{"paper_id":"a4fae072-6b02-4e23-9d25-8972f404edbb","body_text":"Ann Case Rep, an open access journal\nISSN: 2574-7754\n1 V olume 8; Issue 01\nCase Report \nCase Report: Primary Umbilical Endometriosis \nwithout Dysmenorrhea or Dyspareunia\nSung-Tack Oh1,2*, Bum-Chae Choi1\n1Creation and Love Women’s Hospital, Gwangju, Korea\n2Chonnam University Medical School, Gwangju, Korea\n*Corresponding author: Sung-Tack Oh, Creation and Love Women’s Hospital, 409 Sungam Officetel, 62 Jukbongdaero, Seogu, \nGwangju 61932, Korea\nCitation: Oh ST, Choi BC (2023) Case Report: Primary Umbilical Endometriosis without Dysmenorrhea or Dyspareunia. Ann \nCase Report. 8: 1140. DOI:10.29011/2574-7754.101140\nReceived: 19 January 2023, Accepted: 23 January 2023, Published: 26 January 2023\nAnnals of Case Reports\nOh ST and Choi BC.  Ann Case Rep: 8: 1140 \nwww.doi.org/10.29011/2574-7754.101140\nwww.gavinpublishers.com\nAbstract \nObjective: This report presents a rare case of primary umbilical endometriosis and reviews the literature of it. \nCase report: In a 42-year-old woman, umbilical mass with cyclic swelling and tenderness on menstruation was found. It \nwas diagnosed pathologically umbilical endometriosis at small incision biopsy by general surgeon. She had no history of \npelvic or abdominal surgery except local small incision biopsy by general surgeon. There was no history of endometriosis or \nendometriosis-associated symptoms. Therefore, monthly gonadotropin-releasing hormone (GnRH) agonist therapy was done \nfor 6 months. The swelling and pain of umbilical mass were slightly decreased after this therapy. However, these symptoms \nwere reappeared 6 months later again. Therefore, we decided the surgical removal of mass. The mass and adjacent tissue of \numbilicus were much resected sparing the umbilicus under general anaesthesia. Although omphalectomy is choice of surgery, \numbilicus was saved because of her request to save umbilicus. Histologic examination confirmed the diagnosis of umbilical \nendometriosis.\nConclusion: Umbilical endometriosis are rare and difficult to recognize, but the possibility of endometriosis must be considered \nto umbilical mass with cyclic swelling and tenderness on menstruation despite the absence of previous surgery. Complete excision \nof umbilical mass with adjacent normal tissue or complete surgical excision of umbilicus with mass are highly recommended.\nKeywords: Primary Umbilical Endometriosis \nIntroduction\nEndometriosis is the presence of endometrium tissue outside \nthe uterine cavity. It is found at 10–15% of all premenopausal \nwomen and 6% of perimenopauses women [1,2]. Endometriosis \nis mainly found in the vulva, vagina, cervix, ovaries, and pelvic \nperitoneum. However, it is sometimes found at extra-genital sites \nincluding the intestinal tract, urinary tract, lungs, thoracic cage, \nsurgical scars, abdominal wall, inguinal area and umbilicus in \nlesser than 12%. Secondary umbilical endometriosis can occur \non scars of abdominal wall after gynaecologic surgeries such as \nhysterectomy, caesarean section, and laparoscopic operation [3-\n8]. However, primary umbilical endometriosis is not associated \nwith surgical procedure and the prevalence is very rare. The \nexact prevalence is still not well-known but the incidence is \nreported to be 0.5% - 1% of all extra-genital endometriosis [9]. \nPrimary umbilical endometriosis was described by Villar for the \nfirst time in 1886 [10]. When concurrent pelvic endometriosis is \npresent, possible theory of umbilical endometriosis is explained to \nhaematogenous or lymphatic spread [11]. If pelvic endometriosis \nis not present, it can be also explained to metaplasia of urachal \nremnants [11]. The diagnosis is mainly clinical by bluish purple, \nbrown umbilical mass associated with aggravation of expansion \nand pain on every menstruation. We present an uncommon case \nof spontaneous umbilical endometriosis without dysmenorrhea, \npelvic pain or dyspareunia or pelvic endometriosis.\n\nCitation: Oh ST, Choi BC (2023) Case Report: Primary Umbilical Endometriosis without Dysmenorrhea or Dyspareunia. Ann Case Report. 8: 1140. DOI:10.29011/2574-\n7754.101140\n2\nV olume 8; Issue 01\nAnn Case Rep, an open access journal\nISSN: 2574-7754\nPresentation of case\nA 42-year-old multiparous woman with one son and one \ndaughter suffering from a slowly growing swelling in the umbilicus \nthat she noticed 3 years ago, and she also had umbilical tenderness \nduring only menstruation for the last 2 years. She went to general \nsurgeon, and it was diagnosed to umbilical endometriosis by small \nincision biopsy. On physical examination, a slightly tender nodule \nof 10 × 15 mm in size was palpated in the umbilicus without \ncolour change. She had no history of abdominal operations \nexcept small incision biopsy of umbilical mass, and she did not \nreceive any medication. She did not suffer from any symptom of \npelvic endometriosis such as dysmenorrhea, abdominal pain or \ndyspareunia. She had regular menstrual cycles and did not use \noral contraceptive. The concomitant pelvic endometriosis finding \nwas not found on gynaecological physical examination and \nultrasonography. At transvaginal US, the uterus and both ovaries \nappeared normal. Preoperative CA125 level and HE4 level were \nwithin normal range. At the first, monthly gonadotropin - releasing \nhormone (GnRH) agonist, therapy was done for 6 months. The \nswelling and pain of umbilical mass were slightly decreased \nafter this therapy. However, these symptoms were reappeared 6 \nmonths later again. Therefore, we decided the surgical removal \nof mass. The umbilical mass with normal adjacent tissue were \nremoved under general anaesthesia [Figure 1 and Figure 2]. \nAlthough omphalectomy is choice of surgery, umbilicus was \nsaved because of her request to save umbilicus. Concomitant \ndiagnostic laparoscopy with 5mm telescope via umbilicus was \nalso performed to carefully inspect the abdominopelvic cavity \nand to exclude any coexisting pelvic endometriosis lesion. There \nwas no endometriosis lesion in abdominal cavity. Histological \nexamination confirmed the diagnosis of umbilical endometriosis. \nIn the histopathological examination of resected tissues, a well-\ncircumscribed endometriosis focus was identified with dilated \nglands localized with stratified squamous epithelium [Figure 3]. \nFigure 1: Operative findings of this case.