Minimal deviation adenocarcinoma of the cervix and tumorlets of sex-cord stromal tumor with annular tubules of the ovary in Peutz-Jeghers syndrome
- Affiliated Author(s)
- 권선영; 최미선; 이혜원; 이희정; 신소진; 조치흠
- Alternative Author(s)
- Kwon, Sun Young; Choe, Mi Sun; Lee, Hye Won; Lee, Hee Jung; Shin, So Jin; Cho, Chi Heum
- Journal Title
- Journal of Gynecologic Oncology
- ISSN
- 2005-0380
- Issued Date
- 2013
- Keyword
- Cervix; Minimal deviation adenocarcinoma; Mutation; Peutz-Jeghers syndrome; STK11/LKB1
- Abstract
- 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/LKB1
gene is associated with poor clinical outcome in malignant tumors accompanied by Peutz-Jeghers syndrome.
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