{"paper_id":"f2ca09d9-aae2-4366-9609-4f76ab2fe77d","body_text":"WWW.KJOG.ORG 561\nCLEAR CELL ADENOCARCINOMA ARISING FROM \nADENOMYOSIS MIMICKING LEIOMYOMA: A CASE REPORT\nYoo Jeong Shin, MD, Se-Eun Cho, MD, Chang Ohk Sung, MD, Chel Hun Choi, MD, Duk-Soo Bae, MD, PhD\nDepartment of Obstetrics and Gynecology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea\nThe incidence of cancer from adenomyosis is rare. Previously, only two cases of clear cell adenocarcinoma (CCA) arising from \nadenomyosis have been reported in English literature. Here, we report a case of CCA arising from adenomyosis. A 52-year-old \npostmenopausal Korean woman presented with complaints of vaginal bleeding and back pain. Endometrial biopsy revealed \nendometrial polyp with atrophic change and transvaginal ultrasonography showed myomas with cystic change. Magnetic \nresonance imaging revealed a cystic degenerative mass consistent with leiomyoma in the posterior portion of uterus body. And the \nserum level of CA-125 was 17.7 U/mL. Hysterectomy revealed a yellow-ten solid mass in the myometrium that was diagnosed as \nCCA arising from adenomyosis. The tumor was mainly located in the myometrium and transition between adenomyosis and CCA \nalong with endometrial stromal cell was identifi ed. Malignant tumor arising from adenomyosis could be considered as a differential \ndiagnosis when the patient with adenomyosis and intact endometrial surface complained of vaginal bleeding.\nKeywords: Adenocarcinoma, clear cell; Endometriosis; Eestrogen receptor; Progesterone receptor; p53\nCASE REPORT\nReceived: 2011. 5.24.   Revised: 2011. 7. 5.  Accepted: 2011. 7.22.\nCorresponding author: Duk-Soo Bae, MD, PhD \nDepartment of Obstetrics and Gynecology, Samsung Medical \nCenter, Sungkyunkwan University School of Medicine, \n50 Irwon-dong, Gangnam-gu, Seoul 135-710, Korea\nTel: +82-2-3410-3511  Fax: +82-2-3410-0630\nE-mail: ds123.bae@samsung.com\nTh is is an Open Access article distributed under the terms of the Creative Commons \nAttribution Non-Commercial License (http://creativecommons.org/licenses/\nby-nc/3.0/) which permits unrestricted non-commercial use, distribution, and \nreproduction in any medium, provided the original work is properly cited.\nCopyright © 2011. Korean Society of Obstetrics and Gynecology \nKorean J Obstet Gynecol 2011;54(9):561-565\nhttp://dx.doi.org/10.5468/KJOG.2011.54.9.561\npISSN 2233-5188\n · eISSN 2233-5196\nThe malignant transformation of patients with ovarian endometri-\nosis has been reported approximately 0.7-1.0% [1]. On the other \nhand, the development of cancer from adenomyosis is a relatively \nrare occurrence. At present, only about 40 cases of malignant neo-\nplasms arising from adenomyosis have been reported in English \nliterature [2,3]. The most frequent histologic type of malignant \nneoplasm arising from adenomyosis is endometrioid adenocarci-\nnoma. However, until recently, only 2 cases of clear cell carcinoma \n(CCA) arising from adenomyosis has been reported [2,4].\nHere, we report additional one case of CCA arising from adeno-\nmyosis clinically mimicking smooth muscle tumor of myometrium.\nCase Report\nA 52-year-old postmenopausal gravida 2 and para 2 Korean \nwoman presented at the local hospital with complaints of vaginal \nbleeding and back pain. Her medical history was nonspecifi  c ex-\ncept for antihypertensive therapy and surgery for breast fi  broad-\nenoma. She had received routine gynecologic check up, and had \nknown the existence of myomas which had no change in size. She \nwas performed endometrial biopsy, abdomen and pelvis computed \ntomography (CT) and pelvic magnetic resonance imaging (MRI) \nexpecting endometrial pathology. Only a endometrial polyp was \nfound in the biopsy specimen. \nShe was referred to Samsung Medical Center with the impression \nof degenerative myoma without complete exclusion of malignancy \nby MRI. By pelvic examination, the uterus was goose-egg sized, \nand there was no bleeding from uterus. Transvaginal ultrasonogra-\nphy showed two 4 cm sized myomas with cystic change and thin \nendometrium. \nThe serum level of CA-125 was 17.7 U/mL. MRI revealed a 3.4 cm \nintramural leiomyoma in the anterior portion of uterus body and a \n\nWWW.KJOG.ORG562\nKJOG  Vol. 54, No. 9, 2011\n3.3 cm cystic degenerative mass consistent with leiomyoma in the \nposterior portion of uterine body (Fig. 1). The cystic degenerative \nmass was not involved endometrium and no tumor was recog-\nnized in other organs. \nPreoperative differential diagnoses included leiomyoma, leiomyo-\nsarcoma, endometrial carcinoma, or endometrial stromal sarcoma.