Juvenile xanthogranuloma manifesting with LCH‐associated neurodegenerative disease‐like radiological findings

• Published in: Pediatric blood & cancer Vol. 71; no. 7; pp. e31043 - n/a
• Main Authors: Daifu, Tomoo, Umeda, Katsutsugu, Yokoyama, Atsushi, Yoshida, Takeshi, Saida, Satoshi, Kato, Itaru, Hiramatsu, Hidefumi, Kudo, Ko, Higuchi, Yoshihisa, Takita, Junko
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01.07.2024
• Subjects: Central nervous system; Chemotherapy; Child; Child, Preschool; Female; histiocytic disorders; Histiocytosis; Histiocytosis, Langerhans-Cell - complications; Histiocytosis, Langerhans-Cell - diagnostic imaging; Histiocytosis, Langerhans-Cell - drug therapy; Histiocytosis, Langerhans-Cell - pathology; Humans; Infant; juvenile xanthogranuloma; Magnetic Resonance Imaging; Male; Neurodegenerative diseases; Neurodegenerative Diseases - complications; Neurodegenerative Diseases - diagnostic imaging; Neurodegenerative Diseases - pathology; Therapeutic applications; Xanthogranuloma, Juvenile - diagnostic imaging; Xanthogranuloma, Juvenile - pathology; juvenile xanthogranuloma; neurodegenerative diseases; histiocytic disorders
• ISSN: 1545-5009; 1545-5017; 1545-5017
• DOI: 10.1002/pbc.31043

Here, we describe two patients with juvenile xanthogranuloma (JXG) manifesting with Langerhans cell histiocytosis (LCH)‐associated neurodegenerative disease (ND)‐like radiological findings. One patient showed typical radiological abnormalities at onset, which worsened with progressing central nervous system symptoms 7 years after LCH‐oriented chemotherapy. Another showed spontaneous regression of clinical symptoms, with a transient radiological change 1 year after salvage chemotherapy for recurrence of JXG. These data regarding JXG‐associated ND will facilitate future investigation of the disease, as well as development of therapeutic interventions.