Fact-sheet: Splenic angiosarcoma
Updated on 04/23/2019 at 9:53 AM
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Definition
Angiosarcoma is a rare tumor arising from the endothelial cells of blood vessels. It is usually located in the skin and soft tissues.
Splenic angiosarcoma:
- The spleen accounts for only 4% of angiosarcoma locations.
- This primary malignant tumor carries a poor prognosis.
- Mean age at diagnosis: 60 years.
- Male predominance.
- Unknown pathogenesis.
- Predisposing factors: ionizing radiation? Chemotherapy?
Clinical features
- Marked general deterioration, weight loss, painful splenomegaly.
- Palpable abdominal mass.
- Hemorrhagic shock in case of spontaneous rupture.
- Exceptional reported case of hematemesis (due to invasion of the greater gastric curvature).
Laboratory findings
No specific sign.
CT
- One or more focal nodular lesions with irregular margins.
- Variable density of the lesions, which are sometimes spontaneously hyperdense (intralesional hemorrhagic changes).
- Variable enhancement after injection, sometimes pseudo-hemangiomatous.
- Subcapsular hematoma, rupture.
- Search for hepatic or pulmonary metastases.
MRI
Lesions of variable signal intensity on T1 and T2.
Gadolinium injection results in variable enhancement, sometimes intense, pseudo-angiomatous: poorly specific.
Management
- Surgical resection - splenectomy (debated given the tumor's aggressiveness and high metastatic potential).
- example of prolonged survival after chemoradiotherapy.
- Symptomatic treatment.
- Prognosis:
Very poor.
Diffuse, multiple, early metastases.
Overall survival: 20% at 6 months, nearly zero after 1 year.
Differential diagnosis
Metastases (in this case the primary tumor is known).
Splenic hemangioma, hamartoma, and lymphangioma do not cause deterioration of general condition.