![](/images/graphics-bg.png)
PEComa in a Young Patient with Known Li-Fraumeni Syndrome
Joint Authors
Famularo, Simone
Khan, Aamir Z.
Kyriakos, Neofytou
Source
Issue
Vol. 2015, Issue 2015 (31 Dec. 2015), pp.1-4, 4 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2015-03-04
Country of Publication
Egypt
No. of Pages
4
Main Subjects
Abstract EN
Perivascular epithelioid cells neoplasms (PEComas) constitute a family of rare tumours which have been reported virtually in all anatomic sites.
The histological clarification of the malignant potential of these tumours is still problematic despite the proposed risk stratification systems.
Li-Fraumeni syndrome (LFS) is caused by a germline mutation in the TP53 tumour suppressor gene.
It is a rare but well-characterized cancer predisposition syndrome leading to the development of a variety of different tumour types.
To the best of our knowledge, an association between this syndrome and PEComas has not been previously documented.
A 24-year-old lady with known LFS presented with two uncertain-in-nature lesions, one within the right part of the liver and one within the upper pole of the right kidney.
The patient underwent an uncomplicated open simultaneous right partial nephrectomy and resection of segment 7 of the liver.
The morphological and immunohistochemical features of both lesions were of epithelioid angiomyolipoma (PEComa).
Although the obvious scenario was that the liver lesion was a metastasis from the renal lesion, the assessment of their malignant potential according to the existing risk stratification systems was rather in favour of two synchronous primary PEComas, pointing out that the histological assessment of malignant potential of PEComas is still problematic.
American Psychological Association (APA)
Kyriakos, Neofytou& Famularo, Simone& Khan, Aamir Z.. 2015. PEComa in a Young Patient with Known Li-Fraumeni Syndrome. Case Reports in Medicine،Vol. 2015, no. 2015, pp.1-4.
https://search.emarefa.net/detail/BIM-1058929
Modern Language Association (MLA)
Kyriakos, Neofytou…[et al.]. PEComa in a Young Patient with Known Li-Fraumeni Syndrome. Case Reports in Medicine No. 2015 (2015), pp.1-4.
https://search.emarefa.net/detail/BIM-1058929
American Medical Association (AMA)
Kyriakos, Neofytou& Famularo, Simone& Khan, Aamir Z.. PEComa in a Young Patient with Known Li-Fraumeni Syndrome. Case Reports in Medicine. 2015. Vol. 2015, no. 2015, pp.1-4.
https://search.emarefa.net/detail/BIM-1058929
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1058929