Primary splenic angiosarcoma: a rare entity often associated with rupture and hemoperitoneum

Area of Science:

• Oncology
• Pathology
• Vascular Biology

Background:

• Primary splenic angiosarcoma (PSA) is a rare vascular neoplasm.
• PSA is characterized by aggressive behavior and poor prognosis.
• Clinical presentation is often non-specific, mimicking other conditions.

Observation:

• An 80-year-old woman presented with weight loss, fatigue, anemia, and massive splenomegaly.
• Initial diagnosis was uncertain, considered a myelodysplastic disorder.
• The patient experienced spleen rupture, confirmed by imaging and treated with splenectomy.

Findings:

• Histopathology confirmed primary splenic angiosarcoma.
• Staging revealed liver and peritoneal metastases two months post-surgery.
• The patient succumbed to the disease six months after diagnosis.

Implications:

• Splenic rupture can be a presenting sign of primary splenic angiosarcoma.
• Early diagnosis and prompt management are crucial for rare vascular tumors.
• Aggressive nature of PSA necessitates advanced oncological care and further research.