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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2019.1
- 수록면
- 1,549 - 1,556 (8page)
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Purpose Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) is a rare genetic syndrome resulting from germline mutations in fumarate hydratase. The combination of bevacizumab plus erlotinib showed promising interim results for HLRCC-associated renal cell carcinoma (RCC). Based on these results, we analyzed the outcome of bevacizumab plus erlotinib in Korean patients with HLRCC-associated RCC. Materials and Methods We retrospectively reviewed the efficacy and safety of bevacizumab plus erlotinib in patients with HLRCC-associated RCC who were confirmed to have germline mutations in fumarate hydratase. The primary endpoint was the objective response rate (ORR), while the secondary endpoints were progression-free survival (PFS) and overall survival (OS). Results We identified 10 patients with advanced HLRCC-associated RCC who received bevacizumab plus erlotinib. Median age at diagnosis was 41 years, and five of the patients had received the combination as first- or second-line treatments. The ORR was 50% and the median PFS and OS were 13.3 and 14.1 months, respectively. Most adverse events were predictable and manageable by conventional measures, except for one instance where a patient died of gastrointestinal bleeding. Conclusion This is the first real-world outcome of the treatment of advanced HLRCC-associated RCC. Bevacizumab plus erlotinib therapy showed promising activity with moderate toxicity. We should be increasingly aware of HLRCC-associated RCC and bevacizumab plus erlotinib should be a first-line treatment for this condition, unless other promising data are published.
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참고문헌 (16)
참고문헌 신청
Tomlinson IP / 2002 / Germline mutations in FH predispose to dominantly inherited uterine fibroids, skin leiomyomata and papillary renal cell cancer / Nat Genet 30:406~410
Linehan WM / 2013 / Molecular pathways : Fumarate hydratase-deficient kidney cancer : targeting the Warburg effect in cancer / Clin Cancer Res 19:3345~3352
Lehtonen HJ / 2011 / Hereditary leiomyomatosis and renal cell cancer : update on clinical and molecular characteristics / Fam Cancer 10:397~411
Moch H / 2016 / The 2016 WHO classification of tumours of the urinary system and male genital organs-part A : renal, penile, and testicular tumours / Eur Urol 70:93~105
Srinivasan R / 2015 / New strategies in renal cell carcinoma : targeting the genetic and metabolic basis of disease / Clin Cancer Res 21:10~17
Linehan WM / 2013 / Molecular pathways : Fumarate hydratase-deficient kidney cancer : targeting the Warburg effect in cancer / Clin Cancer Res 19:3345~3352
Lehtonen HJ / 2011 / Hereditary leiomyomatosis and renal cell cancer : update on clinical and molecular characteristics / Fam Cancer 10:397~411
Moch H / 2016 / The 2016 WHO classification of tumours of the urinary system and male genital organs-part A : renal, penile, and testicular tumours / Eur Urol 70:93~105
Srinivasan R / 2015 / New strategies in renal cell carcinoma : targeting the genetic and metabolic basis of disease / Clin Cancer Res 21:10~17
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