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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2015.1
- 수록면
- 212 - 217 (6page)
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Purpose: Xp11.2 translocation renal cell carcinoma (RCC) is characterized by various translocations of the TFE3 transcription factor gene. These rare cancers occur predominantly in children and young adults. Here, we review the clinicopathological features of Xp11.2 translocation RCC.
Materials and Methods: We identified 21 patients with Xp11.2 translocation RCC. We retrospectively analyzed patient characteristics, clinical manifestations, and specific pathological features to assess definitive diagnosis, surgical and systemic treatments, and clinical outcomes.
Results: The mean age at diagnosis was 43.4±20.0 years (range, 8–80 years; 8 males and 13 females). Eleven patients were incidentally diagnosed, nine patients presented with local symptoms, and one patient presented with systemic symptoms. The mean tumor size was 6.2±3.8 cm (range, 1.9–14 cm). At the time of diagnosis, 11, 1, and 5 patients showed stage I, II, and III, respectively. Four patients showed distant metastasis. At analysis, 15 patients were disease-free after a median follow-up period of 30.0 months. Four patients received target therapy but not effectively.
Conclusions: Xp11 translocation RCC tends to develop in young patients with lymph node metastasis. Targeted therapy did not effectively treat our patients. Surgery is the only effective therapy for Xp11 translocation RCC, and further studies are needed to assess systemic therapy and long-term prognosis.
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참고문헌 (22)
참고문헌 신청
Linehan WM / 2004 / Focus on kidney cancer / Cancer Cell 6:223~228
Linehan WM / 2010 / Molecular diagnosis and therapy of kidney cancer / Annu Rev Med 61:329~343
Tomlinson GE / 1991 / Cytogenetics of a renal cell carcinoma in a 17-month-old child. Evidence for Xp11.2 as a recurring breakpoint / Cancer Genet Cytogenet 57:11~17
Lopez-Beltran A / 2006 / 2004WHO classification of the renal tumors of the adults / Eur Urol 49:798~805
Bruder E / 2004 / Morphologic and molecular characterization of renal cell carcinoma in children and young adults / Am J Surg Pathol 28:1117~1132
Linehan WM / 2010 / Molecular diagnosis and therapy of kidney cancer / Annu Rev Med 61:329~343
Tomlinson GE / 1991 / Cytogenetics of a renal cell carcinoma in a 17-month-old child. Evidence for Xp11.2 as a recurring breakpoint / Cancer Genet Cytogenet 57:11~17
Lopez-Beltran A / 2006 / 2004WHO classification of the renal tumors of the adults / Eur Urol 49:798~805
Bruder E / 2004 / Morphologic and molecular characterization of renal cell carcinoma in children and young adults / Am J Surg Pathol 28:1117~1132
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