Primary splenic angiosarcoma diagnosed after splenectomy for spontaneous rupture

Area of Science:

• Oncology
• Pathology
• Radiology

Background:

• Primary splenic angiosarcoma is a rare malignancy with an unfavorable prognosis and unknown pathogenesis.
• It frequently manifests as splenic rupture, complicating diagnosis and treatment.
• Early surgical intervention, particularly splenectomy, is crucial for improving patient outcomes.

Purpose of the Study:

• To highlight the diagnostic and therapeutic significance of splenectomy in primary splenic angiosarcoma.
• To present a case study of a patient with splenic angiosarcoma, emphasizing clinical presentation and management.

Main Methods:

• Case report of a 65-year-old female presenting with abdominal symptoms and diagnosed with splenic angiosarcoma post-splenectomy.
• Diagnostic workup included imaging (18F-FDG-PET-CT) to assess for metastasis.
• Treatment involved surgical resection followed by chemotherapy (paclitaxel) for metastatic disease.

Main Results:

• The patient was diagnosed with splenic angiosarcoma confirmed by histopathological examination of the spleen.
• Initial staging via 18F-FDG-PET-CT revealed no distant metastasis.
• Metastasis to the liver and bone occurred five months post-surgery, necessitating chemotherapy.

Conclusions:

• Splenic angiosarcoma is a distinct entity within splenic parenchymal lesions, definitively diagnosed through splenectomy.
• Prompt surgical management before rupture significantly impacts life expectancy.
• Multimodal treatment, including chemotherapy, may be required for advanced or metastatic disease.