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      Mucinous adenocarcinoma of the intestinal type arising from mature cystic teratoma of the ovary: a rare case report and review of the literature

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          Abstract

          Background

          Mature cystic teratomas (MCTs) are the most common germ cell tumors of the ovary. Malignant tranformation occurs in 1-2% of these neoplasms. Although most of the malignancies arising from MCTs are squamous cell carcinomas, adenocarcinoma of the gastrointestinal type is extremery rare. We herein present a case of adenocarcinoma of the intestinal type arising from a MCT.

          Case

          A 49-year-old female underwent surgery for a left ovarian tumor. The histology of the cyst walls revealed a MCT with a few hair shafts and a squamous layer, while another part of the tumor showed adenocarcinoma of the intestinal type. Five years after surgery, she is alive without disease.

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          Most cited references18

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          Squamous-cell carcinoma in mature cystic teratoma of the ovary: systematic review and analysis of published data.

          Up to a quarter of ovarian masses originate from germ cells, and many of these are mature cystic teratomas. The secondary development of malignancy is a rare but well-known phenomenon in patients with ovarian teratomas. Squamous-cell carcinoma accounts for 80% of secondary malignant transformations of ovarian teratomas. We aimed to do an up-to-date systematic review of this rare malignant transformation. 64 suitable studies provided information on 277 patients. Squamous-cell carcinoma in mature cystic teratoma was mainly found in women aged more than 50 years, with high concentrations of squamous-cell-carcinoma antigen and cancer antigen CA125, and with ovarian tumours more than 100 mm in size. Patients with FIGO stage Ia tumours had better survival than those with more advanced disease. Complete resection together with hysterectomy, bilateral salpingo-oophorectomy and lymphadenectomy for patients with advanced disease, followed by adjuvant chemotherapy with an alkylating drug was associated with higher survival, radiotherapy was not. We make proposals for investigation and treatment of this rare disorder.
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            Relative frequency of primary ovarian neoplasms: a 10-year review.

            The relative frequency of ovarian neoplasms varies according to information in different texts. In an attempt to clarify the distribution of primary ovarian neoplasms by decades of life, a 10-year retrospective review of 861 women with a postoperative diagnosis of an ovarian neoplasm was undertaken. Benign cystic teratoma was the single most common ovarian neoplasm, accounting for 44% of all neoplasms, and was 57% more frequent than benign serous tumors. Germ cell neoplasms were the most common group of benign ovarian neoplasms; epithelial neoplasms were the most common malignant neoplasm. Stromal neoplasms and neoplasms of low malignant potential were uncommon at all ages. The risk that an ovarian neoplasm was malignant increased 12-fold from ages 20-29 to 60-69. The overall risk that an ovarian neoplasm was malignant was 13% in premenopausal women and 45% in postmenopausal women.
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              Diagnosis of squamous cell carcinoma arising from mature cystic teratoma of the ovary.

              The prognoses of patients with squamous cell carcinoma of the ovary are quite poor. However, preoperative diagnosis is difficult due to the rarity of this tumor and its similarity to mature cystic teratoma (MCT). The objective of this study was to assess the value of tumor markers and clinical characteristics in making a differential diagnosis between MCT and squamous cell carcinoma arising from MCT. Between September 1979 and June 1996, 37 patients with ovarian squamous cell carcinoma arising from MCT were treated by the Tokai Ovarian Tumor Study Group. The authors evaluated tumor markers, tumor size, and age as parameters for differentiation between MCT and squamous cell carcinoma arising from MCT. Diagnostic efficiency was calculated as the sensitivity multiplied by the specificity. There were significant differences (P < or = 0.0002) in age, tumor size, and levels of squamous cell carcinoma antigen (SCC), CA125, and CEA, as well as a significant difference (P < or = 0.0396) in the CA19-9 level between MCT and squamous cell carcinoma arising from MCT. Diagnostic efficiency was highest for SCC (63.0%), followed by CA125 (50.7%). Receiver operating characteristic (ROC) curves demonstrated that CEA was the best screening marker for squamous cell carcinoma arising from MCT, whereas age and tumor size were better markers than CA125 or CA19-9. The optimal cutoff values for age and tumor size were 45 years and 99 mm, respectively, according to ROC analysis. These findings demonstrate that age and tumor size are important factors in making a differential diagnosis. In addition, SCC and CEA levels should be measured in patients age 45 years or older who have an MCT-like ovarian tumor larger than 99 mm in greatest dimension.
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                Author and article information

                Journal
                J Ovarian Res
                J Ovarian Res
                Journal of Ovarian Research
                BioMed Central
                1757-2215
                2012
                5 December 2012
                : 5
                : 41
                Affiliations
                [1 ]Department of Obstetrics and Gynecology, Osaka Medical College, 2-7, Daigaku-machi, Takatsuki, Osaka, 569-8686, Japan
                [2 ]Department of Pathology, Osaka Medical College, Osaka, Japan
                Article
                1757-2215-5-41
                10.1186/1757-2215-5-41
                3524777
                23216975
                80cf2441-3eeb-402b-b39a-0b778cfa6833
                Copyright ©2012 Takai et al.; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 30 October 2012
                : 27 November 2012
                Categories
                Case Report

                Obstetrics & Gynecology
                malignant transformation,mature cystic teratoma,adenocarcinoma,intestinal type

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