\nWe performed a laparoscopic assisted vaginal hysterectomy and \nfrozen section revealed clear cell adenocacinoma. Laparoscopic \nbilateral salpingo-oophorectomy, pelvic lymphadenectomy, para-\naortic lymphadenectomy and total omentectomy were performed \nas surgical staging. There was no evidence of gross dissemination. \nMacroscopically, the resected uterus weighed 100 g (10 × 7 cm). \nCross-section of the posterior uterine wall revealed an yellow-ten \nsolid mass, measuring 4 × 3 cm in the myometrium with pushing \nthe endometrium (Fig. 2A). The mass was mainly located in the \nmyometrium that was clinically suspected as mesenchymal tumor, \nsuch as leiomyoma or leiomyosarcoma, and endometrium was \nunremarkable. Microscopically, however, the tumor was CCA (Fig. \n2B) and there was no connection between the myometrial mass \nand endometrium (Fig. 2C).\nInterestingly periphery of the myometrial tumor showed numerous \nadenomyosis, which were intimacy related with tumor (Fig. 3A), \nand we identified that CCA arising from the adenomyosis (Fig. \n3B). When we examined entirely endometrium, there was a focal \nisolated CCA in the endometrium (Fig. 3C).\nThe previous reported case with CCA arising from adenomyosis \nshowed estrogen receptor (ER), progesterone receptor  (PR) and \np53 expression in the tumor. In our patient, the clear cell adeno-\ncarcinoma stained positively for ER, but did not express PR and \np53 protein (Fig. 3D).\nBased on these fi  ndings, this case was diagnosed as being CCA, \ngrade III, arising from adenomyosis and classifi  ed as International \nFederation of Gynecology and Obstetrics stage Ib because of the \ninvasion more than half of the myometrium. Postoperatively, whole \npelvis radiotherapy (total dose of 5,040 cGy) was performed to \nprevent local recurrence because laparoscopic assisted vaginal \nhysterectomy had the possibility of tumor spillage and three cycles \nof systemic chemotherapy (paclitaxel 175 mg/m\n2\n and carboplatin \n5 AUC) were performed. \nDiscussion\nThe development of adenocarcinoma arising from adenomyosis \nFig. 1. (A) Magnetic resonance imaging shows two uterine masses. (B) One of the uterine masses have a cystic degenerative change.\nAB\n\nWWW.KJOG.ORG 563\nYoo Jeong Shin, et al. Clear cell adenocarcinoma arising from adenomyosis mimicking leiomyoma: A case report\nFig. 2. (A) Yellowish round mass in the myometrium. (B) Microscopic fi  ndings of the mass show typical morphologies of clear cell carcin oma (H&E, \n×400). (C) There is no connection between the mass and endometrium (H&E, ×100).\nA\nB\nC\nFig. 3. (A) Periphery of the myometrial tumor \nshows numerous adenomyosis (arrows), which are \nintimacy related with tumor (H&E, ×200). (B) Clear \ncell carcinoma (arrows) arising from adenomyosis \n(H&E, ×200). (C)There is a focal isolated clear cell \ncarcinoma in the endometrium (H&E, ×200). (D) \nThe tumor is negative for p53 immunohistochemis-\ntry (Immunohistochemistry, ×200).\nCD\nAB\n\nWWW.KJOG.ORG564\nKJOG  Vol. 54, No. 9, 2011\nhave been rarely reported. And it is diffi  cult to distinguish adeno-\ncarcinoma arising from adenomyosis from endometrial carcinoma \narising from eutopic endometrium extend into preexisting adeno-\nmyosis [5]. In case of adenocarcinoma arising from adenomyosis, \nthe following Sampson’s or Colman’s [6] criteria should be fulfi  lled \nto confi  rm the malignant transformation: 1) the carcinoma must \nnot be located in the endometrium and elsewhere in the pelvis; \n2) the carcinoma must be seen to arise from the epithelium of the \nadenomyosis and not to have invaded from another source; and \n3) endometrial (adenomyotic) stromal cells must be present to \nsupport the diagnosis of adenomyosis. And Jacques and Lawrence \n[7] found a number of histologic features