Synonyms

• RFHE
• rete-like haemangioendothelioma

Category

Soft tissue

Behaviour

Intermediate

Grade

Low

Gender

Both equally

Tissue of Origin

Vascular

Epidemiology

• Rare intermediate-grade Vascular tumour
• Peak incidence in children and young adults
• Acral distribution common

Clinical Features

• Painless or painful red nodules
• Often multifocal
• Slow-growing but locally aggressive

Location

• Acral: finger, toe, palm, sole
• Sometimes proximal sites

Imaging

• MRI: well-defined lesion with Variable enhancement
• Multiple lesions common

Pathology

• Retiform (rete-like) Vascular channels
• Irregular anastomosing pattern
• Low mitotic rate but infiltrative growth

Genetics

FOSB rearrangement reported in some

Treatment

• Wide surgical excision of all lesions
• Observation if stable

Prognosis

• Intermediate - High recurrence risk
• Metastasis rare but can occur

Key Points

• Acral presentation with multifocal lesions typical
• Locally aggressive despite Benign appearance
• Wide excision essential

Workup - Blood Tests

FBC, U&E, LFTs - baseline

Workup - Local Imaging

• MRI primary site - local staging
• Full body imaging - assess for other lesions

Workup - Biopsy

Core needle biopsy - confirm diagnosis

Workup - Staging

No metastatic staging unless High-risk features