{"paper_id":"bf65859e-b352-4aea-b8ba-140cf7cb27b7","body_text":"Clin. Exp. Obstet. Gynecol. - ISSN: 0390-6663\nXLVⅡ, n. 5, 2020\ndoi: 10.31083/j.ceog.2020.05.5316\n©2020 Kim\nPublished by IMR Press.\nThis is an open access article under the CC BY 4.0 license\n(https://creativecommons.org/licenses/by/4.0/).\nCase Report\nMature cystic teratoma of the uterus presenting as an\nendometrial polyp in a patient with a history of ovarian mature\ncystic teratoma with gliomatosis peritonei and ovarian\nendometriosis: a case report\nY.A. Kim1;\u0003\n1Department of Obstetrics and Gynecology, Ilsan Paik Hospital, Inje University, Goyang (Republic of Korea)\nSummary\nTeratomas of the uterus are rare neoplasms and are usually polypous lesions that present clinically as abnormal uterine bleeding.\nGliomatosis peritonei (GP) is characterized by peritoneal implants of non-neuronal glial tissue and is a rarely-encountered complication\nof ovarian teratomas. To the best of our knowledge, mature cystic teratoma of the uterus as well as ovarian teratoma with GP and ovarian\nendometriosis are rare phenomena. Here, we report a case of mature cystic teratoma of the uterus presenting as an endometrial polyp in\na patient with a history of ovarian teratoma with GP and ovarian endometriosis.\nKey words: Mature cystic teratomas of uterus; Ovarian teratoma; Gliomatosis peritonei; Endometriosis.\nIntroduction\nMature cystic teratomas are cystic tumors comprising\nwell-differentiated tissue derived from at least two of the\nthree germ cell layers [1, 2]. Mature cystic teratomas typ-\nically occur in the ovaries. However, they can be extrag-\nonadal, although they are rarely located in the uterus [3].\nHere, we report a case of mature cystic teratoma of the\nuterus presenting as an endometrial polyp in a patient with a\nhistory of ovarian teratoma with gliomatosis peritonei (GP)\nand ovarian endometriosis. To our knowledge, this is the\nfirst reported case and a clinically important example.\nCase Report\nA 24-year-old nulliparous woman presented with an en-\ndometrial mass with vaginal bleeding. Previous gyneco-\nlogic history included the diagnosis of ovarian teratoma\nwith GP at 19 years old and pelvic endometriosis with peri-\ntoneal gliomatosis at 21 years old. The patient had regu-\nlar menstruation after the age of 13. Her history includes\na physical examination at the time of the first operation, in\nwhich normal external genitalia and a black-colored nevus\non the left leg were noted. The subject visited the emer-\ngency room at a local hospital with sudden onset of pain and\nunderwent the first operation three days later. A large 20 \u0002\n13 \u0002 28 cm ovarian tumor was discovered via computed\ntomography (CT), with a CA125 of 115.8 U/mL (reference\nrange: < 35 U/mL). At the time of surgery, a cyst filled the\npelvis and abdomen, with peritoneal adhesions and a 5 cm\nsubserosal-typed myoma on the anterior wall of the uterus.\nThe ovarian lesion was nodular and contained necrotic ma-\nterial and bony tissue with hemorrhage. Further, there were\nnodular-shaped fragile tissues on the surface and in the right\nperitoneum and cul-de-sac area (Figure 1). A glial cell tu-\nmor was suspected after examining the frozen biopsy, after\nwhich ablation via an argon laser was performed. CA125\nlevels dropped to 20.5 U/mL after surgery.\nFigure 1. — Gliomatosis peritonei; nodular-shaped fragile tissue\non right peritoneum and cul-de-sac area on right pelvic wall.\n\nMature cystic teratoma of the uterus presenting as an endometrial polyp... 801\nFigure 2. — (A) Left ovarian cyst with chocolate-colored fluid, (B) pelvic mass with a nodular shape on the right pelvic wall.\nFigure 3. — (A) Ultrasound images of endometrial mass, (B) hysteroscopic finding of polypoid mass.