{"paper_id":"69055db0-92c5-4faf-ac2a-114664d43e17","body_text":"523www.eymj.org\nINTRODUCTION\nA case of intra-endometrial uterine leiomyoma (IEUL) ac -\ncompanied by large Sertoli-Leydig cell tumor (SLCT) of ovary \nis presented. An IEUL is rare and completely different from a \nrelatively common submucosal type of leiomyoma.1 SLCT is \nalso a rare sex cord stromal tumor of ovary, characterized by vir-\nilization and pelvic mass in most patients. Although virilization \nis the most common manifestation, it is unfortunately not in \nall cases, and secondary amenorrhea could be the only symp-\ntom in many cases.2 The majority of SLCT are benign, therefore, \nyoung patient at early stage could prefer fertility preserving \nconservative surgeries, those with risk factors should receive \nchemotherapy and long-term follow up.3-6 The large SLCT in \nthis case did not cause any hormonal imbalance, therefore, \nthe tumor was diagnosed late. Herein, we describe a rare case \nof intra-endometrial leiomyoma presenting heavy menstrual \nbleeding accompanied by SLCT with poor prognostic factors. \nCASE REPORT\nA 50-year-old gravida 7 para 2 woman was referred with pal-\npable pelvic mass and heavy menstrual bleeding. She had no \nvirilization signs and no history of menstrual irregularity, \nwhich is often preceded by the virilization. Pelvic examination \nrevealed a hard enlarged uterus and about fetal head sized \nmass. The ultrasonography revealed a solid hypoechoic mass \nof 13×12 cm size with cystic lesion and increased vascularity \n(Fig. 1A). The uterus enlarged to gestational 3 month size with \nmixed echogenicity, compatible to the findings of adenomyo-\nA Rare Case of Intra-Endometrial Leiomyoma of  \nUterus Simulating Degenerated Submucosal  \nLeiomyoma Accompanied by a Large Sertoli-Leydig \nCell Tumor \nKyungah Jeong1, Sa Ra Lee1, and Sanghui Park2\nDepartments of 1Obstetrics and Gynecology and 2Pathology, Ewha Womans University School of Medicine, Seoul, Korea.\nA 50-year-old peri-menopausal woman presented with hard palpable mass on her lower abdomen and anemia from heavy men-\nstrual bleeding. Ultrasonography showed a 13×12 cm sized hypoechoic solid mass in pelvis and a 2.5×2 cm hypoechoic cystic \nmass in uterine endometrium. Abdomino-pelvic computed tomography revealed a hypodense pelvic mass without enhance-\nment, suggesting a leiomyoma of intraligamentary type or sex cord tumor of right ovary with submucosal myoma of uterus. Lapa-\nroscopy revealed a large Sertoli-Leydig cell tumor of right ovary with a very rare entity of intra-endometrial uterine leiomyoma \naccompanied by adenomyosis. The final diagnosis of ovarian sex-cord tumor (Sertoli-Leydig cell), stage Ia with intra-endometrial \nleiomyoma with adenomyosis, was made. Considering the large size of the tumor and poorly differentiated nature, 6 cycles of \nchemotherapy with Taxol and Carboplatin regimen were administered. There is neither evidence of major complications nor re-\ncurrence during 20 months’ follow-up. \nKey Words:  Sertoli-Leydig cell tumor, intra-endometrial leiomyoma, submucosal myoma, heavy menstrual bleeding\nYonsei Med J 2016 Mar;57(2):523-526\nhttp://dx.doi.org/10.3349/ymj.2016.57.2.523\nCase Report\npISSN: 0513-5796 · eISSN: 1976-2437\nReceived: February 25, 2015   Revised: April 27, 2015\nAccepted: June 2, 2015\nCorresponding author: Dr. Sa Ra Lee, Department of Obstetrics and Gynecology, \nEwha Womans University School of Medicine, 1071 Anyangcheon-ro, Yangcheon-\ngu, Seoul 07985, Korea.