# A Rare Case of Intramedullary Juvenile Xanthogranuloma of the Cervical Spine in an Adult: Cytological Features Aiding Diagnosis

**Authors:** Dung A Vu, Motona Kumagai, Yao Liu, Akihiro Shioya, Sohsuke Yamada

PMC · DOI: 10.7759/cureus.82068 · Cureus · 2025-04-11

## TL;DR

This paper reports a rare case of a spinal tumor in an adult, highlighting how cell analysis helped confirm the diagnosis.

## Contribution

The first reported case of intramedullary cervical spinal JXG in an adult, emphasizing the role of cytopathology in diagnosis.

## Key findings

- MRI identified a lesion at C3-C4 in a 39-year-old man with numbness.
- Histopathology and immunohistochemistry confirmed the diagnosis of JXG.
- Cytopathology provided definitive diagnostic precision for this rare condition.

## Abstract

Juvenile xanthogranuloma (JXG), the most common non-Langerhans cell histiocytosis (non-LCH), typically manifests as a benign cutaneous condition in children but is rare in adults, with extracutaneous cervical spine involvement being uncommon. To the best of our knowledge, we present the first case of intramedullary cervical spinal JXG in a 39-year-old man with persistent right upper extremity numbness. MRI identified a lesion at C3-C4, and surgical resection revealed a 7-mm yellow-whitish nodule. Histopathology demonstrated foamy histiocytes, multinucleated giant cells, and inflammatory infiltrates, with immunohistochemistry positive for CD68, CD163, and vimentin, and negative for CD1a and S-100 protein, distinguishing it from LCH. Cytopathology was instrumental, revealing low cellularity, mononuclear histiocytes, and CD68-positive granules, offering definitive diagnostic precision in this rare presentation. This case underscores cytopathology’s critical role as an essential tool in accurately diagnosing atypical JXG manifestations.

## Linked entities

- **Proteins:** CD68 (CD68 molecule), CD163 (CD163 molecule), PRELID1 (PRELI domain containing 1), CD1A (CD1a molecule)
- **Diseases:** Juvenile xanthogranuloma (MONDO:0015534), non-Langerhans cell histiocytosis (MONDO:0015531), LCH (MONDO:0018310)

## Full-text entities

- **Genes:** CD68 (CD68 molecule) [NCBI Gene 968] {aka GP110, LAMP4, SCARD1}, CD163 (CD163 molecule) [NCBI Gene 9332] {aka M130, MM130, SCARI1}, VIM (vimentin) [NCBI Gene 7431], CD1A (CD1a molecule) [NCBI Gene 909] {aka CD1, FCB6, HTA1, R4, T6}
- **Diseases:** non-LCH (MESH:D015616), Xanthogranuloma of the Cervical Spine (MESH:D002575), numbness (MESH:D006987), inflammatory (MESH:D007249), JXG (MESH:D014972)

## Full text

_Full body text omitted from this summary view._ Fetch the complete paper as Markdown: https://tomesphere.com/paper/PMC11987050/full.md

## Figures

2 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11987050/full.md

## References

14 references — full list in the complete paper: https://tomesphere.com/paper/PMC11987050/full.md

---
Source: https://tomesphere.com/paper/PMC11987050