Angiosarcoma
Synonyms: Hemangiosarcoma, endothelial sarcoma, angioendothelioma
Rare aggressive Vascular malignancy
Quick Facts
Behaviour
Malignant
Category
Soft tissue
Grade
High
Synonyms
- Hemangiosarcoma
- endothelial sarcoma
- angioendothelioma
Category
Soft tissue
Behaviour
Malignant
Grade
High
Gender
M = F
Tissue of Origin
Vascular
Epidemiology
- High-grade vascular malignancy - 2-3% of all soft tissue sarcoma
- Cutaneous: post-radiation (10+ years), chronic lymphoedema
- Deep: primary vascular sarcoma
- Poor prognosis with early metastases
Clinical Features
- Rapidly enlarging mass
- Cutaneous: bruising, bleeding, haemorrhage
- Pain
- Constitutional symptoms if advanced
Location
- Skin/scalp (cutaneous angiosarcoma)
- Soft tissues: extremities, trunk (deep), breast
- Liver, spleen, retroperitoneum
Imaging
- MRI: heterogeneous T1 and T2 signal
- Hemorrhage and necrosis common
- Poorly defined infiltrative margins
Pathology
- Proliferating endothelial cells lining Vascular spaces
- High mitotic rate with atypia
- Necrosis and hemorrhage present
- High-grade Malignant features
Genetics
- TP53, PIK3CA, NRAS, KDR alterations
- Complex genomic alterations
Treatment
- Wide surgical resection if feasible
- Chemotherapy: paclitaxel or doxorubicin first-line
- Radiotherapy: palliative
- Bevacizumab: possible adjuvant anti-VEGF therapy
Prognosis
- Very poor: 20% 5-year survival
- Often metastatic at presentation
- Median survival <2 years
Key Points
- Rare aggressive Vascular malignancy
- Early metastatic spread typical
- Poor prognosis despite treatment
- Paclitaxel shows benefit in angiosarcoma
Workup - Blood Tests
FBC, U&E, LFTs, LDH
Workup - Local Imaging
MRI with contrast of primary site: local staging and extent
Workup - Biopsy
Core needle biopsy
Workup - Staging
- CT chest/abdomen/pelvis: standard staging
- Consider PET-CT for systemic disease
Workup - Other
- Palliative MDT discussion often appropriate
- Consider bevacizumab in multimodal approach
Follow-up Summary
Soft Tissue Tumours - Localised Extremity - Intermediate and High Grade
Year 10+
Continue surveillance - late metastasis (lung, bone, brain) well recognised >10 years post-resection
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