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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Long-lasting stable disease with mTOR inhibitor treatment in a patient with a perivascular epithelioid cell tumor: A case report and literature review</title><author><name sortKey="Flechter, Ezequiel" sort="Flechter, Ezequiel" uniqKey="Flechter E" first="Ezequiel" last="Flechter">Ezequiel Flechter</name><affiliation><nlm:aff id="af1-ol-0-0-5231">Division of Oncology, Rambam Health Care Campus and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 31096, Israel</nlm:aff></affiliation></author><author><name sortKey="Zohar, Yaniv" sort="Zohar, Yaniv" uniqKey="Zohar Y" first="Yaniv" last="Zohar">Yaniv Zohar</name><affiliation><nlm:aff id="af2-ol-0-0-5231">Pathology Institute, Rambam Health Care Campus and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 31096, Israel</nlm:aff></affiliation></author><author><name sortKey="Guralnik, Ludmila" sort="Guralnik, Ludmila" uniqKey="Guralnik L" first="Ludmila" last="Guralnik">Ludmila Guralnik</name><affiliation><nlm:aff id="af3-ol-0-0-5231">Computed Tomography Unit, Radiology Department, Rambam Health Care Campus and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 31096, Israel</nlm:aff></affiliation></author><author><name sortKey="Passhak, Maria" sort="Passhak, Maria" uniqKey="Passhak M" first="Maria" last="Passhak">Maria Passhak</name><affiliation><nlm:aff id="af1-ol-0-0-5231">Division of Oncology, Rambam Health Care Campus and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 31096, Israel</nlm:aff></affiliation></author><author><name sortKey="Sela, Gil Bar" sort="Sela, Gil Bar" uniqKey="Sela G" first="Gil Bar" last="Sela">Gil Bar Sela</name><affiliation><nlm:aff id="af1-ol-0-0-5231">Division of Oncology, Rambam Health Care Campus and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 31096, Israel</nlm:aff></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">28105182</idno><idno type="pmc">5228394</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5228394</idno><idno type="RBID">PMC:5228394</idno><idno type="doi">10.3892/ol.2016.5231</idno><date when="2016">2016</date><idno type="wicri:Area/Pmc/Corpus">003117</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">003117</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Long-lasting stable disease with mTOR inhibitor treatment in a patient with a perivascular epithelioid cell tumor: A case report and literature review</title><author><name sortKey="Flechter, Ezequiel" sort="Flechter, Ezequiel" uniqKey="Flechter E" first="Ezequiel" last="Flechter">Ezequiel Flechter</name><affiliation><nlm:aff id="af1-ol-0-0-5231">Division of Oncology, Rambam Health Care Campus and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 31096, Israel</nlm:aff></affiliation></author><author><name sortKey="Zohar, Yaniv" sort="Zohar, Yaniv" uniqKey="Zohar Y" first="Yaniv" last="Zohar">Yaniv Zohar</name><affiliation><nlm:aff id="af2-ol-0-0-5231">Pathology Institute, Rambam Health Care Campus and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 31096, Israel</nlm:aff></affiliation></author><author><name sortKey="Guralnik, Ludmila" sort="Guralnik, Ludmila" uniqKey="Guralnik L" first="Ludmila" last="Guralnik">Ludmila Guralnik</name><affiliation><nlm:aff id="af3-ol-0-0-5231">Computed Tomography Unit, Radiology Department, Rambam Health Care Campus and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 31096, Israel</nlm:aff></affiliation></author><author><name sortKey="Passhak, Maria" sort="Passhak, Maria" uniqKey="Passhak M" first="Maria" last="Passhak">Maria Passhak</name><affiliation><nlm:aff id="af1-ol-0-0-5231">Division of Oncology, Rambam Health Care Campus and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 31096, Israel</nlm:aff></affiliation></author><author><name sortKey="Sela, Gil Bar" sort="Sela, Gil Bar" uniqKey="Sela G" first="Gil Bar" last="Sela">Gil Bar Sela</name><affiliation><nlm:aff id="af1-ol-0-0-5231">Division of Oncology, Rambam Health Care Campus and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 31096, Israel</nlm:aff></affiliation></author></analytic><series><title level="j">Oncology Letters</title><idno type="ISSN">1792-1074</idno><idno type="eISSN">1792-1082</idno><imprint><date when="2016">2016</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>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.