\nOne year later, a 1.36 \u0002 1.06 cm echogenic mass was\ndiscovered in the right pelvic area via sonographic exam-\nination. One year and eight months later, a 1.72 \u0002 1.37\ncm semisolid cyst in the left ovary, 1.16 \u0002 0.9 cm mass\nin the right pelvic side, and 0.51 \u0002 0.28 cm echogenic\nmass without blood flow in the endometrium were noted;\nCA125 was elevated to 60.1 U/mL, indicating a need for\nlaparoscopic surgery. Despite the endometrial mass, no\nsymptoms such as vaginal bleeding were noted; therefore,\nonly laparoscopic surgery was judged necessary consider-\ning the patient’s virginity. A 3 cm cystic lesion had adhered\nto the left pelvic side wall and cul-de-sac region, while\na chocolate-colored fluid was observed during adhesioly-\nsis. Widespread peritoneal, grayish-colored, tiny lesions\nand gray-colored spots were observed on both side walls\n(Figure 2). The right pelvic mass was removed, including\nthe peritoneal wall. Pathologic results revealed the pelvic\nmass as GP and peritoneum as endometriosis. The GP was\nconsidered benign and follow-up was determined, while the\nendometriosis was treated with gonadotropin-releasing hor-\nmone (GnRH) agonist therapy to prevent recurrence. After\n6 injections of GnRH agonist, progestin was administered\nconservatively to prevent recurrence of endometriosis.\nThree years after the second operation, the endometrial\nmass showing uterine bleeding increased to 1.36 \u0002 0.5 cm\nwith blood flow, necessitating hysteroscopy (Figure 3). The\nhysteroscopic finding suggested an endometrial polyp mi-\ncroscopically composed of vascular smooth muscle, benign\nlooking glands, ducts, and cartilage, all of which are mature\ntissues and consistent with mature cystic teratoma (Figure\n4).\n\n802 Y.A. Kim\nFigure 4. — Pathology images. (A) The ovarian tumor comprises various mature tissues of three germ cell layers, such as squamous\nepithelium, salivary glands, brain tissue, and choroid plexus (H&E, \u000240). (B) and (C) Histologically, the peritoneal nodules consist of\nmature glial tissue, consistent with gliomatosis peritonei. Also, endometrial glands with stroma are scattered and admixed with mature\nglial tissue, which is highlighted via glial fibrillary acidic protein immunostaining (\u0002200). (D) The resected fragments of the endometrial\npolyp show a round configuration lined by respiratory epithelium on the surface and contain mature smooth muscle, cartilage islands,\nand salivary glands (arrow and inset) (H&E, \u000240).\nDiscussion\nTeratomas of the uterus are rare neoplasms. To date,\nmature or immature teratomas of the uterus have been re-\nported in 10 cases [3-12]. Most reported cases were mature\nteratomas; however, there are two reports of immature ter-\natomas [8, 12] and one report of an immature teratoma co-\nexisting with adenocarcinoma [6]. Teratomas of the uterine\ncorpus are usually polypous lesions that present clinically\nas abnormal uterine bleeding, consistent with this case.\nThe origin of endometrial teratoma is not completely un-\nderstood. It has been suggested that teratomas of the ovaries\nand the cervix are of parthenogenetic origin from oocytes\nafter completion of the first division [13]. However, uter-\nine teratoma origins have not been established via molecu-\nlar methods. Wang et al. compared DNA profiles of nor-\nmal uterine tissue and teratoma tissue using short tandem\nrepeats (STR) analysis and found that teratomas do not orig-\ninate from the parthenogenetic process [5]. They suggested\nthat the origin of uterine teratomas may be from pluripoten-\ntial stem cells of the uterus or primordial germ cells before\nmeiosis I.\nGP is characterized by peritoneal implants of non-\nneuronal glial tissue and is a rarely-encountered complica-\ntion of ovarian teratomas [14-22]. The etiology of GP is\nlargely unknown. Two theories regarding the development\nof GP have previously been reported, including a relation-\nship to capsular defects of the primary teratoma or dissemi-\nnation through the vascular lymphatic vessels [21]. Another\ntheory suggests that glial foci are not genetically related to\nteratomas [22]. Ferguson et al . studied polymorphic mi-\ncrosatellite (MS) loci in two cases to address the origin of\nGP [23]. They reported that glial implants and normal tis-\nsue showed heterozygosity, while the teratoma showed ho-\nmozygosity at the same MS loci, indicating that glial im-\nplants in GP are unrelated to the ovarian teratoma and arise\nfrom normal cells such as pluripotent Müllerian stem cells.\nIt is possible that peritoneal stem cells can differentiate into\nglial cells under the stimulation of some factors secreted\nfrom teratomas. Kwan et al. hypothesized that GP could re-\nsult from pluripotent stem cells in the peritoneal cavity that\ndifferentiate along astroglial pathways under the influence\nof mediators produced by either the teratoma or peritoneal\nmacrophages [24].