TY - JOUR
T1 - Platelet-derived growth factor receptor-beta in Gorham's disease
AU - Hagendoorn, Jeroen
AU - Padera, Timothy P.
AU - Yock, Torunn I.
AU - Nielsen, G. Petur
AU - di Tomaso, Emmanuelle
AU - Duda, Dan G.
AU - Delaney, Thomas F.
AU - Gaissert, Henning A.
AU - Pearce, Jennifer
AU - Rosenberg, Andrew E.
AU - Jain, Rakesh K.
AU - Ebb, David H.
PY - 2006/12
Y1 - 2006/12
N2 - 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.
AB - 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.
KW - Gorham's disease
KW - imatinib mesylate
KW - lymphangiomatosis
KW - pediatric
KW - platelet-derived growth factor (PDGF)
KW - MASSIVE OSTEOLYSIS
KW - MOLECULAR REGULATION
KW - CANCER-PATIENTS
KW - THERAPY
KW - LYMPHANGIOGENESIS
KW - RADIATION
U2 - 10.1038/ncponc0660
DO - 10.1038/ncponc0660
M3 - Article
C2 - 17139320
SN - 1743-4254
VL - 3
SP - 693
EP - 697
JO - Nature clinical practice oncology
JF - Nature clinical practice oncology
IS - 12
ER -