IntermediateSoft tissue
PEComa
Synonyms: perivascular epithelioid cell tumour, PEC-derived tumour
Tumour of perivascular epithelioid cells
Quick Facts
Behaviour
Intermediate
Category
Soft tissue
Grade
Low
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
Medical disclaimer
The content on Sarcopedia is for educational and informational purposes only and does not constitute medical advice. It is not intended to be a substitute for professional medical diagnosis or treatment. Always consult with a qualified physician regarding any health concerns or before starting any new treatment. Reliance on any information provided on this site is solely at your own risk.