Upregulation of receptor tyrosine kinase-like orphan receptor 2 in idiopathic pulmonary fibrosis.

BACKGROUND/AIMS: Receptor tyrosine kinase-like orphan receptor 2 (ROR2) is a major regulator of Wnt signaling, which is involved in fibroblast dysfunction. Because its role has not been evaluated in idiopathic pulmonary fibrosis (IPF), we examined the clinical implications of ROR2 expression. METHODS: ROR2 mRNA expression was measured using reverse transcription polymerase chain reaction in lung tissue-derived fibroblasts from IPF patients (n = 14) and from controls (n = 10). ROR2 protein was measured using enzyme-linked immunosorbent assay in primary fibroblasts from IPF patients (n = 14) and controls (n = 10), and in bronchoalveolar lavage (BAL) fluids obtained from normal controls (NC; n = 30). IPF patients (n = 84), and other patients with interstitial lung diseases, including nonspecific interstitial pneumonia (NSIP; n = 10), hypersensitivity pneumonitis (HP; n = 10), and sarcoidosis (n = 10). RESULTS: ROR2 mRNA and protein levels were significantly higher in IPF fibroblasts than in controls (p = 0.003, p = 0.0017, respectively). ROR2 protein levels in BAL fluids from patients with IPF were significantly higher than in those from NC (p < 0.001), and from patients with NSIP (p = 0.006), HP (p = 0.004), or sarcoidosis (p = 0.004). Receiver operating characteristic curves showed a clear difference between IPF and NC in ROR2 protein level (area under the curve, 0.890; confidence interval, 0.829 to 0.950; p < 0.001). ROR2 protein levels were significantly higher in GAP stage III than in GAP stages I and II (p = 0.016). CONCLUSION: ROR2 may be related to the development of IPF, and its protein level may be a useful and severity-dependent candidate marker for IPF.

A case of renal synovial sarcoma: complete remission was induced by chemotherapy with doxorubicin and ifosfamide.

We experienced a case of primary renal synovial sarcoma in a 32 year-old woman. On admission, she complained of intermittent abdominal pain. On radiologic examination, a 12 x 10 cm-sized soft tissue mass was detected on the left kidney. The tumor had histologic and immunophenotypic features that were consistent with spindle cell type monophasic synovial sarcoma. Four months after complete resection of the tumor, a unilateral hematogenous metastasis developed in the lung. She was treated with combined chemotherapy of doxorubicin and ifosfamide every four weeks, and complete remission was achieved. We herein describe the case with a brief review.