Synonyms

• PeriVascular epithelioid cell tumour
• PEC-derived tumour

Category

Soft tissue

Behaviour

Intermediate

Grade

Low

Gender

Female

Tissue of Origin

Other

Epidemiology

• Tumour derived from periVascular epithelioid cells
• Peak incidence in 3rd-4th decades
• Female predominance, especially angiomyolipoma

Clinical Features

• Often asymptomatic and incidental
• Angiomyolipoma: can present with haemorrhage
• Lymphangioleiomyomatosis (LAM): pulmonary symptoms

Location

• Kidney (angiomyolipoma most common)
• Liver
• Lung (LAM form)

Imaging

• CT/MRI: fat-containing lesion (angiomyolipoma)
• Lung: diffuse cystic changes (LAM)

Pathology

• PeriVascular epithelioid cells admixed with smooth muscle and fat
• HMB-45+, melan-A+, SMA+

Genetics

• TSC1/TSC2 mutations associated
• Spontaneous mTOR hyperactivation

Treatment

• Observation for asymptomatic lesions
• Intervention for haemorrhage or symptoms
• mTOR inhibitors (sirolimus) for progressive LAM

Prognosis

• Generally indolent
• LAM progressive in some patients

Key Points

• Tumour of periVascular epithelioid cells
• Fat-containing lesion in kidney characteristic
• TSC association in some cases

Workup - Blood Tests

No specific blood tests required

Workup - Local Imaging

CT or MRI: characterises fat content and Vascular involvement

Workup - Biopsy

Rarely required if imaging typical

Workup - Staging

No staging required