{"paper_id":"118a0220-16e9-40f5-b9f2-849a0cd56535","body_text":"918 Korean J Radiol 14(6), Nov/Dec 2013 kjronline.org\nINTRODUCTION\nEndometriosis is a common gynecologic condition which \nis characterized by the presence of endometrial tissue \nimplants composed of endometriotic glands and stroma \noutside the uterus. Endometriosis is considered to be a \nprecancerous disease, and approximately 2.5% of women \nwith endometriosis develop an endometriosis-associated \novarian cancer (1). Endometrioid and clear cell carcinomas \nare the most commonly reported histologic types of \nendometriosis-associated ovarian cancers (2, 3), but \nendocervical MBTs are also reported to be endometriosis-\nassociated tumors that are much less common than \nMR Imaging Findings of Extraovarian Endocervical \nMucinous Borderline Tumors Arising from Pelvic \nEndometriosis\nDong Myung Yeo, MD 1 , Sung Eun Rha, MD 1 , Jae Young Byun, MD 1 , Ahwon Lee, MD 2 , Mee-Ran Kim, MD 3\nDepartments of 1 Radiology, 2 Hospital Pathology, and 3 Obstetrics and Gynecology, Seoul St. Mary’s Hospital, The Catholic University of Korea, Seoul \n137-701, Korea\nWe report MR imaging findings of a rare case of endocervical mucinous borderline tumor (MBT) involving the cul-de-sac \nand left fallopian tube arising from extensive pelvic endometriosis with pathologic correlation in a 35-year-old woman \npresented with vague pelvic pain. Endocervical MBT is a type of endometriosis-associated carcinoma. Imaging findings of \nendocervical MBT are unilocular or oligolocular cystic lesions with enhancing mural nodules, which are different from those \nof the more common intestinal type MBT.\nIndex terms:  Endocervical; Müllerian; Endometriosis; Mucinous borderline tumor\nReceived April 27, 2013; accepted after revision July 19, 2013.\nCorresponding author: Sung Eun Rha, MD, Department of \nRadiology, Seoul St. Mary’s Hospital, The Catholic University of \nKorea, 222 Banpo-daero, Seocho-gu, Seoul 137-701, Korea.\n• Tel: (822) 2258-1430  • Fax: (822) 599-6771\n• E-mail: serha@catholic.ac.kr\nThis is an Open Access article distributed under the terms of \nthe Creative Commons Attribution Non-Commercial License \n(http://creativecommons.org/licenses/by-nc/3.0) which permits \nunrestricted non-commercial use, distribution, and reproduction in \nany medium, provided the original work is properly cited. \nCase Report |  Genitourinary Imaging\nKorean J Radiol 2013;14(6):918-922\nendometrioid or clear cell tumors. To the best of our \nknowledge, there is no literature on the imaging features of \nextraovarian endocervical MBT (4, 5). Therefore, we report \na rare case of extraovarian endocervical MBT involving the \ncul-de-sac and fallopian tube associated with extensive \npelvic endometriosis.\nCASE REPORT\nA 35-year-old woman (gravida 0, para 0, abortus 0) \npresented with dysmenorrhea and vague lower abdominal \npain. Her past history was unremarkable. At the time of \npresentation, she was having regular menstrual cycles. She \nwas afebrile and her abdomen was soft and flat without any \ntender points. Laboratory data were normal except for the \nelevated level of CA 125 (44 U/mL; normal 0-35 U/mL) and \nCA 19-9 (271 U/mL; normal 0-37 U/mL).\nMRI showed an elongated tubular cystic lesion with high \nsignal intensity on both T1- and T2-weighted images in \nthe left adnexa, which was diagnosed as hematosalpinx. \nAn axial T2-weighted image showed a stellate low signal \nintensity lesion with obliteration of the cul-de-sac, \nsuggestive of deep infiltrating endometriosis. The uterus \nwas diffusely enlarged with focal adenomyosis and an \nhttp://dx.doi.org/10.3348/kjr.2013.14.6.918\npISSN 1229-6929  ·  eISSN 2005-8330\n\nKorean J Radiol 14(6), Nov/Dec 2013kjronline.org 919\nMRI of Endocervical Tumor\nA\nC\nE\nB\nD\nF\nFig. 1. Thirty five-year-old woman with extraovarian endocervical mucinous borderline tumor arising from pelvic endometriosis.