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Primary T cell central nervous system lymphoblastic lymphoma in a child: case report and literature review
Marcus D Mazur1, Vijay M Ravindra, Mouied Alashari
1Division of Pediatric Neurosurgery, Department of Neurosurgery, Primary Children's Hospital, University of Utah, 100 North Mario Capecchi Drive, Suite 1475, Salt Lake City, UT, 84113-1100, USA.
Insights
Primary central nervous system lymphoma (PCNSL) in children is rare. A brain biopsy is crucial for diagnosing T cell PCNSL, a challenging condition requiring further research.
Area of Science:
- Pediatric Oncology
- Neuropathology
- Neuro-oncology
Background:
- Primary central nervous system lymphoma (PCNSL) of T cell origin is an uncommon malignancy in pediatric patients.
- Diagnosis of PCNSL in children is often challenging due to non-specific symptoms and the need for definitive tissue confirmation.
Observation:
- A 12-year-old boy presented with neurological symptoms including weakness, nausea, headache, blurred vision, and diplopia.
- Initial investigations including imaging, serum, and cerebrospinal fluid analysis were inconclusive.
- A brain biopsy was essential for the definitive diagnosis of lymphoblastic T cell lymphoma.
Findings:
- T cell PCNSL in pediatric patients is exceedingly rare, with only 18 cases reported in the last 30 years.
- Brain biopsy remains the gold standard for establishing a definitive PCNSL diagnosis in children.
- This case highlights the diagnostic challenges and rarity of T cell PCNSL in this age group.
Implications:
- Increased awareness and earlier diagnosis of pediatric T cell PCNSL are necessary.
- Further research into the molecular biology of T cell PCNSL is needed.
- Development of standardized treatment protocols for pediatric T cell PCNSL is crucial for improved outcomes.
Purpose:
Primary central nervous system lymphoma (PCNSL) of T cell origin is rare in pediatric patients. We report a case of T cell PCNSL in a 12-year-old boy and review the literature to highlight the importance of brain biopsy to definitively establish the diagnosis when PCNSL is suspected.
Case Report:
A 12-year-old boy presented with worsening left-sided weakness, nausea, vomiting, headache, blurred vision, and diplopia. Magnetic resonance imaging revealed right parietal gyral thickening with faint meningeal contrast enhancement. No clear diagnosis was identified after serum testing, cerebrospinal fluid analysis, and cerebral angiography. To establish the diagnosis definitively, a right craniotomy and open, frameless stereotactic biopsy were performed, which yielded the diagnosis of lymphoblastic T cell lymphoma.
Conclusions:
PCNSL of T cell origin in children remains poorly studied, with only 18 detailed cases reported over the last three decades, including this case. Establishing a definitive diagnosis of PCNSL is challenging, and a brain biopsy is often required to obtain enough tissue for pathological analysis. Increasing awareness and identification of children diagnosed with T cell PCNSL is needed to better understand the molecular biology of this disease and develop more standardized treatment regimens.
