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


Nat Clin Pract Oncol. 2006;3(12):693-697. 

In This Article


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-2b, pamidronate, zoledronate, thoracotomy, pleurectomy, talc pleurodesis, and imatinib mesylate.


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