Synonyms

• Hemangiosarcoma
• endothelial sarcoma
• angioendothelioma

Category

Soft tissue

Behaviour

Malignant

Grade

High

Gender

Both equally

Tissue of Origin

Vascular

Epidemiology

• High-grade Vascular malignancy
• Cutaneous: post-radiation (10+ years), chronic lymphoedema
• Deep: primary Vascular sarcoma
• Elderly for cutaneous; 4th-5th decade for deep
• 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)
• 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 primary site: local staging and extent
• CT chest/abdomen/pelvis: metastatic staging
• PET-CT: systemic assessment

Workup - Biopsy

Core needle: confirm diagnosis and High-grade features

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

• Post-operative review at 6 weeks
• Year 1: Post-operative visit within first 6 weeks; 3–4 monthly clinical examination and CXR; image prosthesis at 6 months and 1 year
• Year 2: 3–4 monthly clinical examination and CXR; image prosthesis annually
• Years 3–4: 6-monthly clinical examination and CXR; image prosthesis annually
• Years 5–10: Annual clinical examination and CXR; image prosthesis annually
• Discharge at 10 years after surgery
• Radiotherapy patients: monitor irradiated field for late effects at each review
• Angiosarcoma specifically listed by LSESN as a histotype NOT to discharge at 10 years due to late recurrence