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Long-term tumor-free survival case of congenital embryonal tumor with various pathological components
Junko Sato1, Norihito Shimamura, Masato Naraoka
1Department of Neurosurgery, Hirosaki University School of Medicine, 5-Zaihuchou, Hirosaki, Aomori, Japan.
Insights
A rare congenital embryonal brain tumor initially diagnosed as meningioma recurred aggressively. Multimodal adjuvant therapy including chemotherapy and radiation led to a 34-month tumor-free survival in this infant case.
Area of Science:
- Pediatric neuro-oncology
- Congenital brain tumors
- Rare pediatric neoplasms
Background:
- Malignant congenital brain tumors present complex treatment challenges.
- Early diagnosis and intervention are critical for infant outcomes.
Observation:
- An infant presented with macrocephaly and a large right-sided brain tumor identified via magnetic resonance imaging (MRI).
- Initial subtotal tumor removal revealed a meningioma, but the tumor recurred aggressively with diverse embryonal components.
Findings:
- The recurrent tumor exhibited a high MIB-1 proliferation index (48%) and disseminated rapidly.
- A combination of chemotherapy (vincristine, cisplatin, cyclophosphamide, etoposide) and radiation therapy was initiated.
- Adjuvant therapies resulted in significant tumor regression and a 34-month disease-free interval.
Implications:
- This case highlights the diagnostic difficulties and aggressive nature of some congenital brain tumors.
- Multimodal adjuvant therapy can be effective in managing recurrent, malignant embryonal brain tumors in infants.
- Further discussion on treatment strategies for these rare and challenging pediatric malignancies is warranted.
Purpose:
Treatment strategy of malignant congenital brain tumor is controversial. We report a congenital embryonal tumor case with various pathological components.
Methods:
A normally delivered male infant had an enlarged head circumference at 1 month after birth. The abnormality of the right side of the head was also noted during the routine 4-month health check. The head circumference was 45.1 cm (+2.25, SD); neurological status, however, was normal, with a pediatric GCS of 9 and body weight of 6,370 g (-0.85, SD). Magnetic resonance imaging (MRI) revealed right brain tumor whose size was 99 × 91 × 86 mm. The tumor was enhanced homogeneously with central necrosis, and the margin of the tumor was well circumscribed.
Results:
We performed a subtotal removal of the tumor. The pathological diagnosis was meningioma (MIB-1 index was 2 %). The residual tumor gradually shrank, and we performed monthly MRI follow-up. The tumor abruptly recurred 7 months after the operation. The level of patient consciousness deteriorated, and emergency removal surgery was performed. The histological examination showed various types of embryonal components without meningioma-like parts. The pathological diagnosis was an embryonal tumor. The MIB-1 index was 48 %. One month after the second operation, dissemination of the tumor occurred at the right temporal lobe, cerebellum, and in subcutaneous tissue. Chemotherapy (vincristine, cisplatin, cyclophosphamide, and etoposide) was initiated following radiation therapy (3 Gy/day, 8×). Adjuvant therapies were effective, and no tumor recurrence was detected during 34 months follow-up.
Conclusion:
Treatment strategies for malignant indefinite diagnosed tumor need to be discussed.
