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Brain and Spinal Tumors Originating from the Germ Line Cells
Tai-Tong Wong1,2,3,4, Min-Lan Tsai5,6, Hsi Chang5
1Graduate Institute of Clinical Medicine, College of Medicine, Taipei Medical University, Taipei, 110, Taiwan. ttwong99@gmail.com.
Advances in Experimental Medicine and Biology
|July 15, 2023
Summary
Central nervous system germ cell tumors (CNS GCTs) are diverse tumors with varied presentation and location. Treatment integrates surgery, chemotherapy, and radiotherapy, with outcomes influenced by tumor type and response.
Area of Science:
- Neuro-oncology
- Pediatric Oncology
- Tumor Biology
Background:
- Primary central nervous system germ cell tumors (CNS GCTs) exhibit geographic, age, and sex variations, with two incidence peaks in infancy and adolescence.
- These tumors are histopathologically diverse, with subtypes including germinoma and nongerminomatous GCT (NGGCT), and can secrete tumor markers like AFP and β-hCG.
- Common intracranial locations include the pineal region and neurohypophysis, often presenting with obstructive hydrocephalus, while spinal GCTs are rare.
Purpose of the Study:
- To provide a comprehensive overview of CNS GCTs, encompassing their classification, clinical presentation, diagnosis, and therapeutic strategies.
- To highlight the heterogeneity of CNS GCTs, including specific subtypes like teratoma with somatic-type malignancy and growing teratoma syndrome.
- To discuss the correlation between clinical manifestations and factors such as age, tumor location, hydrocephalus, and tumor marker levels, emphasizing delayed diagnosis challenges.
Main Methods:
- Review of CNS GCT classification according to WHO standards.
- Analysis of diagnostic work-up including CSF cytology and contrast-enhanced MRI.
- Examination of therapeutic approaches integrating surgery, chemotherapy, and radiotherapy based on tumor characteristics.
Main Results:
- CNS GCTs are classified into germinoma and NGGCT, with NGGCTs (excluding mature teratoma) being malignant.
- Obstructive hydrocephalus affects over 50% of intracranial GCTs (IGCTs).
- Germinomas are highly radiosensitive, while NGGCTs require more intensive treatment; negative prognostic factors include residual disease and high AFP levels.
Conclusions:
- Treatment strategies for CNS GCTs are tailored to tumor location, histology, staging, and marker levels.
- Effective management requires a multimodal approach, including surgery, chemotherapy, and radiotherapy, with ongoing research into molecular profiling for refractory cases.
- Survivors require continuous surveillance for long-term complications such as cognitive dysfunction and hormonal deficits, aiming to improve quality of life.
