期刊
NATURE CLINICAL PRACTICE ONCOLOGY
卷 3, 期 12, 页码 693-697出版社
NATURE PUBLISHING GROUP
DOI: 10.1038/ncponc0660
关键词
Gorham's disease; imatinib mesylate; lymphangiomatosis; pediatric; platelet-derived growth factor (PDGF)
类别
资金
- NCI NIH HHS [R01-CA85140, R01 CA085140-09, P01 CA080124, R01 CA085140] Funding Source: Medline
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.
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