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Multifocal neuroblastoma: biologic behavior and surgical aspects
E Hiyama1, T Yokoyama, K Hiyama
1Department of General Medicine, Hiroshima University, Faculty of Medicine, School of Medicine, Hiroshima, Japan.
Cancer
|April 13, 2000
Summary
Multifocal neuroblastoma, though rare, often presents with favorable biologic features. Surgical preservation of organ function, particularly adrenal function, is recommended for optimal patient outcomes.
Area of Science:
- Pediatric Oncology
- Cancer Biology
- Surgical Oncology
Background:
- Multifocal neuroblastoma is a rare but increasingly diagnosed condition due to improved screening.
- This study analyzed 8 multifocal neuroblastoma cases treated between 1984-1998 and reviewed 53 published cases.
Observation:
- Most multifocal neuroblastomas were detected via mass screening or incidentally.
- Histologically, tumors were predominantly favorable, lacking MYCN amplification and exhibiting specific gene/protein expression.
- Cellular DNA content indicated near-triploidy, with a low proliferative index and no high telomerase activity.
Findings:
- Multifocal neuroblastomas exhibit favorable biologic characteristics.
- Surgical intervention focused on organ function preservation, especially adrenal glands, yielded positive results.
- Patients treated with multistage or minimally invasive surgery showed excellent prognoses without requiring corticosteroid replacement.
Implications:
- Favorable biologic features suggest a good prognosis for multifocal neuroblastoma.
- Surgical strategies should prioritize organ function preservation, utilizing minimally invasive techniques.
- Multistage surgery is a safe and effective approach for thoracoabdominal multifocal neuroblastomas.