Neuro / Head & Neck for Thursday, April 10th, 2025

History

15-year-old girl with a remote history of B -ALL, presented with two weeks of headache, fever and myalgias. 

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Question

What is the most likely diagnosis for this patient?

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Correct answer

Juvenile Xanthogranuloma

Discussion

Juvenile xanthogranuloma JXG is a rare clonal, myeloid, neoplastic disorder composed of non-Langerhans cell histiocytes. They often present as self limited condition in infancy with skin lesions however any organ system can be involved. Extracutaneous systemic involvement may include the eyes, oral cavity, heart, lung ,gastrointestinal tract, kidneys, bone marrow etc. 

CNS involvement observed in about 7% of cases with three primary patterns: unifocal, multifocal and multifocal leptomeningeal. CNS juvenile xanthogranuloma may demonstrate varied neuroimaging appearances and can involve any site including supratentorial, infratentorial, extra axial spaces or spine. Typical imaging features include small to medium size masses, isointense on T1WI, iso to hyperintense on T2WI, reduced diffusivity, perilesional edema and avid homogenous contrast enhancement. 

Differential diagnosis

Differential diagnoses for unifocal disease as in our case include primary brain neoplasm, lymphoma, chloroma, other histolytic disorders such as LCH. Multifocal disease can mimic metastatic disease, infections or granulomatous diseases including tuberculosis.

References

• Serrallach BL, Kralik SF, Tran BH, et al. Neuroimaging in Pediatric Patients with Juvenile Xanthogranuloma of the CNS. AJNR Am J Neuroradiol. 2022;43(11):1667-1673. doi:10.3174/ajnr.A7683
• Chen Y, Yang Z, Shi J, Qiao Z. Imaging features of juvenile xanthogranuloma. Pediatr Radiol. 2023;53(2):265-272. doi:10.1007/s00247-022-05486-5
• Ginat DT, Vargas SO, Silvera VM, Volk MS, Degar BA, Robson CD. Imaging Features of Juvenile Xanthogranuloma of the Pediatric Head and Neck. AJNR Am J Neuroradiol. 2016;37(5):910-916. doi:10.3174/ajnr.A4644