{"paper_id":"81a27511-c579-4c85-9e52-58b7659af36d","body_text":"Mohaghegh et al. \nJournal of Medical Case Reports          (2022) 16:256  \nhttps://doi.org/10.1186/s13256-022-03483-8\nCASE REPORT\nCoexistence of cutaneous endometriosis \nand ovarian endometrioma: a case report\nFatemeh Mohaghegh1, Parvaneh Hatami2*, Parvin Rajabi3 and Zeinab Aryanian2,4* \nAbstract \nBackground: Umbilical endometriosis is a rare entity accounting for 0.5–4% of cases with endometriosis.\nCase presentation: Here we report a rare case of umbilical endometriosis with concurrent ovarian endometriomas \nin a 37-year old primiparous Iranian woman.\nConclusion: This interesting coexistence reflects the importance of thorough gynecological assessment in patients \nwith cutaneous endometriosis to enable appropriate management.\nKeywords: Cutaneous endometriosis, Umbilical endometriosis, Endometrioma, Ovarian cyst\n© The Author(s) 2022. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which \npermits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the \noriginal author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or \nother third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line \nto the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory \nregulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this \nlicence, visit http:// creat iveco mmons. org/ licen ses/ by/4. 0/. The Creative Commons Public Domain Dedication waiver (http:// creat iveco \nmmons. org/ publi cdoma in/ zero/1. 0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.\nIntroduction\nEndometriosis is a common gynecologic condition \ncharacterized by the presence of endometrial tissue in \nanatomical sites other than the uterus and can lead to \nchronic pelvic pain or even infertility in women. The \nmost common sites of involvement are ovaries followed \nby the Douglas pouch and pelvic ligaments, respec -\ntively [1]. It can be rarely found in other organs such as \nskin. Umbilical endometriosis is an unusual condition \naccounting for 0.5–1% of extra pelvic endometriosis [2]. \nHere we report a case of umbilical endometriosis with \nconcurrent ovarian endometriomas.\nCase presentation\nA 37-year old primiparous Iranian woman presented to \nour dermatology clinic with a complaint of an asympto -\nmatic lesion in the umbilicus. The lesion appeared 1.5 \nyears ago and had slightly increased in size. She denied \nprevious piercing or trauma. However, she had had a \nlaparoscopic cholecystectomy with concurrent ovarian \ndermoid cystectomy about 10 years ago. She had regular \nmenstrual cycles with normal flow.\nPhysical examination revealed a well-circumscribed, \nfirm, bilobulated 20 × 22  mm2, nontender nodule on her \numbilicus (Fig.  1a). An incisional biopsy was performed \nwith differential diagnosis of adnexal tumor, umbilical \ngranuloma, and metastasis, but surprisingly, histopatho -\nlogic assessment showed the presence of a few dilated \nglands with stratified columnar epithelium in secretory \nphase (Fig.  2). The glands were surrounded by hyper -\ncellular stroma. Immunohistochemistry (IHC) staining \nshowed positivity for estrogen receptor in the nuclei of \nepithelial cells lining glandular structure endometrial-like \ncells, CD10 diffuse and intense positivity in the stroma, \nand Ki67 positivity below 1% of epithelial cell nuclei \n(Fig. 3). These findings confirmed the diagnosis of cuta -\nneous endometriosis. No signs of atypia or malignancy \nwere observed. After consultation with gynecology ser -\nvice, abdominopelvic sonography was conducted, which \nshowed an increased density at the umbilicus and also \nmultiple ovarian cysts with appearance compatible with \nendometrioma (two 15  ×  10  mm2 cysts in right ovary \nand a 35 × 48  mm2 cyst in left one).\nAfter establishing the diagnosis, total umbilec -\ntomy was suggested. However, due to the patient’s \nphobia regarding that, just the nodule was excised \nOpen Access\n*Correspondence:  p_hatami2001@yahoo.com; aryanian@sina.tums.ac.ir; \nz_aryanian@yahoo.com\n2 Autoimmune Bullous Diseases Research Center, Razi Hospital, Tehran \nUniversity of Medical Sciences, Tehran, Iran\nFull list of author information is available at the end of the article\n\nPage 2 of 3Mohaghegh et al. Journal of Medical Case Reports          (2022) 16:256 \nas completely as possible by dermatologist, and oral \nprogestin (Dienogest) was initiated. The remnants of \nthe umbilical lesion had dramatically improved after \n1 month (Fig. 1 b). Follow-up is in progress.\nDiscussion\nEndometriosis is a benign disease with incidence of \n6–10% in women of childbearing age [1 ], usually being \nseen in the pelvic area. Cutaneous endometriosis can \nappear in less than 5% of cases [2 ], most of which have a \nhistory of former surgery [3 ]. Umbilical endometriosis \nappeared in 0.5–4% of affected cases, usually presenting \nwith painful umbilical mass with periodic discharge or \nbleeding. An interesting feature of this case is that the \nlesion was totally asymptomatic. Another prominent \nfeature is the presence of bilateral ovarian endome -\ntrioma, which reveals the importance of gynecological \nassessment in patients with cutaneous endometriosis \nto identify any pelvic involvement, which is reported to \noccur in 15% of these patients [4 ].\nRegarding treatment of cutaneous endometriosis, \ncomplete excision is considered as the treatment of \nchoice with or without hormonal therapy for ameliorat -\ning the symptoms [5 – 7].