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


METHODS
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.


METHODS
Gorham's lymphangiomatosis with expression of platelet-derived growth factor receptor-beta and elevated circulating platelet-derived growth factor-BB.


RESULTS
Spine stabilization, thalidomide, celecoxib, interferon-alpha2b, pamidronate, zoledronate, thoracotomy, pleurectomy, talc pleurodesis, and imatinib mesylate.
Hagendoorn
Jeroen
J
University Medical Center Utrecht, The Netherlands.
Padera
Timothy P
TP
Yock
Torunn I
TI
Nielsen
G Petur
GP
di Tomaso
Emmanuelle
E
Duda
Dan G
DG
Delaney
Thomas F
TF
Gaissert
Henning A
HA
Pearce
Jennifer
J
Rosenberg
Andrew E
AE
Jain
Rakesh K
RK
Ebb
David H
DH
D000293:Adolescent; D002916:Chylothorax / Q000209:etiology; D017809:Fatal Outcome; D006801:Humans; D008204:Lymphangiosarcoma / Q000209:etiology* / Q000378:metabolism / Q000473:pathology; D008297:Male; D010015:Osteolysis, Essential / Q000150:complications* / Q000175:diagnosis; D020574:Proto-Oncogene Proteins c-sis / Q000378:metabolism*
D020574:Proto-Oncogene Proteins c-sis