Synonyms

• Post-mastectomy angiosarcoma
• lymphangiosarcoma

Category

Soft tissue

Behaviour

Malignant

Grade

High

Gender

Female

Tissue of Origin

Vascular

Epidemiology

• Rare angiosarcoma arising in chronic lymphoedematous extremity
• Classically described after radical mastectomy and lymph node dissection
• Incidence 0.07–0.45% of post-mastectomy patients
• Median onset 10 years post-lymphoedema onset
• Also reported in Lower limb lymphoedema

Clinical Features

• Violaceous or bruise-like skin nodules in oedematous extremity
• Satellite lesions and multifocality characteristic
• Rapid progression and ulceration
• Painless initially, becoming painful as disease advances

Location

• Upper extremity (arm, forearm) in post-mastectomy setting
• Lower extremity in Lower limb lymphoedema
• Skin and subcutaneous tissue of affected limb

Imaging

• Multifocal skin lesions on clinical examination
• MRI shows ill-defined enhancing subcutaneous masses
• PET-CT for staging - often multifocal FDG-avid disease
• Chest CT for pulmonary metastases

Pathology

• Angiosarcoma histology - anastomosing Vascular channels lined by atypical endothelial cells
• CD31, CD34, ERG positive
• MYC amplification characteristic
• Multifocal involvement of skin and subcutis

Genetics

• MYC amplification present in 80% (distinguishes from atypical Vascular lesion)
• KDR mutations
• Complex karyotype

Treatment

• Amputation often required for disease control
• Wide local excision rarely curative due to multifocality
• Paclitaxel-based chemotherapy
• Radiotherapy for palliation

Prognosis

• Very poor: median survival 19 months from diagnosis
• 5-year survival <10%
• Haematogenous metastasis to lungs predominant pattern
• Early diagnosis improves outcomes marginally

Key Points

• Pathognomonic association with chronic lymphoedema
• MYC FISH useful to confirm diagnosis and distinguish from Benign Vascular proliferations
• Multidisciplinary management involving oncology, plastic surgery, and lymphoedema specialists essential
• Prevention: optimal lymphoedema management post-mastectomy

Workup - Blood Tests

FBC, U&E, LFTs - baseline and pre-chemotherapy

Workup - Local Imaging

• MRI affected limb - ill-defined enhancing multifocal subcutaneous masses
• CT chest/abdomen/pelvis - staging; assess for pulmonary metastases
• PET-CT - multifocal FDG-avid disease

Workup - Biopsy

• Core needle biopsy - confirm angiosarcoma diagnosis
• IHC: CD31+, CD34+, ERG+
• MYC FISH - present in 80%; distinguishes from Benign Vascular proliferation

Workup - Staging

• CT chest/abdomen/pelvis - metastatic disease (lungs most common)
• PET-CT - systemic staging

Workup - Other

• MDT including oncology, Vascular surgery, lymphoedema specialist
• Paclitaxel-based chemotherapy standard
• Optimal lymphoedema management essential for prevention

Follow-up Summary

• Years 1–2: 3–4 monthly clinical review + CXR; PET-CT or CT chest at 3–4 monthly intervals (multifocal disease common)
• MRI of affected limb at each staging point to assess local extent
• Years 3–5: 6-monthly clinical review + CXR; CT chest 6-monthly
• Years 6–10: Annual clinical review + CXR; CT chest annually
• Lymphoedema management: ongoing specialist lymphoedema clinic referral essential
• Angiosarcoma specifically listed by LSESN as a histotype NOT to discharge at 10 years due to late recurrence