지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2012.4
- 수록면
- 205 - 210 (6page)
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초록· 키워드
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Purpose: The aim of this study was to investigate the incidence and spectrum of malignant tumors in Korean neurofibromatosis type 1 (NF1) patients. Methods: We retrospectively reviewed 125 patients who were diagnosed with NF1 at a single institution from 1995 to 2010. The incidence, location, histologic type, and radiologic findings of malignant tumors as well as development of multiple primary tumors were analyzed. Results: Eighteen malignant tumors occurred in 16 patients (12.8%) among 125 Korean NF1 patients; 9 carcinomas, 8 sarcomas and 1 central nervous system (CNS) tumor. Five tumors were of nervous system origin and 13 were non-nervous system tumors. The locations of the tumors were as follow: 1 CNS, 2 lung, 3 breast, 3 stomach, 3 small bowel, 1 colon, 1 liver, 1 uterus, 1 neck, and 2 in extremities. Three malignant peripheral nerve sheath tumors (MPNSTs) occurred at the neck and extremity, and one in the liver. All three gastrointestinal stromal tumors (GISTs) had multiple tumors in the jejunum, and one MPNST and one pheochromocytoma were accompanied in two GISTs. Multiple primary tumors, benign or malignant were reported in 4 patients (25.0%), synchronously or metachronously. Conclusion: Korean NF1 patients had a high risk of developing malignant tumors. The common malignant tumors in Koreans such as breast, lung and stomach cancers developed frequently in addition to the NF1-related tumors such as MPNST or GIST.
목차
INTRODUCTION
METHODS
RESULTS
DISCUSSION
CONFLICTS OF INTEREST
REFERENCES
참고문헌 (20)
참고문헌 신청
1. [학술지(정기간행물)] - Zoller ME / 1997 / Malignant and benign tumors in patients with neurofibromatosis type 1 in a defined Swedish population / Cancer 79 : 2125 ~ 2131
2. [학술지(정기간행물)] - Korf BR / 2000 / Malignancy in neurofibromatosis type 1 / Oncologist 5 : 477 ~ 485
3. [학술지(정기간행물)] - / 1988 / Neurofibromatosis. Conference statement. National Institutes of Health Consensus Development Conference / Arch Neurol 45 : 575 ~ 578
4. [학술지(정기간행물)] - Jett K / 2010 / Clinical and genetic aspects of neurofibromatosis 1 / Genet Med 12 : 1 ~ 11
5. [학술지(정기간행물)] - Brems H / 2009 / Mechanisms in the pathogenesis of malignant tumours in neurofibromatosis type 1 / Lancet Oncol 10 : 508 ~ 515
2. [학술지(정기간행물)] - Korf BR / 2000 / Malignancy in neurofibromatosis type 1 / Oncologist 5 : 477 ~ 485
3. [학술지(정기간행물)] - / 1988 / Neurofibromatosis. Conference statement. National Institutes of Health Consensus Development Conference / Arch Neurol 45 : 575 ~ 578
4. [학술지(정기간행물)] - Jett K / 2010 / Clinical and genetic aspects of neurofibromatosis 1 / Genet Med 12 : 1 ~ 11
5. [학술지(정기간행물)] - Brems H / 2009 / Mechanisms in the pathogenesis of malignant tumours in neurofibromatosis type 1 / Lancet Oncol 10 : 508 ~ 515
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UCI(KEPA) : I410-ECN-0101-2013-514-002677123