Stewart-Treves Syndrome
Synonyms: Post-mastectomy angiosarcoma, lymphangiosarcoma
Pathognomonic association with chronic lymphoedema
Quick Facts
Behaviour
Malignant
Category
Soft tissue
Grade
High
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
- 1
Years 1–2
3–4 monthly clinical review + CXR; PET-CT or CT chest at 3–4 monthly intervals (multifocal disease common)
- 2
MRI of affected limb at each staging point to assess local extent
- 3
Years 3–5
6-monthly clinical review + CXR; CT chest 6-monthly
- 4
Years 6–10
Annual clinical review + CXR; CT chest annually
- 5
Lymphoedema management
ongoing specialist lymphoedema clinic referral essential
- 6
Angiosarcoma specifically listed by LSESN as a histotype NOT to discharge at 10 years due to late recurrence
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