Platelet-derived growth factor receptor-β in Gorham’s disease
Identifieur interne : 007A65 ( Main/Merge ); précédent : 007A64; suivant : 007A66Platelet-derived growth factor receptor-β in Gorham’s disease
Auteurs : Jeroen Hagendoorn ; Timothy P. Padera ; Torunn I. Yock ; G Petur Nielsen ; Emmanuelle Di Tomaso ; Dan G. Duda ; Thomas F. Delaney ; Henning A. Gaissert ; Jennifer Pearce ; Andrew E. Rosenberg ; Rakesh K. Jain ; David H. EbbSource :
- Nature clinical practice. Oncology [ 1743-4254 ] ; 2006.
Abstract
A 17-year-old male presented with pain in his lower-left chest. He had no significant medical history and was previously in good health. He had a fractured ninth left anterior rib and the tenth, eleventh and twelfth ribs were absent, which was thought to be a congenital anomaly. Several months later, he presented again with back pain, an enlarging mass in the lower-left chest wall, erosion of the lateral pedicles of the lower thoracic vertebrae and pleural effusion.
Physical examination, chest X-ray, MRI of the spine, incisional biopsy, serial CT imaging of the hemithorax, immunohistochemistry, flow cytometry, and enzyme-linked immunosorbent assays.
Gorham’s lymphangiomatosis with expression of platelet-derived growth factor receptor-β and elevated circulating platelet-derived growth factor-BB.
Spine stabilization, thalidomide, celecoxib, interferon-α2b, pamidronate, zoledronate, thoracotomy, pleurectomy, talc pleurodesis, and imatinib mesylate.
Url:
DOI: 10.1038/ncponc0660
PubMed: 17139320
PubMed Central: 2693369
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PMC:2693369Le document en format XML
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<author><name sortKey="Yock, Torunn I" sort="Yock, Torunn I" uniqKey="Yock T" first="Torunn I" last="Yock">Torunn I. Yock</name>
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<author><name sortKey="Nielsen, G Petur" sort="Nielsen, G Petur" uniqKey="Nielsen G" first="G Petur" last="Nielsen">G Petur Nielsen</name>
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<author><name sortKey="Di Tomaso, Emmanuelle" sort="Di Tomaso, Emmanuelle" uniqKey="Di Tomaso E" first="Emmanuelle" last="Di Tomaso">Emmanuelle Di Tomaso</name>
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<author><name sortKey="Padera, Timothy P" sort="Padera, Timothy P" uniqKey="Padera T" first="Timothy P" last="Padera">Timothy P. Padera</name>
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<author><name sortKey="Yock, Torunn I" sort="Yock, Torunn I" uniqKey="Yock T" first="Torunn I" last="Yock">Torunn I. Yock</name>
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<author><name sortKey="Nielsen, G Petur" sort="Nielsen, G Petur" uniqKey="Nielsen G" first="G Petur" last="Nielsen">G Petur Nielsen</name>
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<author><name sortKey="Di Tomaso, Emmanuelle" sort="Di Tomaso, Emmanuelle" uniqKey="Di Tomaso E" first="Emmanuelle" last="Di Tomaso">Emmanuelle Di Tomaso</name>
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<author><name sortKey="Duda, Dan G" sort="Duda, Dan G" uniqKey="Duda D" first="Dan G" last="Duda">Dan G. Duda</name>
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<author><name sortKey="Delaney, Thomas F" sort="Delaney, Thomas F" uniqKey="Delaney T" first="Thomas F" last="Delaney">Thomas F. Delaney</name>
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<author><name sortKey="Gaissert, Henning A" sort="Gaissert, Henning A" uniqKey="Gaissert H" first="Henning A" last="Gaissert">Henning A. Gaissert</name>
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<author><name sortKey="Pearce, Jennifer" sort="Pearce, Jennifer" uniqKey="Pearce J" first="Jennifer" last="Pearce">Jennifer Pearce</name>
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<author><name sortKey="Rosenberg, Andrew E" sort="Rosenberg, Andrew E" uniqKey="Rosenberg A" first="Andrew E" last="Rosenberg">Andrew E. Rosenberg</name>
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<author><name sortKey="Jain, Rakesh K" sort="Jain, Rakesh K" uniqKey="Jain R" first="Rakesh K" last="Jain">Rakesh K. Jain</name>
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<series><title level="j">Nature clinical practice. Oncology</title>
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<front><div type="abstract" xml:lang="en"><title>SUMMARY</title>
<sec id="S1"><title>Background</title>
<p id="P2">A 17-year-old male presented with pain in his lower-left chest. He had no significant medical history and was previously in good health. He had a fractured ninth left anterior rib and the tenth, eleventh and twelfth ribs were absent, which was thought to be a congenital anomaly. Several months later, he presented again with back pain, an enlarging mass in the lower-left chest wall, erosion of the lateral pedicles of the lower thoracic vertebrae and pleural effusion.</p>
</sec>
<sec id="S2"><title>Investigations</title>
<p id="P3">Physical examination, chest X-ray, MRI of the spine, incisional biopsy, serial CT imaging of the hemithorax, immunohistochemistry, flow cytometry, and enzyme-linked immunosorbent assays.</p>
</sec>
<sec id="S3"><title>Diagnosis</title>
<p id="P4">Gorham’s lymphangiomatosis with expression of platelet-derived growth factor receptor-β and elevated circulating platelet-derived growth factor-BB.</p>
</sec>
<sec id="S4"><title>Management</title>
<p id="P5">Spine stabilization, thalidomide, celecoxib, interferon-α2b, pamidronate, zoledronate, thoracotomy, pleurectomy, talc pleurodesis, and imatinib mesylate.</p>
</sec>
</div>
</front>
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