\nOvaries are the most common sites of endometriosis \n[8], and ovarian endometrioma accounts for 35% of all \nbenign ovarian cysts [9 ]. Interestingly, ovarian endo -\nmetriomas are more frequent in the left versus right \novary, possibly due to anatomical asymmetry and com -\npression leading to venous congestion and hypoxia in \nthe left side, affecting release of cytokines and sex hor -\nmones [10, 11]. In our case, endometriomal cyst in left \novary was considerably larger than on the other side, \nwhich might be in favor of the above-mentioned theory.\nFig. 1 A bilubolated umbilical mass, before (a) and after (b) \ntreatment\nFig. 2 Endometrial glandular structures surrounded by scant \nhypercellular stroma. a H&E ×40, b H&E ×100\nFig. 3 Immunohistochemistry staining for estrogen receptor (×40, \n×100) (a, b) and CD10 (×40, ×100) (c, d)\n\nPage 3 of 3\nMohaghegh et al. Journal of Medical Case Reports          (2022) 16:256 \n \n•\n \nfast, convenient online submission\n •\n  \nthorough peer review by experienced researchers in your field\n• \n \nrapid publication on acceptance\n• \n \nsupport for research data, including large and complex data types\n•\n  \ngold Open Access which fosters wider collaboration and increased citations \n \nmaximum visibility for your research: over 100M website views per year •\n  At BMC, research is always in progress.\nLearn more biomedcentral.com/submissions\nReady to submit y our researc hReady to submit y our researc h  ?  Choose BMC and benefit fr om: ?  Choose BMC and benefit fr om: \nIn addition to pain, discomfort, and fertility issues, an \nincreased risk of infection, rupture, or transformation \ninto ovarian cancer [12] obligates surgical intervention \nin larger ovarian endometriomas [9]. Nonsteroidal anti -\ninflammatory drugs, gonadotropin-releasing hormone \n(GnRH) agonists, and progestins are also considered as \nmainstream therapeutic options [9].\nConclusion\nUmbilical endometriosis is a rare entity that might occur \nwith concurrent pelvic endometriosis. Hence, thorough \ngynecological assessment is necessary in such patients to \nenable proper management.\nAcknowledgements\nWe acknowledge the staff of Alzahra Dermatology Clinic, with special thanks \nto Parto Noor Aseman company (www. parnoa. ir) staff, who further observed \nand scanned stained skin sections using microvisioneer manual whole-slide \nimaging.\nAuthor contributions\nFM: visiting the patient. PH: writing drafts. PR: histopathological evaluation. ZA: \nsupervision. All listed authors participated equally in preparing the manu-\nscript. All authors read and approved the final manuscript.\nFunding\nNone.\nAvailability of data and materials\nThe data that support the findings of this study are available from the cor-\nresponding author upon reasonable request.\nDeclarations\nEthics approval and consent to participate\nEthical approval from the Medical Ethics Committee of Isfahan University of \nMedical Sciences was provided.\nConsent for publication\nWritten informed consent was obtained from the patient for publication of \nthis case report and any accompanying images. A copy of the written consent \nis available for review by the Editor-in-Chief of this journal.\nCompeting interests\nThe authors declare that they have no competing interests.\nAuthor details\n1 Department of Dermatology, Skin Diseases and Leishmaniasis Research \nCenter, Isfahan University of Medical Sciences, Isfahan, Iran. 2 Autoimmune \nBullous Diseases Research Center, Razi Hospital, Tehran University of Medical \nSciences, Tehran, Iran. 3 Department of Pathology, Isfahan University of Medical \nSciences, Isfahan, Iran. 4 Department of Dermatology, Babol University of Medi-\ncal Sciences, Babol, Iran. \nReceived: 28 September 2021   Accepted: 8 June 2022\nReferences\n 1. Giudice LC, Kao LC. Endometriosis. Lancet. 2004;364:1789–99.\n 2. Kyamidis K, Lora V, Kanitakis J. Spontaneous cutaneous umbilical endo-\nmetriosis: report of a new case with immunohistochemical study and \nliterature review. Dermatol Online J. 2011;17:5.\n 3. Choi JK, Bae HA, Sang JH, Chung SH. Postmenopausal spontaneous \numbilical endometriosis: a case report. J Menopausal Med. 2020;26(1):44–\n6. https:// doi. org/ 10. 6118/ jmm. 19016.\n 4. Gin TJ, Gin AD, Gin D, Pham A, Cahill J. Spontaneous cutaneous endome-\ntriosis of the umbilicus. Case Rep Dermatol. 2013;5:368–72.\n 5. Cucinella G, Granese R, Calagna G, Candiani M, Perino A. Laparoscopic \ntreatment of diaphragmatic endometriosis causing chronic shoulder and \narm pain. Acta Obstet Gynecol Scand. 2009;88(12):1418–9. https:// doi. \norg/ 10. 3109/ 00016 34090 33140 88.\n 6. Obata K, Ikoma N, Oomura G, Inoue Y. Clear cell adenocarcinoma arising \nfrom umbilical endometriosis. J Obstet Gynaecol Res. 2013;39(1):455–61. \nhttps:// doi. org/ 10. 1111/j. 1447- 0756. 2012. 01964.x.\n 7. Gopalan A, Sharp DS, Fine SW, et al. Urachal carcinoma: a clinicopatho-\nlogic analysis of 24 cases with outcome correlation. Am J Surg Pathol. \n2009;33(5):659–68. https:// doi. org/ 10. 1097/ pas. 0b013 e3181 9aa4ae.\n 8. 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Com-\nplement pathway is frequently altered in endometriosis and endometri-\nosis-associated ovarian cancer. Clin Cancer Res. 2014;20:6163–74. https:// \ndoi. org/ 10. 1158/ 1078- 0432. CCR- 14- 1338.\nPublisher’s Note\nSpringer Nature remains neutral with regard to jurisdictional claims in pub-\nlished maps and institutional affiliations.","source_license":"CC0","license_restricted":false}