Long-lasting stable disease with mTOR inhibitor treatment in a patient with a perivascular epithelioid cell tumor: A case report and literature review
Identifieur interne : 000E90 ( Main/Exploration ); précédent : 000E89; suivant : 000E91Long-lasting stable disease with mTOR inhibitor treatment in a patient with a perivascular epithelioid cell tumor: A case report and literature review
Auteurs : Ezequiel Flechter [Israël] ; Yaniv Zohar [Israël] ; Ludmila Guralnik [Israël] ; Maria Passhak [Israël] ; Gil Bar Sela [Israël]Source :
- Oncology Letters [ 1792-1074 ] ; 2016.
Abstract
Perivascular epithelioid cell tumor (PEComa) of the small intestine is extremely rare, and there is no established treatment at the present time. In 10% of patients with PEComas, genetic alterations of tuberous sclerosis complex have been reported. These genetic alterations activate mechanistic target of rapamycin (mTOR) in AMP-activated protein kinase and Ras/mitogen-activated protein kinase pathways, resulting in high mTOR activity. Since 2007, several cases of treatment with mTOR inhibitors in advanced PEComa have been reported. The current study presents the case of a patient with small bowel PEComa that metastasized to the brain and lungs. Following resection of the brain metastasis, the patient was treated with everolimus, a mTOR inhibitor, resulting in improvement if the patient's quality of life and a long period of stable disease. In conclusion, the use of mTOR inhibitors as a first-line treatment option in advanced PEComa patients appears to be reasonable, according to the increasing evidence from data observed from reported cases with this rare malignancy.
Url:
DOI: 10.3892/ol.2016.5231
PubMed: 28105182
PubMed Central: 5228394
Affiliations:
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In 10% of patients with PEComas, genetic alterations of tuberous sclerosis complex have been reported. These genetic alterations activate mechanistic target of rapamycin (mTOR) in AMP-activated protein kinase and Ras/mitogen-activated protein kinase pathways, resulting in high mTOR activity. Since 2007, several cases of treatment with mTOR inhibitors in advanced PEComa have been reported. The current study presents the case of a patient with small bowel PEComa that metastasized to the brain and lungs. Following resection of the brain metastasis, the patient was treated with everolimus, a mTOR inhibitor, resulting in improvement if the patient's quality of life and a long period of stable disease. In conclusion, the use of mTOR inhibitors as a first-line treatment option in advanced PEComa patients appears to be reasonable, according to the increasing evidence from data observed from reported cases with this rare malignancy.</p></div></front></TEI><affiliations><list><country><li>Israël</li></country></list><tree><country name="Israël"><noRegion><name sortKey="Flechter, Ezequiel" sort="Flechter, Ezequiel" uniqKey="Flechter E" first="Ezequiel" last="Flechter">Ezequiel Flechter</name></noRegion><name sortKey="Guralnik, Ludmila" sort="Guralnik, Ludmila" uniqKey="Guralnik L" first="Ludmila" last="Guralnik">Ludmila Guralnik</name><name sortKey="Passhak, Maria" sort="Passhak, Maria" uniqKey="Passhak M" first="Maria" last="Passhak">Maria Passhak</name><name sortKey="Sela, Gil Bar" sort="Sela, Gil Bar" uniqKey="Sela G" first="Gil Bar" last="Sela">Gil Bar Sela</name><name sortKey="Zohar, Yaniv" sort="Zohar, Yaniv" uniqKey="Zohar Y" first="Yaniv" last="Zohar">Yaniv Zohar</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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