British Journal of Neurosurgery, December 2014; 28(6): 817–818 © 2014 The Neurosurgical Foundation ISSN: 0268-8697 print / ISSN 1360-046X online DOI: 10.3109/02688697.2014.918582

NEUROSURGICAL IMAGE

Recurrent juvenile xanthogranuloma manifesting as diffuse miliary brain lesions Lee A. Tan, Benjamin R. Uy & Lorenzo F. Munoz Departments of Neurosurgery, Rush University Medical Center, Chicago, IL, USA

ease that presents cutaneous lesions or nodules in infancy and early childhood. Extra-cutaneous JXG is much less common but can involve sites such as the gastrointestinal tract, liver, spleen, and lungs.2–6 JXG involving the central nervous system is very rare with only less than 30 cases reported in the literature.1–3 Treatment options for CNS JXG include steroid, chemotherapy, radiation, or surgical resection. We report this rare case to highlight that although rare, JXA can recur as diffuse, innumerable intracranial lesions many years after the initial diagnosis. Clinical suspicion for extra-cutaneous JXG recurrence and dissemination should remain high in this group of patients.

A 23-year-old man with a past medical history of cutaneous juvenile xanthogranuloma (JXG), diagnosed at 1 month of age via skin biopsy and acute myeloid leukemia (AML) status post chemotherapy, presented with confusion and altered mental status. Infectious workup was negative. Bone marrow biopsy showed his AML was in remission. Magnetic resonance imaging (MRI) of the brain demonstrated innumerable, diffuse, miliary, enhancing lesions trough out the brain (Fig. 1A–C). An open brain biopsy was done and the pathology features of the specimen were consistent with JXG. Steroids and chemotherapy were used to treat CNS JXG, and repeat brain MRI at 12 weeks revealed complete resolution of the intracranial lesions (Fig. 1D–F). JXG is the most common non-Langerhan cell histocytic disorder originating primarily from dermal dendrocytes.1 It is usually a benign, self-limiting, and self-resolving dis-

Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.

Fig. 1. A, B & C – Pre-operative MRI T1-weighted with contrast demonstrating innumerable enhancing lesions throughout the brain parenchyma; D, E, F – Repeat MRI 12 weeks after treatment showing complete resolution of previous brain lesions.

Correspondence: Lee A. Tan, MD, Departments of Neurosurgery, Rush University Medical Center, 1725 W. Harrison St. Suite 855, Chicago, IL 60612, USA. Tel: ⫹ 312-942-6644. Fax: ⫹ 312-563-3358. E-mail: [email protected] Received for publication 15 January 2014; accepted 21 April 2014

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References 1. Gressot LV, Patel AJ, Bollo RJ, Mohila CA , Jea A . Disseminated intracranial juvenile xanthogranulomatosis in a neonate without cutaneous lesions: case report. J Neurosurg Pediatr 2013;12:187–91. 2. Orsey A , Paessler M, Lange BJ, Nichols KE. Central nervous system juvenile xanthogranuloma with malignant transformation. Pediatr Blood Cancer 2008;50:927–30. 3. Chiba K , Aihara Y, Eguchi S, et al. Diagnostic and management difficulties in a case of multiple intracranial juvenile xanthogranuloma. Child’s Nerv Syst 2013;29:1039–45.

4. Dölken R, Weigel S, Schröder H, et al. Treatment of severe disseminated juvenile systemic xanthogranuloma with multiple lesions in the central nervous system. J Pediatr Hematol Oncol 2006;28:95–7. 5. Meshkini A , Shahzadi S, Zali A , et al. Systemic juvenile xanthogranuloma with multiple central nervous system lesions. J Cancer Res Ther 2012;8:311–3. 6. Dehner LP. Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol 2003 ; 27 : 579 –93 .

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Recurrent juvenile xanthogranuloma manifesting as diffuse miliary brain lesions.

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