Minimal deviation adenocarcinoma of the cervix and tumorlets of sex-cord stromal tumor with annular tubules of the ovary in Peutz-Jeghers syndrome.
- Author:
Sun Young KWON
1
;
Mi Sun CHOE
;
Hye Won LEE
;
Hee Jung LEE
;
So Jin SHIN
;
Chi Heum CHO
Author Information
- Publication Type:Case Report
- Keywords: Cervix; Minimal deviation adenocarcinoma; Mutation; Peutz-Jeghers syndrome; STK11/LKB1
- MeSH: Adenocarcinoma; Cervix Uteri; Cystadenoma, Mucinous; Female; Frameshift Mutation; Humans; Ovary; Peutz-Jeghers Syndrome; Sequence Analysis, DNA
- From:Journal of Gynecologic Oncology 2013;24(1):92-95
- CountryRepublic of Korea
- Language:English
- 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-year-old 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.
