| Literature DB >> 17139320 |
Jeroen Hagendoorn1, 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.
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-alpha2b, pamidronate, zoledronate, thoracotomy, pleurectomy, talc pleurodesis, and imatinib mesylate.Entities:
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Year: 2006 PMID: 17139320 PMCID: PMC2693369 DOI: 10.1038/ncponc0660
Source DB: PubMed Journal: Nat Clin Pract Oncol ISSN: 1743-4254