Synonyms

Hemangiopericytoma with Malignant features

Category

Soft tissue

Behaviour

Malignant

Grade

High

Gender

Both equally

Tissue of Origin

Vascular

Epidemiology

• Rare Malignant Vascular tumour
• Peak incidence in 5th-6th decades
• Predilection for Lower extremity

Clinical Features

• Rapidly growing painful soft tissue mass
• Often presents with advanced disease

Location

• Lower extremity most common
• Soft tissue

Imaging

• MRI: heterogeneous mass with Variable enhancement
• CT: assess for metastases

Pathology

• Malignant hemangiopericytoma
• Vascular channels with prominent pericytes
• High mitotic rate and necrosis

Genetics

AGGF1 mutations may be present

Treatment

• Wide surgical excision
• Chemotherapy for metastatic disease

Prognosis

Poor prognosis with high metastatic potential

Key Points

• Rare Malignant variant of hemangiopericytoma
• High-grade malignancy with aggressive course

Workup - Blood Tests

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

Workup - Local Imaging

MRI with contrast

Workup - Biopsy

• Core needle biopsy - confirm diagnosis
• IHC: CD34+, STAT6+

Workup - Staging

CT chest/abdomen/pelvis

Workup - Other

• MDT at soft tissue sarcoma centre
• Aggressive behaviour requires multimodal therapy