Related Experiment Videos
[Expanding mature pineal teratoma syndrome. Case report]
1Service de Neurochirurgie, CHRU Pontchaillou, 35033 Rennes Cedex 2. ahanna@lsuhsc.edu
Insights
This case study details growing teratoma syndrome in a young boy, highlighting a mature teratoma that grew despite chemotherapy. The successful treatment involved chemotherapy and surgery for complete remission.
Area of Science:
- Neuro-oncology
- Pediatric oncology
- Germ cell tumors
Background:
- Growing teratoma syndrome is a rare complication of mixed germ cell tumors.
- It is characterized by a mature teratoma component that persists or grows during chemotherapy.
Observation:
- A 13-year-old boy presented with intracranial hypertension and a pineal region tumor.
- Elevated human chorionic gonadotrophin (HCG) levels were detected in cerebrospinal fluid (CSF) and serum.
- Despite chemotherapy, the tumor showed significant growth on MRI.
Findings:
- Histological examination revealed a mature teratoma.
- The patient achieved complete remission after surgical resection and radiotherapy.
- This case underscores the complex behavior of growing teratoma syndrome.
Implications:
- Treatment requires a multimodal approach, including chemotherapy for malignant components and surgery for mature teratoma.
- Early diagnosis and tailored treatment are crucial for favorable outcomes in growing teratoma syndrome.
- Further research into the pathogenesis and optimal management of this rare condition is warranted.
Abstract:
We present a case of growing teratoma syndrome of the pineal region. To our knowledge, this is the fourth case reported in the literature. A 13-year-old boy was referred for intracranial hypertension and bilateral papillary edema. CT scan showed a pineal region tumor with obstructive hydrocephalus. After CSF (cerebrospinal fluid) shunting, MRI showed that the tumor had a heterogenous signal enhancement. The tumor marker HCG (human chorionic gonadotrophin) was elevated in CSF and serum. After three cycles of chemotherapy, MRI showed an important increase in tumor size with morphologic modifications. However, HCG in CSF and serum returned to normal. Surgical resection was performed and histological examination of the whole specimen showed mature teratoma. On postoperative MRI, there was a small area of signal enhancement of the left thalamus. Radiotherapy was given. The child was in complete remission 15 months after the diagnosis. Growing teratoma syndrome is a mixed germ cell tumor with a secreting portion that responds to chemotherapy and a non secreting portion of mature teratoma that continues to grow under chemotherapy. The treatment should include chemotherapy for the malignant secreting portion and surgery for the mature teratoma.