Platelet-derived growth factor receptor-beta in Gorham's disease

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

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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-beta and elevated circulating platelet-derived growth factor-BB.

Management Spine stabilization, thalidomide, celecoxib, interferon-alpha 2b, pamidronate, zoledronate, thoracotomy, pleurectomy, talc pleurodesis, and imatinib mesylate.

Original languageEnglish
Pages (from-to)693-697
Number of pages5
JournalNature clinical practice oncology
Volume3
Issue number12
DOIs
Publication statusPublished - Dec 2006

Keywords

  • Gorham's disease
  • imatinib mesylate
  • lymphangiomatosis
  • pediatric
  • platelet-derived growth factor (PDGF)
  • MASSIVE OSTEOLYSIS
  • MOLECULAR REGULATION
  • CANCER-PATIENTS
  • THERAPY
  • LYMPHANGIOGENESIS
  • RADIATION

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