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

Identifieur interne : 007889 ( Main/Exploration ); précédent : 007888; suivant : 007890

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


Affiliations:


Links toward previous steps (curation, corpus...)


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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<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>
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<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>
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