Osteogenic sarcoma of the skull: long-term outcome of a rare tumor.

BACKGROUND: Osteogenic sarcoma of the skull is uncommon and long-term outcome is not well defined. We review the literature and present a pediatric case of calvarial osteogenic sarcoma with good long-term oncological and cosmetic outcome and excellent quality of life. This case presented major surgical challenges, which are detailed. CASE DESCRIPTION: A 6-year-old boy presented with a painless 5 cm × 5 cm lump over the vertex region. He was neurologically normal. Imaging showed an extensive bony lesion with intradural extension. After incisional biopsy showed probable low grade osteosarcoma, a complete en bloc resection with margins was attempted via a concentric craniotomy around the lesion after embolization to reduce blood loss. Invasion of the brain by the tumor precluded the complete en bloc resection, but gross total resection was achieved. The final pathology was consistent with a low-grade osteosarcoma and adjuvant chemotherapy was provided. Follow-up for 8 years has shown no recurrence with good cosmetic and functional outcome.