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권선영,최미선,이혜원,이희정,신소진,조치흠 대한부인종양학회 2013 Journal of Gynecologic Oncology Vol.24 No.1
We report 2 cases of minimal deviation adenocarcinoma of the cervix and tumorlets of sex cord tumor with annular tubules (SCTATs) of the ovaries, accompanied by Peutz-Jeghers syndrome. Case 1 is a 36-year-old woman and case 2 is a 35-yearold woman. Grossly, the cervix of both cases showed markedly barrel shaped enlargement with an infiltrating tumor. Microscopically, well-differentiated atypical glands were infiltrating into the entire thickness of the cervix. The ovarian masses in case 1 were diagnosed as metastatic carcinoma in mucinous cystadenoma with tumorlets of SCTATs of the ovaries. Multiple scattered tumorlets of SCTATs were also found in the ovary of case 2. By direct DNA sequencing analysis, a frame shift mutation of the STK11/LKB1 gene was identified in case 1. Case 1 represented the more aggressive clinical course, and although the patient received additional combined chemo-radiation therapy, she expired 1 year later. In general, mutation of the STK11/LKB1gene is associated with poor clinical outcome in malignant tumors accompanied by Peutz-Jeghers syndrome.
권선영,조치흠 대한부인종양학회 2006 Journal of Gynecologic Oncology Vol.16 No.3
난소의 성기삭 종양을 닮은 자궁종양은 조직학적으로 난소 성기삭-버팀질 종양과 유사한 드문 자궁체부에서 발생하는 간엽성 종양이다. 1976년 Clement와 Scully에 의해 최초로 난소의 성기삭 종양을 닮은 자궁종양으로 명명되었고 성기삭 분화를 보이는 종양의 정도에 따라 크게 제1군 및 제2군 난소의 성기삭 종양을 닮은 자궁종양으로 분류되었다. 제1군 종양은 저등급의 자궁내막버팀질육종에 일부분이 성기삭 구조를 보이는 경우이고, 제2군 종양은 종양의 대부분 혹은 전부가 성기삭 구조를 보이는 경우로 정의하였다. 저자들은 최근 52세 여자의 자궁 기저부에서 발생한 제2군의 난소의 성기삭 종양을 닮은 자궁종양 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다. Uterine tumor resembling ovarian sex cord tumors (UTROSCTs) are uncommon neoplasms that histologically resemble ovarian sex cord tumor. Over 50 cases have been reported up to recently. Clement and Scully reported 14 cases of UTROSCT, which they devided into two groups by clinicopathological features. Group I tumors are endometrial stromal sarcomas with partially sex-cord like elements (less than 40% of total tumor volume). On the other hand, group II tumors are mostly or entirely composed of sex-cord like elements. We have experienced a very rare case of type II UTROSCT at the uterine fundus in a 52-year-old woman and report it with a brief review of the literatures.