Morice Anne, Fraitag Sylvie, Miquel Catherine, Rose Christian Sainte, Puget Stéphanie
Departments of1Pediatric Plastic and Maxillofacial Surgery.
2Neurosurgery, and.
J Neurosurg Pediatr. 2017 Dec;20(6):556-560. doi: 10.3171/2017.6.PEDS17113. Epub 2017 Sep 29.
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 confined to the skin; however, it can affect 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 first case of spontaneous resolution of an intramedullary spinal cord lesion of JXG associated 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.
幼年性黄色肉芽肿（JXG）是一种罕见疾病，属于非朗格汉斯细胞组织细胞增多症。它具有广泛的临床谱，通常在5岁前发病，且通常局限于皮肤；然而，它可累及多个部位，包括神经系统，并可导致严重疾病。尽管JXG是一种通常会自发消退的良性疾病，但对于器官受累的病例，已提出了几种治愈性治疗方法。治疗选择包括皮质类固醇、化疗和放疗；然而，这些治疗方法可能会对儿童产生严重的长期不良反应。本文作者描述了首例幼年性黄色肉芽肿脊髓内病变自发消退的病例，该病例伴有脑和皮肤病变，对一名小男孩进行了9年的随访。最初的神经症状未经任何手术或药物治疗即得以缓解。该病例表明，JXG的皮肤外病变，包括那些累及脊髓内的病变，可像皮肤病变一样在未经治愈性治疗的情况下消退——尽管应实施多学科护理和密切随访。
