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Updated: Jul 14, 2026
10:52
Tumor Engraftment in a Xenograft Mouse Model of Human Mantle Cell Lymphoma
Published on: March 30, 2018
Central nervous system juvenile xanthogranuloma with malignant transformation
Andrea Orsey1, Michele Paessler, Beverly J Lange
1Division of Pediatric Oncology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Pediatric Blood & Cancer
|May 24, 2007
Summary
Juvenile xanthogranuloma (JXG) can rarely affect the central nervous system (CNS). This case shows JXG evolving into a clonal neoplasm, highlighting treatment challenges.
Area of Science:
- Histiocytic disorders
- Neuropathology
- Oncology
Background:
- Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder primarily affecting the skin.
- Central nervous system (CNS) involvement in JXG is exceptionally rare and poses significant diagnostic and therapeutic challenges.
Observation:
- We report a unique case of JXG with diffuse CNS involvement.
- The patient's condition initially responded to treatment but later disseminated to the peritoneum and bone marrow.
Findings:
- Histopathological analysis at dissemination revealed pleomorphic histiocytes with tetraploidy.
- This genetic finding suggests the evolution of JXG into a clonal histiocytic neoplasm.
- Despite aggressive chemotherapy, the patient succumbed to disease progression.
Implications:
- This case underscores the significant clinical and pathological heterogeneity of JXG.
- It highlights the difficulties in managing multi-focal CNS JXG and its potential malignant transformation.
- Further research into the molecular mechanisms and treatment strategies for aggressive JXG is warranted.
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