Pineal germ cell tumors: review

Area of Science:

• Neuro-oncology
• Pediatric Oncology
• Neurosurgery

Background:

• Intracranial germ cell tumors (GCTs) are rare neoplasms affecting children and young adults, classified as germinomas or non-germinomas.
• Commonly located in the pineal and suprasellar regions, GCTs present with symptoms like increased intracranial pressure, Parinaud's syndrome, and endocrine deficiencies.
• While MRI is crucial for diagnosis, histological confirmation via biopsy is often necessary, unless tumor markers like AFP and β-HCG are characteristically elevated.

Observation:

• A 26-year-old male presented with visual disturbances, fatigue, nausea, headaches, and Parinaud's syndrome, indicative of a pineal germ cell tumor.
• MRI revealed tumor expansion in the pineal region and mesencephalon. Following radical tumor extirpation, a relapse was observed.
• The patient received craniospinal radiation therapy and three cycles of cisplatin and etoposide chemotherapy, achieving clinical remission 30 months post-treatment.

Findings:

• Germinomas exhibit significantly better survival rates (>90% 5-year survival with radiotherapy alone) compared to non-germinomas.
• Adjuvant chemotherapy in germinoma treatment allows for reduced radiation doses and smaller irradiated areas, minimizing side effects without compromising curability.
• Non-germinomas, while less radiosensitive, demonstrate improved survival with adjuvant chemotherapy, though optimal management strategies remain under investigation.

Implications:

• This case highlights the efficacy of combined radiotherapy and chemotherapy in managing recurrent intracranial germ cell tumors.
• The findings underscore the importance of multimodal treatment approaches in improving outcomes for patients with GCTs.
• Further research is warranted to optimize treatment protocols for different subtypes of intracranial germ cell tumors, particularly non-germinomas.