\n \nFigure 2: Removed tissue.\nFigure 3: Pathological findings of removed tissue.\nDiscussion\nEndometriosis is classically defined as an estrogen-dependent \ndisorder, with the presence of endometrial tissue outside of the \nuterus in lesions of varying sizes and appearance. Endometriosis \ncan be broadly classified as pelvic and extra-pelvic endometriosis. \nUmbilical endometriosis is a form of extra-pelvic endometriosis. \nThe etiology of endometriosis is still debated, there are several \ntheories. The “hypothesis of migratory pathogenesis” is the \nmost widely accepted theory. According to main this hypothesis \nby Sampson, endometriosis was developed by retrograde \nmenstruation thorough the fallopian tube to the pelvis. However, \nthere are other theories such as caulomic metaplasia, lymphatic \nspread or haematogenous spread. The hypothesis of origin of \nextra-genital endometriosis is explained by them, endometrial \ntissues enter the uterine venous circulation and can then reach the \nbrain, nasal mucosa, spine or other distant sites as endometriosis \n[12,13]. In metaplastic theory, the peritoneal mesothelial cells \nof caulomic origin change into endometrial cells by metaplastic \ntransformation and it can be explained the endometriosis in the \nbladder and prostate of male [14]. The umbilical endometriosis \nwith pelvic endometriosis can be explained by the theory of \nlymphatic or teratogenic transplantation. Secondary umbilical \nendometriosis may occur by iatrogenic spread of endometrial \n\nCitation: Oh ST, Choi BC (2023) Case Report: Primary Umbilical Endometriosis without Dysmenorrhea or Dyspareunia. Ann Case Report. 8: 1140. DOI:10.29011/2574-\n7754.101140\n3\nV olume 8; Issue 01\nAnn Case Rep, an open access journal\nISSN: 2574-7754\ncells after operations such as caesarean section and laparoscopy. \nHowever, primary spontaneous umbilical endometriosis without \npelvic endometriosis is not explained by this theory. It can be \nexplained by the metaplasia of urachus residues. The possibility \nof endometriosis must be considered during the evaluation of \nan umbilical mass despite the absence of previous surgery, and \nespecially it is expanded and tender at only menstruation. Villar first \nreported primary spontaneous umbilical endometriosis in 1886. \nThe prevalence is known for 0.5-1% of all cases of extra-genital \nendometriosis [15-17]. In primary umbilical endometriosis, there \nis an umbilical nodule, which causes periodic pain and may have \nbluish colour change at menstrual period. Sometimes, there may be \na constant pain rather than periodic pain. In differential diagnosis \nof umbilical nodules, benign diseases such as haemangioma, \numbilical hernia, sebaceous cyst, granuloma, lipoma, abscess, \nkeloid, and urachus anomaly, and should always be considered [17]. \nThe treatment of umbilical endometriosis has not been certain due \nto limited number of cases. The medical treatment to GnRH agonist \nhas not provided sufficient cure results. However, some studies \nreported success in the reduced size of nodule and improvement \nof symptoms using this medical therapy [18]. However, almost of \npatients required surgical treatment [19]. The choice of surgery \nis total umbilical resection with or without repair of underneath \nfascia and peritoneum, but it can be performed local excision of \nendometrial nodule with preserving umbilicus. Total resection \nof umbilicus is mostly preferred because of recurrence [19, 20]. \nLocal excision of endometrial lesion also should be performed \nby achieving adequate edge of the surrounding normal tissue in \norder to avoid local recurrence [18]. It is important with complete \nexcision to perform the diagnostic laparoscopy in order to identify \npossible pelvic endometriosis lesion and to investigate peritoneal \nendometriosis. The successive histology is highly recommended \nfor obtaining a definitive diagnosis and optimal treatment. \nConclusion\nPrimary umbilical endometriosis is a rare disease with \na limited number of cases reported in the literature. Primary \numbilical endometriosis should be differentially diagnosed without \nany previous surgical history, if the women present umbilical mass \nwith painful and expanded cyclically on menstruation. Diagnosis \nis carried out by US, MRI or needle biopsy with the diagnostic \nlaparoscopy in order to identify possible pelvic endometriosis \nlesion. The successive histology is highly recommended for \nobtaining a definitive diagnosis. Complete surgical excision of \numbilicus with mass is the treatment of choice. If local excision \nonly possible due to various reasons, local excision of endometrial \nlesion should be performed by achieving adequate edge of the \nsurrounding normal tissue in order to avoid local recurrence\nConflicts of interest: The authors declare that they have no \nconflicts of interest.\nReferences\n1. Spaziani E, Picchio M, Di Filippo A, De Cristofano C, Ceci F, et al \n(2009) Spontaneous umbilical endometriosis: a case report with one-\nyear follow-up. 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