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Nat Clin Pract Oncol. 2006 Dec;3(12):693-7.

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

Author information

1
University Medical Center Utrecht, The Netherlands.

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.

PMID:
17139320
PMCID:
PMC2693369
DOI:
10.1038/ncponc0660
[Indexed for MEDLINE]
Free PMC Article

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