\nConsecutive axial T2-weighted (A, B) , fat-suppressed T1-weighted (C, D)  and contrast-enhanced fat-suppressed T1-weighted (E, F)  MR images. \nMR images show elongated tubular cystic lesion (asterisk in A , C ) with high signal intensity on both T1- and T2-weighted images in left adnexa, \nwhich is diagnosed as hematosalpinx. Axial T2-weighted image (B)  shows stellate low signal intensity lesion (arrow in B ) with obliteration of \ncul-de-sac, suggestive of deep infiltrating endometriosis. Uterus is diffusely enlarged with focal adenomyosis and intramural myoma in posterior \nwall. Two small cystic lesions (arrowheads in A , D ) with high signal intensity on T2-weighted image and slightly high signal intensity on T1-\nweighted image are present in cul-de-sac along posterior margin of uterus. Following contrast-infusion, peripheral nodular enhancement is noted \nin cystic lesions (arrowheads in E , F ), raising possibility of malignancy.\n\nKorean J Radiol 14(6), Nov/Dec 2013 kjronline.org920\nYeo et al.\nintraumral myoma in the posterior wall. Two small cystic \nlesions with mural nodules were present in the cul-de-\nsac along the posterior margin of the uterus. These cystic \ncomponents showed high signal intensity similar to that \nof urine on T2-weighted images as well as high signal \nintensity that was slightly higher than that of myometrium \non fat-suppressed T1-weighted images. Peripheral nodular \nenhancement was seen in the cystic lesions on gadolinium-\nenhanced T1-weighted images, raising the possibility of \nmalignancy (Fig. 1). Mural nodules were not definitely seen \nin the left fallopian tube.\nSurgery revealed severe pelvic endometriosis involving \nbilateral ovaries, the cul-de-sac, rectosigmoid colon and \nleft hydrosalpinx. On histopathologic examination, cul-\nde-sac masses and the left fallopian tube showed mucin-\nfilled cysts and branching papillary projections. The \npapillae were lined by endocervical type mucinous cells. \nMicroscopic examination revealed a gradual transition from \nthe epitheliums of endometeriosis to mucinous tumor cells. \nThe pathologic diagnosis was confirmed as endocervical \nmucinous boderline tumor involving the cul-de-sac and left \nfallopian tube arising from extensive pelvic endometriosis.\nDISCUSSION\nEndometriosis, like cancer, is characterized by growth \nof blood vessels (angiogenesis) and a decrease in the \nnumber of cells undergoing apoptosis (6). Therefore, \nendometriosis is considered as a possible precancerous \ndisease. Malignant transformations have been reported in \nwomen with endometriosis with a frequency of about 2.5%; \nwith 80% in the ovary and 20% in the extraovarian sites \n(2, 7, 8). Extraovarian endometriosis-associated cancers \nwere reported to occur in various anatomic locations in the \nfollowing frequencies: rectovaginal sites (36%), colorectum \n(11%), bladder (9%), vagina (7%), pelvic ligaments (4%), \numbilicus (4%), cervix (4%) and fallopian tube (4%) (7). \nThe histopathologic criteria for the diagnosis of \nendometriosis-associated ovarian carcinoma, first proposed \nby Sampson in 1924, require a combination of the following \nthree findings: 1) demonstration of both cancerous and \nbenign endometrial tissues in the tumor, 2) histology of \nthe neoplasm is compatible with an endometrial origin, \nand 3) no other primary tumor sites affected. These criteria \nare still used and extended to extraovarian sites (8). In \naddition, Scott added the strict criteria of the presence of \nthe dysplastic phase or transition zone between the benign \nendometriosis and the carcinoma, but the transition zone is \nonly detected in 36-42% of cases at the present time (8, 9). \nAlthough much less common than clear cell or endometrioid \ncarcinomas, endocervical MBT is known to be associated \nwith pelvic endometriosis (10).\nOvarian mucinous tumors are currently classified into \ntwo subtypes based on pathologic findings: intestinal \ntype and endocervical or müllerian type (11). Intestinal \ntype mucinous ovarian tumors are much more common \nthan endocervical type tumors, and nearly all reports of \nmucinous ovarian tumors describe the former. However, \nG H\nFig. 1. Thirty five-year-old woman with extraovarian endocervical mucinous borderline tumor arising from pelvic endometriosis.