therapy, and outcome.

Review methods: A search of PubMed, Embase, and Cochrane Library for all articles published between 1981 and 2022 was performed with variations and combinations of the following search terms: extracutaneous, visceral, systemic, and juvenile xanthogranuloma (JXG). Data extracted included demographics, organ involvement, treatment, outcome, and permanent sequelae.

Results: A total of 103 articles encompassing 159 patients met the inclusion criteria. The median onset age was 9 months, with a male predominance (61%). The distribution of major involved organs varied by age, and younger onset age was associated with more organ involvement. The most commonly involved site was the central nervous system (CNS) (40.9%), followed by the liver (31.4%), the lung (18.9%), and the eye (18.2%). At the termination of follow-up, 93 patients (58.5%) were alive with no disease, 56 (35.2%) were alive with disease, and 10 (6.3%) were dead of disease. There was a significant difference in outcome between patients with and without spleen involvement (p = .0003), and patients with spleen involvement suffered a higher risk of death. Permanent sequelae mainly comprised CNS symptoms and ocular manifestations.

Conclusions: SJXG can involve varying numbers and combinations of extracutaneous sites. There is no standard therapy for SJXG and clinicians should choose individualized therapy modalities.

Keywords: congenital; outcome; systemic juvenile xanthogranuloma; treatment.

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