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A 14-year-old girl with multiple tumors
Marina Paola Gardiman1, Matteo Fassan, Enrico Orvieto
1Department of Medicine, Surgical Pathology & Cytopathology Unit, University Hospital of Padua, 35100, Padua, Italy.
Brain Pathology (Zurich, Switzerland)
|October 12, 2012
Summary
This report details a rare multicentric pleomorphic xanthoastrocytoma (PXA) with leptomeningeal spread in a young patient. The case highlights an unusual presentation of this typically favorable prognosis brain tumor.
Area of Science:
- Neuro-oncology
- Pediatric Neurosurgery
- Pathology
Background:
- Pleomorphic xanthoastrocytoma (PXA) is a rare, typically low-grade (WHO grade II) astrocytic tumor.
- PXA commonly affects children and young adults, usually in the cerebral hemispheres, with a generally favorable prognosis.
Observation:
- A 14-year-old girl presented with sciatica and ataxic gait.
- Brain and spinal MRI revealed multiple solid, cystic lesions in the cerebellum and spinal cord.
- Extensive leptomeningeal involvement was observed concurrently with the primary lesions.
Findings:
- Histological and immunohistochemical analyses confirmed the diagnosis of WHO grade II PXA.
- The tumor exhibited multicentricity and leptomeningeal dissemination, an uncommon presentation for PXA.
- This represents the third reported case of multicentric PXA with leptomeningeal spread in the literature.
Implications:
- This case underscores the potential for pleomorphic xanthoastrocytoma to present with unusual patterns, including multicentricity and leptomeningeal dissemination.
- Further research may be needed to understand the biological behavior and optimal management strategies for such rare presentations.
- Clinicians should consider PXA in the differential diagnosis of pediatric patients with spinal and cerebellar lesions, even with disseminated disease.
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