\nHistopathologic findings of cul-de-sac masses show multiple mucin-filled cysts (asterisks in G ) and papillary projections (arrows in G ) lined by \nendocervical type mucinous cells. These masses were confirmed as endocervical mucinous borderline tumors. Gradual transition from epitheliums \nof endometriosis (white arrow in H ) to mucinous tumor cells (black arrow in H ) was identified.\n\nKorean J Radiol 14(6), Nov/Dec 2013kjronline.org 921\nMRI of Endocervical Tumor\nendocervical MBTs show different clinicopathologic features \nfrom intestinal MBTs. First, intestinal MBTs are bilateral \nin only 0-10%, whereas endocervical MBTs are commonly \nbilateral (12-47%). Secondly, intestinal types are almost \nnever associated with endometriosis, but endocervical \ntypes are often associated with endometriosis (30-53% \nof cases). Thirdly, intestinal types are usually multilocular \ncysts without papillary features, whereas endocervical \ntypes are unilocular or oligolocular cysts with fine \npapillary projections, resembling serous borderline tumors \nor malignant tumors arising in endometriosis such as \nendometrioid or clear cell carcinomas.\nThe imaging findings of intestinal and endocervical MBTs \nare also quite different. The well-known imaging features \nof ovarian MBTs are multilocular cystic lesions with variable \nsignal intensities in each locule, known as stained glass \nappearance, which may reflect the imaging findings of more \ncommon intestinal MBTs. In contrary, imaging findings of \nendocervical MBTs in our case and from other reports show \nunilocular or paucilocular cystic lesions with papillary \nprojections or mural nodules corresponding to the gross \nfeatures (4, 12). However, these findings can also be seen \nin clear cell or endometrioid borderline tumors as well \nas in carcinomas (5). Kataoka et al. (4) suggested mural \nnodules in three cases of endocervical MBTs showing marked \nhigh signal intensity on T2-weighted MR images due to \nprominent mucin and stromal edema, which may be helpful \nto differentiate endocervical mucinous borderline tumors \nfrom other carcinomas arising from endometriotic cysts. \nHowever, Tanaka et al. (13) analyzed 49 cases of ovarian \nendometriotic cysts and revealed that both benign and \nmalignant mural nodules may show high signal intensity on \nT2-weighted images. Also, the mural nodules in our case \ndid not show striking high signal intensity on T2-weighted \nimages. Therefore, it may be difficult to differentiate \nborderline ovarian tumors from invasive carcinomas based \non the signal intensity of mural nodules. Our case is \ndifferent from previous reports in that the endocervical MBT \noccurred in the extraovarian site including the cul-de-sac \nand fallopian tube, but the imaging findings of extraovarian \nendocervical MBTs were similar to those of ovarian \nendocervical MBTs.\nThe prognosis of endocervical MBTs is much better \nthan clear cell or endometrioid carcinomas arising from \nendometriotic cysts (4). However, endocervical MBT is of \nclinical significance because the tumor occurs more often \nin young, nulliparous or infertile women, many of whom \nwish to preserve their fertility and thus agree to only \nlimited surgery such as cystectomy (10). If preservation \nof potential fertility is supreme, then careful inspection of \nboth the remaining and contralateral ovary for any residual \ntumor or endometriotic foci is critical, as these may be \nfuture sources of recurrent tumors.\nIn summary, endocervical MBT is an additional tumor \ntype arising from endometriosis and can involve the \novary as well as extraovarian sites. Imaging findings of \nendocervical MBT arising from endometriosis involving the \novary or extraovarian sites are unilocular or oligolocular \ncystic lesions with enhancing mural nodules, which are \nquite different from those of the more common intestinal \ntype MBT. Although the imaging findings of endocervical \nMBT are not specific, radiologists should keep in mind that \nextraovarian involvement of MBT can occur in patients with \nendometriosis.\nREFERENCES\n1. Melin A, Sparén P, Persson I, Bergqvist A. 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