\nTo date, eight case reports describe the association be-\n\nMature cystic teratoma of the uterus presenting as an endometrial polyp... 803\ntween GP and endometriosis [16, 18, 25-30]. Six cases\nwere associated with immature teratoma, while the remain-\ning cases involved a mature teratoma as in the present\ncase. The most scientifically supported and widely ac-\ncepted mechanism of endometriosis is retrograde effluent\nflow to the pelvic peritoneal cavities through the fallopian\ntubes during menstruation [25]. Further, tissue degener-\nation of retrograde menstrual flow plays a central role in\ninducing inflammatory pain of endometriosis by activat-\ning innate immune cells and peripheral nerve endings [31].\nIn particular, macrophages among the inflammatory pro-\ncess are a major contributor to pro-inflammatory chemotac-\ntic cytokines and a major cause of neovascularization [32].\nThe coelomic metaplasia theory has also been suggested\nas a possible mechanism, stating that embryonic cells from\nthe Müllerian ducts persist in ectopic locations. Coelomic\nmetaplasia involves the transformation of normal peritoneal\ntissue to ectopic endometrial tissue, which may be induced\nby unknown stimuli in other uncommon peritoneal lesions\n[33, 34].\nAs suggested via the prevailing theories, endometrio-\nsis and GP may have a common pathogenesis. As such,\na shared origin of both GP and endometriosis from totipo-\ntential peritoneal stem cells would explain their occasional\ncoexistence.\nIn this case study, we observed a mature cystic teratoma\ncausing vaginal bleeding in a patient having ovarian ter-\natoma with GP and endometriosis. To the best of our knowl-\nedge, mature cystic teratoma of the uterus as well as ovarian\nteratoma with GP and ovarian endometriosis are rare phe-\nnomena that have not yet been described in the English lit-\nerature. The findings in this case might lend further support\nto the theory of a stem cell origin for GP , endometriosis, and\nuterine teratoma.\nEthics Approval and Consent to Participate\nThe study was conducted in accordance with the Dec-\nlaration of Helsinki, and the protocol was approved by the\nInstitutional Review Board of Ilsan Paik Hospital (approval\nnumber: IRB 2019-03-029-001).\nAcknowledgments\nI would like to express my gratitude to all those who\nhelped me during the writing of this manuscript.\nConflict of Interest\nThe authors declare no conflict of interest.\nSubmitted: July 10, 2019\nAccepted: October 08, 2019\nPublished: October 15 2020\nReferences\n[1] Talerman A., V ang R.: “Blaustein’s pathology of the female genital\ntract. Germ cell tumors of the ovary”. 2011, 847-907.\n[2] Ayhan A., Bukulmez O., Genc C., Karamursel B.S., Ayhan A.: “Ma-\nture cystic teratomas of the ovary: case series from one institution\nover 34 years”. Eur . J. Obstet. Gynecol. Reprod. 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JSLS,\n1997, 1, 267-268.\n[29] Kim N.R., Lim S., Jeong J., Cho H.Y .: “Peritoneal and nodal\ngliomatosis with endometriosis, accompanied with ovarian imma-\nture teratoma: a case study and literature review”. Korean. J.\nPathol., 2013, 47, 587-591.\n[30] Muller A.M., Sondgen D., Strunz R., Muller K.M.: “Gliomatosis\nperitonei: a report of two cases and review of the literature”. Eur . J.\nObstet. Gynecol. Reprod. Biol. , 2002, 100, 213-222.\n[31] Laux-Biehlmann A., d’Hooghe T., Zollner T.M.: “Menstruation\npulls the trigger for inflammation and pain in endometriosis”.\nTrends. Pharmacol. Sci., 2015, 36, 270-276.\n[32] Ahn S.H., Monsanto S.P ., Miller C., Singh S.S., Thomas R., Tayade\nC.: “Pathophysiology and immune dysfunction in endometriosis”.\nBiomed. Res. Int., 2015, 2015, 795976\n[33] Burney R.O., Giudice L.C.: “Pathogenesis and pathophysiology of\nendometriosis”. Fertil. Steril., 2012, 98, 511-519.\n[34] Clement P .B., Y oung R.H., Oliva E., Sumner H.W., Scully R.E.:\n“Hyperplastic mesothelial cells within abdominal lymph nodes:\nmimic of metastatic ovarian carcinoma and serous borderline\ntumor–a report of two cases associated with ovarian neoplasms.”\nMod. Pathol., 1996, 9, 879-886.\nCorresponding Author:\nYOUNG AH KIM, M.D., Ph.D.\nDepartment of Obstetrics and Gynecology, Inje\nUniversity Ilsan-Paik Hospital, Juwha-ro 170,\nIlsandong-gu, Goyang-si, Gyeonggi-do, 10380\n(Republic of Korea)\ne-mail: camambal@gmail.com","source_license":"CC0","license_restricted":false}