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  • Case Study
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Platelet-derived growth factor receptor-β in Gorham's disease

Nature Clinical Practice Oncology volume 3, pages 693–697 (2006)Cite this article

Abstract

Background 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.

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Figure 1: CT and MRI scans showing disease progression in the patient.
Figure 2: Images from the patient's thoracotomy.
Figure 3: Immunohistochemical analysis of the patient's tumor tissue.

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Acknowledgements

The authors thank R Padera, K Cohen, Y-L Chen and M Booth for their help in obtaining and analyzing samples; K Kozak and J Lahdenranta for help and advice; and C Smith for excellent technical assistance. This study was supported in part by the NIH (Bioengineering Research Partnership grant R01-CA85140).

Author information

Authors and Affiliations

  1. surgical resident at the University Medical Center, Utrecht, The Netherlands

    Jeroen Hagendoorn

  2. Instructors of Radiation Oncology,

    Timothy P Padera, Torunn I Yock, G Petur Nielsen, Emmanuelle di Tomaso, Dan G Duda, Thomas F Delaney, Henning A Gaissert, Andrew E Rosenberg, Rakesh K Jain & David H Ebb

  3. Assistant Professor of Radiation Oncology,

    Timothy P Padera, Torunn I Yock, G Petur Nielsen, Emmanuelle di Tomaso, Dan G Duda, Thomas F Delaney, Henning A Gaissert, Andrew E Rosenberg, Rakesh K Jain & David H Ebb

  4. Associate Professors of Pathology,

    Timothy P Padera, Torunn I Yock, G Petur Nielsen, Emmanuelle di Tomaso, Dan G Duda, Thomas F Delaney, Henning A Gaissert, Andrew E Rosenberg, Rakesh K Jain & David H Ebb

  5. Associate Professor of Radiation Oncology,

    Timothy P Padera, Torunn I Yock, G Petur Nielsen, Emmanuelle di Tomaso, Dan G Duda, Thomas F Delaney, Henning A Gaissert, Andrew E Rosenberg, Rakesh K Jain & David H Ebb

  6. Associate Professor of Surgery,

    Timothy P Padera, Torunn I Yock, G Petur Nielsen, Emmanuelle di Tomaso, Dan G Duda, Thomas F Delaney, Henning A Gaissert, Andrew E Rosenberg, Rakesh K Jain & David H Ebb

  7. Andrew Werk Cook Professor of Tumor Biology,

    Timothy P Padera, Torunn I Yock, G Petur Nielsen, Emmanuelle di Tomaso, Dan G Duda, Thomas F Delaney, Henning A Gaissert, Andrew E Rosenberg, Rakesh K Jain & David H Ebb

  8. Assistant Professor of Pediatrics, all at Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Timothy P Padera, Torunn I Yock, G Petur Nielsen, Emmanuelle di Tomaso, Dan G Duda, Thomas F Delaney, Henning A Gaissert, Andrew E Rosenberg, Rakesh K Jain & David H Ebb

  9. practicing pediatric hematologist and oncologist at the Albany Medical Center, Albany, NY, USA

    Jennifer Pearce

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  1. Jeroen Hagendoorn
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Corresponding author

Correspondence to Rakesh K Jain.

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Competing interests

RK Jain is a recipient of a research grant from AstraZeneca and serves as a consultant. RK Jain has at one time been a consultant (as a Scientific Advisory Board member) for Novartis, SK Bio-Pharmaceuticals and GLG Life Tech Ltd.

The other authors declared they have no competing interests.

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Hagendoorn, J., Padera, T., Yock, T. et al. Platelet-derived growth factor receptor-β in Gorham's disease. Nat Rev Clin Oncol 3, 693–697 (2006). https://doi.org/10.1038/ncponc0660

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