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Minimal deviation adenocarcinoma of the cervix and tumorlets of sex-cord stromal tumor with annular tubules of the ovary in Peutz-Jeghers syndrome

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Affiliated Author(s)
권선영최미선이혜원이희정신소진조치흠
Alternative Author(s)
Kwon, Sun YoungChoe, Mi SunLee, Hye WonLee, Hee JungShin, So JinCho, Chi Heum
Journal Title
Journal of Gynecologic Oncology
ISSN
2005-0380
Issued Date
2013
Keyword
CervixMinimal deviation adenocarcinomaMutationPeutz-Jeghers syndromeSTK11/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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