\nTel: 82-2-2650-6011, Fax: 82-2-2647-9860, E-mail: sarahmd@ewha.ac.kr\n•The authors have no financial conflicts of interest.\n© Copyright: Yonsei University College of Medicine 2016\nThis is an Open Access article distributed under the terms of the Creative Com -\nmons Attribution Non-Commercial License (http://creativecommons.org/licenses/\nby-nc/3.0) which permits unrestricted non-commercial use, distribution, and repro-\nduction in any medium, provided the original work is properly cited.\n\nhttp://dx.doi.org/10.3349/ymj.2016.57.2.523524\nRare Case of Intraendometrial Leiomyoma\nFig. 1. Preoperative ultrasonographic and computed tomography findings. (A) A 13×12 cm solid hypoechoic mass with multiple cystic lesions was noted \non the right pelvic area accompanied by blood flow shadow. (B) About 2.5×2 cm hypoechoic solid mass (arrowheads) with internal cystic lesion was \nnoted in the near endometrium. (C) A ring like hypodense mass in uterine cavity (arrow) and a large pelvic mass without enhancement, suggesting a leio-\nmyoma of intraligamentary type or sex cord tumor of right ovary.\nA\n B\n C\nFig. 2. Pelviscopic findings. (A) A 13×12 cm sized, yellow-tan colored ovarian tumor with multiple vessel engorgement. (B) Multiple fragments of yellow-\ntan colored ovarian tumor. (C) Cut section of the uterus shows white-gray tan tumor like-lesion with focal cystic degeneration centered in the submucosal \nlayer of uterine corpus. (D) Enlarged photo of endometrial cystic mass (arrowheads).\nA\nC\nB\nD\n\n525http://dx.doi.org/10.3349/ymj.2016.57.2.523\nKyungah Jeong, et al.\nsis. Notably, about 2.5×2 cm hypoechoic solid mass with in-\nternal cystic lesion was noted in near endometirum (Fig. 1B). \nLeft ovary was normal, however, right ovary was not found. \nComputed tomography revealed a hypodense pelvic mass \nwithout enhancement, suggesting a leiomyoma of intraliga-\nmentary type or sex cord tumor of right ovary (Fig. 1C). En-\nlarged uterus with isodense mass protruding into uterine cav-\nity with internal cystic lesion was noted. Laboratory data, \nincluding testosterone, estrogen, thyroid stimulating hormone, \nand CA-125, were within normal limit, with the exception of \ndecreased hemoglobin level (6.8 g/dL). A provisional diagno-\nsis was ovarian fibroma or intraligamentary leiomyoma ac-\ncompanied by uterine adenomyosis with degenerated submu-\ncosal myoma. Laparoscopy revealed a solid, yellow-tan colored, \nsmooth-surface mass with intact capsule abundant of blood \nvessels (Fig. 2A). Frozen biopsy for the right ovarian mass re-\nvealed benign ovarian tumor, and laparoscopically assisted \nvaginal hysterectomy with right oophorectomy was performed. \nThe right ovarian mass was yellow colored solid tumor (Fig. \n2B). Cut section of the uterus revealed white to gray tan-col-\nored tumor-like lesion with cystic degeneration in submucosal \nlayer (Fig. 2C and D). Histopathologically, several discrete \nnodular lesions composed of spindle-shaped smooth muscle \ncells were present within the endometrium (Fig. 3A and B). \nOvarian tumor revealed predominantly spindle cell growth \npattern characterized by minimal differentiation of Sertoli \ncells. Only focal area showed nests and thin cords resembling \nsex cords (Fig. 3C). Immunohistochemically, tumor cells were \npositive for α-inhibin, and some Leydig cells were highlighted \nby the calretinin stain (Fig. 3D and E). \nConsidering the high-risk factors (poor differentiation, large \ntumor size, and old age) of this patient, we decided to perform \nthe second laparoscopy for complete staging of ovarian tumor. \nAbdominal cavity was explored systematically, however, there \nwas no tumor deposit anywhere else in the cavity and perito-\nneal washing cytology was negative for malignant cells. Left \nsalpingo-oophorectomy and cholecystectomy were performed \nsimultaneously for multiple gallbladder stones.