Perivascular epithelioid cell tumour

rdf:langString Perivascular epithelioid cell tumour

rdf:langString PEComa

rdf:langString PEComa

rdf:langString D054973

rdf:langString Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. H&E stain.

rdf:langString Perivascular epithelioid cell tumour, also known as PEComa or PEC tumour, is a family of mesenchymal tumours consisting of perivascular epithelioid cells (PECs). These are rare tumours that can occur in any part of the human body. The cell type from which these tumours originate remains unknown. Normally, no perivascular epitheloid cells exist; the name refers to the characteristics of the tumour when examined under the microscope. Establishing the malignant potential of these tumours remains challenging although criteria have been suggested; some PEComas display malignant features whereas others can cautiously be labeled as having 'uncertain malignant potential'. The most common tumours in the PEComa family are renal angiomyolipoma and pulmonary lymphangioleiomyomatosis, both of which are more common in patients with tuberous sclerosis complex. The genes responsible for this multi-system genetic disease have also been implicated in other PEComas. Many PEComa types shows a female predominance in the sex ratio.

rdf:langString orthoped

rdf:langString orthoped

rdf:langString 377