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Primary angiosarcoma of the skull: A rare case report
Ashish P Chugh1, Charandeep Singh Gandhoke1, Anirudha G Mohite1
1Department of Surgery (Neurosurgery unit), B. J. Medical College and Sassoon General Hospitals, Pune, Maharashtra, India.
Surgical Neurology International
|July 16, 2014
Summary
Primary angiosarcoma of the skull is an extremely rare, aggressive tumor. Complete surgical removal and adjuvant radiotherapy are crucial for good locoregional control in these rare endothelial neoplasms.
Area of Science:
- Oncology
- Pathology
- Neurosurgery
Background:
- Angiosarcomas are rare, high-grade endothelial tumors originating from blood vessels.
- Primary skull neoplasms are uncommon, with malignant types being even rarer.
- This case highlights the rarity of primary angiosarcoma of the skull.
Observation:
- A 32-year-old female presented with a progressively enlarging parieto-occipital swelling.
- Radiology revealed a destructive calvarial lesion with intracranial extension.
- Histopathology confirmed angiosarcoma, with immunohistochemistry positive for CD34, CD31, and factor VIII-related antigen.
Findings:
- Complete surgical excision of the skull angiosarcoma was achieved.
- Adjuvant radiotherapy was administered to the tumor bed post-surgery.
- The patient demonstrated good locoregional control at 2-year follow-up.
Implications:
- Primary skull angiosarcoma is exceptionally rare, with fewer than 20 reported cases globally.
- Multimodal treatment involving complete surgical excision and adjuvant radiotherapy is recommended.
- Long-term surveillance with regular imaging is essential to monitor for recurrence.