\nThe postoperative course was uneventful. The final patho-\nlogic diagnosis was poorly differentiated SLCT of ovary stage \nIa and IEUL with adenomyosis. We had a detailed discussion \nabout treatment options and decided to initiate chemothera-\npy considering the large size of tumor and poorly differentiat-\ned nature. Six cycles of chemotherapy with Taxol and Carbo-\nplatin regimen were administered 3 times a week. There is no \nevidence of recurrence during 20 months’ follow-up. \n \nDISCUSSION\nIEUL is an extremely rare and is completely different from a \nsubmucosal leiomyoma connected to myometrium covered \nFig. 3. Pathologic examination. (A) Scanning view of the endometrial tumor shows proliferative endometrioid-type glands varying in number and shape. \nTwo discrete nodular lesions formed by smooth muscle are identified in the endometrium (arrowheads). (B) High-power view shows spindle-shaped \nsmooth muscle cells arranged in fascicles (H&E, ×200). (C) Tumor shows diffuse sarcomatoid growth pattern focally associated with cord formation of \nSertoli cells (arrowheads). Leydig cells are not conspicuous (H&E, ×100). (D) Tumor cells are immunoreactive for α-inhibin (×100). (E) Calretinin immuno-\nhistochemistry shows Leydig cells which are focally found in peripheral clusters (arrowheads) (×100).  H&E, hematoxylin and eosin.\nC\nA\n B\nD\n E\n\nhttp://dx.doi.org/10.3349/ymj.2016.57.2.523526\nRare Case of Intraendometrial Leiomyoma\nwith submucosal layer. IEUL means multiple leiomyoma is-\nlands embedded in submucosal layer without connection \nwith myometrium. These foci have sometimes been referred \nas smooth muscle metaplasia. A pleuripotential cell in uterus \ncan differentiate into endometrial stroma and smooth mus-\ncle.7 Other differential diagnosis includes endometrial polyp, \nadenomyomatous polyp, and atypical polypoid adenomyo-\nma. Adenomyomatous polyp is very similar to IEUL since ad-\nenomyomatous polyp has a mixture of myomatous stroma \nand glands. However, it can be excluded because there is no \ndiscrete nodular lesion formed by the smooth muscle cells in \nthe endometrium. Atypical polypoid adenomyoma can also \nbe excluded because it has irregular gland architecture in ad-\ndition to a myomatous stroma. \nSLCT is a rare sex cord stromal tumor of the ovary, charac-\nterized by virilization and pelvic mass. It is typically diagnosed \nin young women.2 However, in this case, it manifested late in \nher 50’s. Poorly differentiaed cells in this case did not release \ntestosterone or estrogen. It did not cause any hormonal dis-\nturbance, which is shown in about two thirds of SLCT cases. \nNormal serum hormonal status may delay the diagnosis until \nthe tumor is growing to 13 cm, a relatively large size compared \nwith the other reported SLCTs.2,8 The large tumors (over 10 \ncm), tumor rupture, and tumors of poor differentiation are \nknown to be related with no endocrine changes and more ag-\ngressive behaviors.9,10 Most SLCT are benign, and few cases \nare low grade malignancy and stage I at the time of surgery. \nWell-differentiated SLCT are benign and there is no recur -\nrence. However, 