CASE REPORT J Neurosurg Pediatr 20:556–560, 2017 J uvenile xanthogranuloma (JXG) is a rare disease af- fecting both cutaneous and extracutaneous sites. 3,7 It was previously considered to be endothelium derived and was thus named “nevoxanthoendothelioma.” 18 It was initially classifed under the family of non-Langerhans cell histiocytoses. 3,7 However, a recently revised classifcation distinguishes cutaneous JXG, which belongs to the family of non-Langerhans cell histiocytoses, from extracutane- ous or disseminated JXG, which is closer to Langerhans histiocytosis. 9 Juvenile xanthogranuloma has a wide spec- trum of clinical presentations and usually occurs before 5 years of age, mostly in the frst 6 months of life. 8 Fre- quently, JXG is revealed by a solitary cutaneous lesion—in up to 81% of cases. 7,13 The lesion consists of a frm nodule and/or a papule or a raised plaque-like lesion with reddish to yellowish coloration. 7 Juvenile xanthogranuloma can also affect multiple skin sites, affect only extracutaneous sites, or involve both cutaneous and extracutaneous sites. 7 Soft tissues, bones, viscera, eyes/orbits, oropharynx, and muscles are the most frequently affected extracutaneous sites. 7,10 Relatively few cases of nervous system involve- ment by JXG have been reported in the literature, with an incidence ranging from 1% to 2.3%. 7,8,13 The lesions can af- fect both central and peripheral nervous systems including the brain (53%), spinal cord (intradural, extramedullary; 13%), trigeminal nerve ganglion (10%), and spinal nerve roots (5%). 8 These lesions are benign and most cases re- gress spontaneously within months to years; 9,21 this is par- ticularly the case with skin lesions. No instance of an in- tramedullary spinal cord lesion of JXG has been reported ABBREVIATIONS JXG = juvenile xanthogranuloma. SUBMITTED March 3, 2017. ACCEPTED June 15, 2017. INCLUDE WHEN CITING Published online September 29, 2017; DOI: 10.3171/2017.6.PEDS17113. Systemic juvenile xanthogranuloma: a case of spontaneous regression of intramedullary spinal cord, cerebral, and cutaneous lesions Anne Morice, MD, 1,2 Sylvie Fraitag, MD, 3 Catherine Miquel, MD, 4 Christian Sainte Rose, MD, PhD, 2 and Stéphanie Puget, MD, PhD 2 Departments of 1 Pediatric Plastic and Maxillofacial Surgery, 2 Neurosurgery, and 3 Pathology, Hôpital Necker, Université Paris Descartes, Sorbonne Paris Cité; and 4 Department of Pathology, Saint-Louis Hospital, Université Paris Diderot, Sorbonne Paris Cité, Paris, France Juvenile xanthogranuloma (JXG) is a rare disease that belongs to the non-Langerhans cell histiocytoses. It presents a wide clinical spectrum, usually occurs before 5 years of age, and is commonly confned to the skin; however, it can af- fect multiple sites, including the nervous system, and can lead to severe disorders. Although JXG is a benign disease that usually regresses spontaneously, several curative treatments have been proposed in cases of organ involvement. Treatment options include corticosteroids, chemotherapy, and radiotherapy; however, these can have severe, long-term adverse effects in children. The authors here describe the frst case of spontaneous resolution of an intramedullary spinal cord lesion of JXG as- sociated with cerebral and cutaneous lesions in a young boy with 9 years of follow-up. The initial neurological symptoms resolved without any surgical or medical treatment. This case shows that extracutaneous lesions of JXG, including those with intramedullary spinal cord involvement, can regress without curative treatment—like cutaneous lesions—although both multidisciplinary care and close follow-up should be implemented. https://thejns.org/doi/abs/10.3171/2017.6.PEDS17113 KEY WORDS juvenile xanthogranuloma; intramedullary spinal cord lesion; spontaneous regression; spine; oncology ©AANS, 2017 J Neurosurg Pediatr Volume 20 • December 2017 556 Unauthenticated | Downloaded 02/16/23 02:39 PM UTC