Epithelioid Angiosarcoma
Synonyms: Epithelioid haemangioendothelioma (Malignant spectrum), epithelioid variant of angiosarcoma
Cytokeratin positivity is a diagnostic pitfall - may be confused with carcinoma or mesothelioma
Quick Facts
Behaviour
Malignant
Category
Soft tissue
Grade
High
Synonyms
- Epithelioid haemangioendothelioma (Malignant spectrum)
- epithelioid variant of angiosarcoma
Category
Soft tissue
Behaviour
Malignant
Grade
High
Gender
Male
Tissue of Origin
Vascular
Epidemiology
- Rare variant of angiosarcoma with epithelioid morphology
- Wide age range, predominantly adults
- Slight Male predominance
- Can arise de novo, post-irradiation, or in chronic lymphoedema
Clinical Features
- Haemorrhagic skin lesion or deep soft tissue mass
- May mimic carcinoma clinically and histologically
- Rapid progression
- Ulceration and satellite nodules common in cutaneous form
Location
- Skin (scalp, face, extremities)
- Deep soft tissue
- Viscera (liver, lung)
- Bone
Imaging
- Heterogeneous poorly defined mass
- Haemorrhagic areas on MRI (T1 hyperintensity)
- Intense enhancement on CT
- Multiple lesions may be present
Pathology
- Sheets of epithelioid cells with abundant eosinophilic cytoplasm
- Primitive Vascular channel formation (intracytoplasmic lumina)
- CD31, CD34, ERG, factor VIII positive
- Cytokeratin may be positive - mimics carcinoma or mesothelioma
Genetics
- MYC amplification in radiation-associated cases
- CIC rearrangements in subset
- Complex karyotype
Treatment
- Wide surgical resection when feasible
- Paclitaxel-based chemotherapy for advanced disease
- Radiotherapy for local control
- Anti-angiogenic agents (sorafenib, pazopanib)
Prognosis
- Very poor - similar to conventional angiosarcoma
- 5-year survival <30%
- Rapid metastatic dissemination
- Cytokeratin positivity can lead to misdiagnosis and delayed appropriate treatment
Key Points
- Cytokeratin positivity is a diagnostic pitfall - may be confused with carcinoma or mesothelioma
- CD31 and ERG are the most sensitive Vascular markers
- MYC amplification confirms radiation-association
- Management as per conventional angiosarcoma
Workup - Blood Tests
- FBC - anaemia common in visceral angiosarcoma
- LFTs - hepatic involvement
- Coagulation screen - consumptive coagulopathy
- LDH - elevated; prognostic marker
Workup - Local Imaging
- Plain radiograph
- MRI + conrast primary site
Workup - Biopsy
- Core needle biopsy - mandatory; exclude carcinoma before treatment
- IHC: CD31+, CD34+, ERG+ (most sensitive Vascular markers); AE1/AE3 may be positive (pitfall)
- MYC FISH - amplification confirms radiation-associated angiosarcoma
- CK7/CK20 - to further distinguish from metastatic carcinoma
Workup - Staging
- CT chest/abdomen/pelvis - metastases at presentation in majority
- Bone scan or PET-CT - Bone metastases
Workup - Other
- MDT at specialist sarcoma centre
- Paclitaxel-based chemotherapy for cutaneous angiosarcoma; anthracycline/ifosfamide for deep/visceral
Follow-up Summary
- 1
Year 1
Post-operative visit within first 6 weeks; 3–4 monthly clinical examination and CXR; image prosthesis at 6 months and 1 year
- 2
Year 2
3–4 monthly clinical examination and CXR; image prosthesis annually
- 3
Years 3–4
6-monthly clinical examination and CXR; image prosthesis annually
- 4
Years 5–10
Annual clinical examination and CXR; image prosthesis annually
- 5
Discharge at 10 years after surgery
Medical disclaimer
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