PEComa in a Young Patient with Known Li-Fraumeni Syndrome.

摘要: Perivascular epithelioid cells neoplasms (PEComas) constitute a family of rare tumours which have been reported virtually in all anatomic sites. The histological clarification the malignant potential these is still problematic despite proposed risk stratification systems. Li-Fraumeni syndrome (LFS) caused by germline mutation TP53 tumour suppressor gene. It but well-characterized cancer predisposition leading to development variety different types. To best our knowledge, an association between this and PEComas has not previously documented. A 24-year-old lady with known LFS presented two uncertain-in-nature lesions, one within right part liver upper pole kidney. patient underwent uncomplicated open simultaneous partial nephrectomy resection segment 7 liver. morphological immunohistochemical features both lesions were angiomyolipoma (PEComa). Although obvious scenario was that lesion metastasis from renal lesion, assessment their according existing systems rather favour synchronous primary PEComas, pointing out problematic.