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Paradoxical response of a pineal immature teratoma to combination chemotherapy
1Department of Paediatrics, University of Hong Kong, Queen Mary Hospital, Pokfulam.
Insights
This case study highlights a rare intracranial germ cell tumor in a child. Combined chemotherapy and surgery were crucial for successful treatment and long-term remission.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Tumor Biology
Background:
- Primary intracranial germ cell tumors (GCTs) are rare, particularly in children.
- Accurate diagnosis and effective treatment strategies are critical for improving patient outcomes.
Observation:
- A 5-year-old boy presented with symptoms of increased intracranial pressure, diagnosed via CT scan as a pineal region tumor.
- Biopsy revealed immature teratoma with elevated tumor markers (alpha-fetoprotein and beta-human chorionic gonadotropin), indicating embryonal components.
Findings:
- Neoadjuvant chemotherapy (cisplatin, etoposide, bleomycin) normalized tumor markers but paradoxically increased tumor size.
- Subsequent surgical removal revealed mature teratoma, leading to complete remission with minimal residual deficits.
Implications:
- This case underscores the necessity of integrating neoadjuvant chemotherapy with surgical resection for mixed intracranial GCTs.
- Continuous monitoring of both biochemical and radiological parameters is vital for assessing treatment response and tumor behavior in mixed GCTs.
Abstract:
A case of primary intracranial germ cell tumour is reported in a 5-year-old Chinese boy who presented with symptoms and signs of increased intracranial pressure. Computed tomography (CT) scan of the brain revealed a multicystic tumour over the pineal region. Stereotactic biopsy yielded enteric epithelium and isolated cells with hyperchromatic nuclei and high nuclear-to-cytoplasmic ratio, suggesting the diagnosis of an immature teratoma. Grossly elevated serum and cerebrospinal fluid (CSF) levels of alpha-fetoprotein and mildly elevated levels of beta human chorionic gonadotrophin suggested the simultaneous presence of embryonal tissues. Combination chemotherapy with cisplatin, etoposide, and bleomycin resulted in a gradual but complete normalisation of the serum and CSF tumour markers. Paradoxically, the patient's consciousness deteriorated and repeat CT scan after 3 months of treatment showed that the tumour was actually increasing in size. Complete surgical removal was attempted and histologic sections of the specimen revealed only mature teratomatous tissues. The child is now well and remains disease-free with minimal left hemiparesis 12 months after completion of treatment. This case illustrates the importance of combined neoadjuvant chemotherapy and surgery in the management of intracranial non-germinomatous germ cell tumour harbouring both germ cell and teratomatous components, while monitoring of both biochemical and radiological parameters are necessary in assessing the clinical behaviour of mixed germ cell tumours.