Initial Presentation of Primary Splenic Angiosarcoma with Multiple Bone Lesions

Background

Primary splenic angiosarcoma is an exceptionally rare and aggressive malignant vascular neoplasm, with a low incidence rate. Its clinical manifestations are often nonspecific, leading to delayed diagnosis, especially when bone lesions are the first sign.

Data Highlights

No numerical data or trial data provided in the article.

Key Findings

• PSA can present with osseous metastasis, complicating diagnosis.
• Histological differentiation does not always correlate with clinical behavior; well-differentiated lesions may exhibit aggressive progression.
• Imaging findings may mimic benign vascular tumors, such as hemangiomas.
• Biopsy of vascular tumors carries a significant risk of hemorrhage, necessitating careful pre-procedural assessment.
• Rapid progression of bone lesions can occur despite high-grade histological appearance.

Clinical Implications

Clinicians should consider PSA in patients presenting with splenic and skeletal lesions.

Conclusion

This case emphasizes the need for integration of clinical, imaging, and pathological findings for accurate diagnosis and management of primary splenic angiosarcoma.

Related Resources & Content

1. NCCN Guidelines® Insights: Soft Tissue Sarcoma, Version 1.2025 - PubMed
2. Primary splenic angiosarcoma: a case series of a rare oncological entity and diagnostic challenge | Acta Oncologica
3. Journal of Gastrointestinal Surgery — Hepatic Osteosarcoma: A Rare Primary Tumor with Successful Surgical Intervention
4. Frontiers in Oncology — A case report of splenic malignancy originating from the liver
5. Clinical Research in Cardiology — Combination of Cadonilimab and Anlotinib Shows Promise in Treating Rare Cardiac Angiosarcoma with Multiple Metastases: A Case Study and Review of Existing Literature
6. Frontiers in Oncology — Isolated splenic littoral cell angioma in a child: a case report
7. NCCN Guidelines® Insights: Soft Tissue Sarcoma, Version 1.2025 - PubMed
8. Primary splenic angiosarcoma: a case series of a rare oncological entity and diagnostic challenge | Acta Oncologica
9. Metastatic splenic angiosarcoma presenting with anemia and bone marrow fibrosis mimicking primary myelofibrosis: A case report and literature review - PMC
10. Phase II Trial of Weekly Paclitaxel for Unresectable Angiosarcoma: The ANGIOTAX Study | Journal of Clinical Oncology
11. Paclitaxel Given Once Per Week With or Without Bevacizumab in Patients With Advanced Angiosarcoma: A Randomized Phase II Trial | Journal of Clinical Oncology
12. Angiosarcoma: Outcomes of systemic therapy for patients with metastatic disease - PMC
13. Angiosarcoma: Role of Immunotherapy - PubMed
14. Clinical activity of checkpoint inhibitors in angiosarcoma: A retrospective cohort study - PubMed
15. Efficacy of immune checkpoint inhibitors in the treatment of soft tissue sarcoma: A systematic review and meta-analysis of clinical trials - ScienceDirect
16. Clinical recommendations for treatment of localized angiosarcoma: A consensus paper by the Italian Sarcoma Group - PubMed