useful in identifying \nadenocarcinoma arising from adenomyosis, including a smooth, \nround contour of surrounding myometrium, adenomyotic glands \nand endometrial-type stroma within the carcinoma foci, and the \nabsence of desmoplasia or an infl  ammatory response. Kumar and \nAnderson [8] emphasized the necessity of presence of the transi-\ntion between the benign adenomyotic endometrial glands and the \ncarcinomatous glands to prove the diagnosis of an ectopic endo-\nmetrium-derived adenocarcinoma. In our case, although there was \na focal isolated CCA in the endometrium, the diagnosis was com-\npatible with CCA arising from adenomyosis because of the tumor \nbeing mainly located in the myometrium and identifying obvious \ntransition between adenomyosis and CCA along with endometrial \nstromal cell.\nBecause of the rarity, the prognosis features of adenocarcinoma \narising from adenomyosis are not well characterized. Niwa et al. [9] \nreported that patients with p53-overexpressing tumors also lacked \nER or PR had a poor prognosis.\nIn summary, we report a case of clear cell adenocarcinoma arising \nfrom adenomyosis. Adenocarcinomas arising from adenomyosis \nare very rare and preoperative diagnosis is actually difficult. In \nsome cases, carcinomas arising in adenomyosis may be associated \nwith a delay in diagnosis, potentially resulting in a more advanced \nstage at presentation. The early diagnosis can be achieved by \nperiodic follow-up using ultrasonography to detect any change in \nadenomyosis and if there is any change, MRI can be performed \nfor further evaluation. Malignant tumor arising from adenomyosis \ncould be considered as a differential diagnosis when the patient \nwith adenomyosis and intact endometrial surface complained of \nvaginal bleeding. \nReferences\n  1. Irvin W, Pelkey T, Rice L, Andersen W. Endometrial stromal \nsarcoma of the vulva arising in extraovarian endometriosis: a \ncase report and literature review. Gynecol Oncol 1998;71:313-6.\n  2. Koshiyama M, Suzuki A, Ozawa M, Fujita K, Sakakibara A, \nKawamura M, et al. Adenocarcinomas arising from uterine \nadenomyosis: a report of four cases. Int J Gynecol Pathol \n2002;21:239-45.\n  3. Puppa G, Shozu M, Perin T, Nomura K, Gloghini A, Cam-\npagnutta E, et al. Small primary adenocarcinoma in adeno-\nmyosis with nodal metastasis: a case report. BMC Cancer \n2007;7:103.\n  4. Hirabayashi K, Yasuda M, Kajiwara H, Nakamura N, Sato S, \nNishijima Y , et al. Clear cell adenocarcinoma arising from ad-\nenomyosis. Int J Gynecol Pathol 2009;28:262-6.\n  5. Motohara K, Tashiro H, Ohtake H, Saito F , Ohba T, Katabuchi \nH. Endometrioid adenocarcinoma arising in adenomyosis: elu-\ncidation by periodic magnetic resonance imaging evaluations. \nInt J Clin Oncol 2008;13:266-70.\n  6. Colman HI, Rosenthal AH. Carcinoma developing in areas of \nadenomyosis. Obstet Gynecol 1959;14:342-8.\n  7. Jacques SM, Lawrence WD. Endometrial adenocarcinoma \nwith variable-level myometrial involvement limited to adeno-\nmyosis: a clinicopathologic study of 23 cases. Gynecol Oncol \n1990;37:401-7.\n  8. Kumar D, Anderson W. Malignancy in endometriosis interna. J \nObstet Gynaecol Br Emp 1958;65:435-7.\n  9. Niwa K, Murase T, Morishita S, Hashimoto M, Itoh N, Tamaya T. \np53 overexpression and mutation in endometrial carcinoma: \ninverted relation with estrogen and progesterone receptor \nstatus. Cancer Detect Prev 1999;23:147-54.\n\nWWW.KJOG.ORG 565\nYoo Jeong Shin, et al. Clear cell adenocarcinoma arising from adenomyosis mimicking leiomyoma: A case report\n평활근종으로 오인된 샘근육증에서 기원한 투명세포암\n성균관대학교 의과대학 산부인과학교실\n신유정, 조세은, 성창옥, 최철훈, 배덕수\n샘근육증에서 기원한 악성종양은 매우 드물게 발생한다. 지금까지 샘근육증에서 기원한 투명세포암은 단 두 사례가 보고되었다. 우리는 \n이 논문을 통해 샘근육종에서 기원한 투명세포암의 새로운 사례를 보고하고자 한다. 52세 여자 환자가 질출혈 및 허리통증을 주소로 산\n부인과에 내원하였다. 자궁내막 조직검사 결과 위축성 변화를 동반한 자궁내막 용종이 진단되었고, 질식초음파와 자기공명영상에서는 낭\n성 변화를 동반한 평활근종 소견을 보였다. CA-125의 혈중 수치는 17.7 U/mL였다. 자궁절제술을 시행하였고, 근육층의 괴사된 부위에서 \n샘근육증에서 기원한 투명세포암이 진단되었다. 종양은 주로 근육층에 위치하고 있었고, 자궁내막 간질세포를 따라 샘근육증과 투명세포\n암 사이의 세포 변화를 관찰할 수 있었다. 샘근육증을 진단받은 환자에서 자궁내막에 이상 소견이 없으면서 질출혈이 발생한 경우 샘근육\n증에서 기원한 악성종양이 감별진단으로 고려되어야 한다.\n중심단어: 투명세포암, 자궁내막증, 에스트로겐 수용체, 프로게스테론 수용체, p53","source_license":"CC0","license_restricted":false}