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Child's Nervous System : ChNS :... Jan 2020Juvenile xanthogranuloma (JXG) with the central nervous system (CNS) involvement is a rare disease entity that remains poorly understood, especially when the condition... (Review)
Review
INTRODUCTION
Juvenile xanthogranuloma (JXG) with the central nervous system (CNS) involvement is a rare disease entity that remains poorly understood, especially when the condition develops following treatment for Langerhans cell histiocytosis (LCH).
CASE REPORT
A 21-year-old man who was diagnosed with LCH at age 2, several years following which he developed signs and symptoms of CNS involvement. Magnetic resonance imaging (MRI) of the brain revealed JXG with bilateral choroid plexus involvement. As radiation therapy for the intraventricular masses proved unsuccessful, he underwent two surgical resections. In the following years, he developed another large JXG in the meninges, which was managed conservatively until he required surgery due to symptom progression. Twelve years after the first surgery, the patient is in stable condition with no evidence of recurrence.
CONCLUSION
Due to the rarity of JXG in the CNS, optimal treatment strategies and the precise duration of therapy remain to be determined. Future studies should aim to develop an appropriate treatment algorithm for such rare cases.
Topics: Adult; Algorithms; Brain; Histiocytosis, Langerhans-Cell; Humans; Magnetic Resonance Imaging; Male; Xanthogranuloma, Juvenile; Young Adult
PubMed: 31385088
DOI: 10.1007/s00381-019-04323-9 -
The Journal of Pediatrics Apr 2021
Topics: Humans; Infant; Male; Xanthogranuloma, Juvenile
PubMed: 33326759
DOI: 10.1016/j.jpeds.2020.12.013 -
Acta Neurochirurgica Jun 2013
Review
Topics: Humans; Infant; Magnetic Resonance Imaging; Male; Skull Base Neoplasms; Treatment Outcome; Xanthogranuloma, Juvenile
PubMed: 23605373
DOI: 10.1007/s00701-013-1718-9 -
Dermatology (Basel, Switzerland) 2009
Topics: Adolescent; Child; Dermoscopy; Humans; Male; Skin; Xanthogranuloma, Juvenile
PubMed: 18997453
DOI: 10.1159/000172831 -
South African Medical Journal =... May 1972
Topics: Child; Diagnosis, Differential; Female; Granuloma; Humans; Infant; Infant, Newborn; Infant, Premature, Diseases; Lymphatic Diseases; Male; Skin Neoplasms; Xanthogranuloma, Juvenile
PubMed: 4627754
DOI: No ID Found -
Zhonghua Xue Ye Xue Za Zhi = Zhonghua... Sep 2011To report the clinical characteristics and treatment of 3 patients with juvenile xanthogranuloma (JXG). (Review)
Review
OBJECTIVE
To report the clinical characteristics and treatment of 3 patients with juvenile xanthogranuloma (JXG).
METHODS
A retrospective review of the medical records of 3 patients with JXG.
RESULTS
JXG was characterized by solitary or multiple yellowish cutaneous nodules, or eye involvement . It could also affect pituitary. JXG was easily misdiagnosed as Langerhans cell histiocytosis (LCH). Treatment for JXG was surgical excision of a solitary skin lesion and some cases might be, spontaneous regression. In cases with multisystem involvement, chemotherapy regimens used to treat LCH may be effective.
CONCLUSIONS
JXG is one of the more common non-Langerhans histiocytic proliferations and is frequently seen in infants and children. LCH-like chemotherapy is effective for patients with symptomatic multisystem JXG.
Topics: Child, Preschool; Female; Humans; Infant; Male; Xanthogranuloma, Juvenile
PubMed: 22338156
DOI: No ID Found -
Canadian Journal of Ophthalmology.... Feb 2022
Topics: Administration, Topical; Glucocorticoids; Humans; Infant; Iris; Iris Diseases; Xanthogranuloma, Juvenile
PubMed: 34077746
DOI: 10.1016/j.jcjo.2021.04.022 -
European Journal of Pediatric Surgery :... Dec 1994Juvenile xanthogranuloma (JXG) is a rare affliction of early childhood comprising cutaneous and deep-seated lesions. Accurate diagnosis is important as the condition is... (Review)
Review
Juvenile xanthogranuloma (JXG) is a rare affliction of early childhood comprising cutaneous and deep-seated lesions. Accurate diagnosis is important as the condition is self-limiting with spontaneous regression over a period of months. A case of congenital JXG is reported and the literature briefly reviewed.
Topics: Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Remission, Spontaneous; Skin; Xanthogranuloma, Juvenile
PubMed: 7748839
DOI: 10.1055/s-2008-1066137 -
Pediatric Radiology 1992Two patients with different patterns of soft tissue and bone involvement demonstrate the variability of extracutaneous manifestations of juvenile xanthogranuloma, a... (Review)
Review
Two patients with different patterns of soft tissue and bone involvement demonstrate the variability of extracutaneous manifestations of juvenile xanthogranuloma, a non-X histiocytosis, better known to dermatologists than to radiologists.
Topics: Female; Humans; Infant; Infant, Newborn; Xanthogranuloma, Juvenile
PubMed: 1408450
DOI: 10.1007/BF02016261 -
European Journal of Dermatology : EJD Sep 2022
Topics: Humans; Xanthogranuloma, Juvenile
PubMed: 36468726
DOI: 10.1684/ejd.2022.4327