</p></div></front></TEI><pmc article-type="research-article"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<front><journal-meta><journal-id journal-id-type="nlm-ta">Oncol Lett</journal-id><journal-id journal-id-type="iso-abbrev">Oncol Lett</journal-id><journal-id journal-id-type="publisher-id">OL</journal-id><journal-title-group><journal-title>Oncology Letters</journal-title></journal-title-group><issn pub-type="ppub">1792-1074</issn><issn pub-type="epub">1792-1082</issn><publisher><publisher-name>D.A. Spandidos</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">28105182</article-id><article-id pub-id-type="pmc">5228394</article-id><article-id pub-id-type="doi">10.3892/ol.2016.5231</article-id><article-id pub-id-type="publisher-id">OL-0-0-5231</article-id><article-categories><subj-group subj-group-type="heading"><subject>Articles</subject></subj-group></article-categories><title-group><article-title>Long-lasting stable disease with mTOR inhibitor treatment in a patient with a perivascular epithelioid cell tumor: A case report and literature review</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Flechter</surname><given-names>Ezequiel</given-names></name><xref ref-type="aff" rid="af1-ol-0-0-5231">1</xref></contrib><contrib contrib-type="author"><name><surname>Zohar</surname><given-names>Yaniv</given-names></name><xref ref-type="aff" rid="af2-ol-0-0-5231">2</xref></contrib><contrib contrib-type="author"><name><surname>Guralnik</surname><given-names>Ludmila</given-names></name><xref ref-type="aff" rid="af3-ol-0-0-5231">3</xref></contrib><contrib contrib-type="author"><name><surname>Passhak</surname><given-names>Maria</given-names></name><xref ref-type="aff" rid="af1-ol-0-0-5231">1</xref></contrib><contrib contrib-type="author"><name><surname>Sela</surname><given-names>Gil Bar</given-names></name><xref ref-type="aff" rid="af1-ol-0-0-5231">1</xref><xref rid="c1-ol-0-0-5231" ref-type="corresp"></xref></contrib></contrib-group><aff id="af1-ol-0-0-5231"><label>1</label>Division of Oncology, Rambam Health Care Campus and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 31096, Israel</aff><aff id="af2-ol-0-0-5231"><label>2</label>Pathology Institute, Rambam Health Care Campus and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 31096, Israel</aff><aff id="af3-ol-0-0-5231"><label>3</label>Computed Tomography Unit, Radiology Department, Rambam Health Care Campus and Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 31096, Israel</aff><author-notes><corresp id="c1-ol-0-0-5231"><italic>Correspondence to</italic>: Dr Gil Bar Sela, Division of Oncology, Rambam Health Care Campus and Faculty of Medicine, Technion-Israel Institute of Technology, PO Box 9602, Haifa 31096, Israel, E-mail: <email>g_barsela@rambam.health.gov.il</email></corresp></author-notes><pub-date pub-type="ppub"><month>12</month><year>2016</year></pub-date><pub-date pub-type="epub"><day>06</day><month>10</month><year>2016</year></pub-date><pub-date pub-type="pmc-release"><day>06</day><month>10</month><year>2016</year></pub-date><pmc-comment> PMC Release delay is 0 months and 0 days and was based on the
<volume>12</volume><issue>6</issue><fpage>4739</fpage><lpage>4743</lpage><history><date date-type="received"><day>17</day><month>3</month><year>2015</year></date><date date-type="accepted"><day>04</day><month>4</month><year>2016</year></date></history><permissions><copyright-statement>Copyright © 2016, Spandidos Publications</copyright-statement><copyright-year>2016</copyright-year></permissions><abstract><p>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.</p></abstract><kwd-group><kwd>everolimus</kwd><kwd>mTOR inhibitor</kwd><kwd>PEComa</kwd><kwd>literature review</kwd></kwd-group></article-meta></front></pmc></record>Pour manipuler ce document sous Unix (Dilib)
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