59% of poorly differentiated SLCT and 11% of \nthose with intermediate differentiation are malignant.9 Total \nhysterectomy and bilateral salpingo-oophorectomy are rec-\nommended for women who finished childbearing. For early \nstaged young women who want preserve fertility, conserva-\ntive surgery of unilateral salpingo-oophorectomy could be an \nalternative treatment option. The necessity of pelvic lymphad-\nenectomy is controversial.11 Postoperative chemotherapy has \nnot demonstrated the benefit, and the optimal regimen still \nremain unclear. In this case, however, considering high-risk \nfactors (poor differentiation, large tumor size, and old age) we \ncounseled thoroughly with the patient and conducted 6 cycles \nof chemotherapy. In this case, we could learn the rare entity of \nIEUL which can present as a degenerated submucosal leio-\nmyoma. Although the clinical symptom and prognosis of IEUL \nare not different from those of submucosal myoma, the histo-\nlogic location of the tumor in submucosal layer is distinct from \neach other. The hormone which is known to be secreted from \nSLCT is not estrogen, but androgen. Therefore, the co-occur-\nrence of IEUL is thought to be not related to the presence of \nSLCT . We should think of the possibility of SLCT whenever we en-\ncounter a hypoechoic solid pelvic mass in ultrasonography, and \nshould perform a thorough physical examination, including \nvirilization signs which are usually accompanied in most cas-\nes, although it was not manifested in this case. \nREFERENCES\n1. Crum CP , Nucci MR, Lee KR. Diagnostic Gynecologic and Obstet-\nric Pathology. 2nd ed. Philadelphia: Saunders; 2011.\n2. Young RH, Scully RE. Ovarian Sertoli-Leydig cell tumors. A clini-\ncopathological analysis of 207 cases. Am J Surg Pathol 1985;9:543-\n69.\n3. Zhang M, Cheung MK, Shin JY, Kapp DS, Husain A, Teng NN, et al. \nPrognostic factors responsible for survival in sex cord stromal tu-\nmors of the ovary--an analysis of 376 women. Gynecol Oncol 2007; \n104:396-400.\n4. Bhat RA, Lim YK, Chia YN, Yam KL. Sertoli-Leydig cell tumor of \nthe ovary: analysis of a single institution database. J Obstet Gynae-\ncol Res 2013;39:305-10.\n5. Roth LM, Anderson MC, Govan AD, Langley FA, Gowing NF , \nWoodcock AS. Sertoli-Leydig cell tumors: a clinicopathologic \nstudy of 34 cases. Cancer 1981;48:187-97.\n6. Kurman RJ, Ellenson HL, Ronnett BM. Blaustein’s Pathology of \nthe Female Genital Tract. 6th ed. New York: Springer; 2011.\n7. Gui T , Cao D, Shen K, Yang J, Zhang Y, Yu Q, et al. A clinicopatho-\nlogical analysis of 40 cases of ovarian Sertoli-Leydig cell tumors. \nGynecol Oncol 2012;127:384-9.\n8. Lantzsch T , Stoerer S, Lawrenz K, Buchmann J, Strauss HG, Koelbl \nH. Sertoli-Leydig cell tumor. Arch Gynecol Obstet 2001;264:206-8.\n9. McGuire WP , Hoskins WJ, Brady MF , Kucera PR, Partridge EE, \nLook KY, et al. Cyclophosphamide and cisplatin compared with \npaclitaxel and cisplatin in patients with stage III and stage IV ovar-\nian cancer. N Engl J Med 1996;334:1-6.\n10. Persechini ML, Motton S, Leguevaque P , Donadille F , Escourrou \nG, Vierasu B, et al. Virilising ovarian tumour: a case associating a \nSertoli-Leydig cell tumour and a Brenner tumour. Gynecol Endo-\ncrinol 2011;27:345-50.\n11. Brown J, Sood AK, Deavers MT , Milojevic L, Gershenson DM. Pat-\nterns of metastasis in sex cord-stromal tumors of the ovary: can rou-\ntine staging lymphadenectomy be omitted? Gynecol Oncol 2009; \n113:86-90.","source_license":"CC0","license_restricted":false}