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Platelet-derived growth factor receptor-β in Gorham’s disease

Identifieur interne : 002813 ( Pmc/Corpus ); précédent : 002812; suivant : 002814

Platelet-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. Ebb

Source :

RBID : PMC:2693369

Abstract

SUMMARYBackground

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.

Investigations

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.

Diagnosis

Gorham’s lymphangiomatosis with expression of platelet-derived growth factor receptor-β and elevated circulating platelet-derived growth factor-BB.

Management

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

Links to Exploration step

PMC:2693369

Le document en format XML

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<name sortKey="Hagendoorn, Jeroen" sort="Hagendoorn, Jeroen" uniqKey="Hagendoorn J" first="Jeroen" last="Hagendoorn">Jeroen Hagendoorn</name>
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<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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<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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<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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<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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<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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<name sortKey="Pearce, Jennifer" sort="Pearce, Jennifer" uniqKey="Pearce J" first="Jennifer" last="Pearce">Jennifer Pearce</name>
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<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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<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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<article-title>Platelet-derived growth factor receptor-β in Gorham’s disease</article-title>
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<contrib contrib-type="author">
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<given-names>Jeroen</given-names>
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<given-names>Timothy P</given-names>
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<given-names>Torunn I</given-names>
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<name>
<surname>Nielsen</surname>
<given-names>G Petur</given-names>
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<surname>di Tomaso</surname>
<given-names>Emmanuelle</given-names>
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<surname>Duda</surname>
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<name>
<surname>Delaney</surname>
<given-names>Thomas F</given-names>
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<contrib contrib-type="author">
<name>
<surname>Gaissert</surname>
<given-names>Henning A</given-names>
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<contrib contrib-type="author">
<name>
<surname>Pearce</surname>
<given-names>Jennifer</given-names>
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<surname>Rosenberg</surname>
<given-names>Andrew E</given-names>
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<name>
<surname>Jain</surname>
<given-names>Rakesh K</given-names>
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<xref rid="FN1" ref-type="author-notes">*</xref>
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<contrib contrib-type="author">
<name>
<surname>Ebb</surname>
<given-names>David H</given-names>
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<aff id="A1">J Hagendoorn is a surgical resident at the University Medical Center Utrecht, The Netherlands. TP Padera, E di Tomaso and DG Duda are Instructors of Radiation Oncology; TI Yock is an Assistant Professor of Radiation Oncology, GP Nielsen and AE Rosenberg are Associate Professors of Pathology, TF Delaney is an Associate Professor of Radiation Oncology, HA Gaissert is an Associate Professor of Surgery, RK Jain is the Andrew Werk Cook Professor of Tumor Biology, and DH Ebb is Assistant Professor of Pediatrics, all at Massachusetts General Hospital, Harvard Medical School, Boston, MA. J Pearce is a practicing pediatric hematologist and oncologist at the Albany Medical Center, Albany, NY, USA</aff>
</contrib-group>
<author-notes>
<corresp id="FN1">
<label>*</label>
Correspondence Edwin L. Steele Laboratory for Tumor Biology, Department of Radiation Oncology, Massachusetts General Hospital, 100 Blossom Street, Cox 7, Boston, MA 02114, USA
<email>jain@steele.mgh.harvard.edu</email>
</corresp>
</author-notes>
<pub-date pub-type="nihms-submitted">
<day>21</day>
<month>4</month>
<year>2009</year>
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<pub-date pub-type="ppub">
<month>12</month>
<year>2006</year>
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<pub-date pub-type="pmc-release">
<day>9</day>
<month>6</month>
<year>2009</year>
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<volume>3</volume>
<issue>12</issue>
<fpage>693</fpage>
<lpage>697</lpage>
<abstract>
<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>
</abstract>
<kwd-group>
<kwd>Gorham’s disease</kwd>
<kwd>imatinib mesylate</kwd>
<kwd>lymphangiomatosis</kwd>
<kwd>pediatric</kwd>
<kwd>platelet-derived growth factor (PDGF)</kwd>
</kwd-group>
<contract-num rid="CA1">R01 CA085140-09</contract-num>
<contract-sponsor id="CA1">National Cancer Institute : NCI</contract-sponsor>
</article-meta>
</front>
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