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

• 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