{"paper_id":"7792df85-6e57-44b1-af9e-c5c616fd951c","body_text":"Multidisciplinary Journal of Women’s Health 2013; 2 (1): 6-86\nRecurrence of primary umbilical endometriosis:\ncase report and review of the literature\nDanilo Buca, \nMartina Leombroni, \nEleonora Falò, \nEttore Clementini, \nMariapia Santomauro, \nSerena Frondaroli,\nGrazia Camastra, \nMarco Liberati\nDepartment of Obstetrics and Gynecology, G. d'Annun-\nzio University, Chieti-Pescara, Italy\nCorrespondence to:\nEleonora Falò\nDepartment of Obstetrics and Gynecology \nG. d'Annunzio University Chieti-Pescara\nVia dei Vestini, 5\n66100 Chieti, Italy\nE-mail: falo.eleonora@libero.it\nSummary\nThe authors report the case of a 33-year-old nulli-\nparous patient with recurrence of primary sponta-\nneous cutaneous umbilical endometriosis and no\nhistory of abdominal or pelvic surgery. The litera-\nture describing similar cases is reviewed. \nPrimary endometriosis at extrapelvic sites, and um-\nbilical endometriosis in particular, is very uncom-\nmon. The diagnosis is confirmed only by histopatho-\nlogical examination, specifically hematoxylin and\neosin staining. Conservative surgical excision with\nwide margins is the recommended treatment. Adju-\nvant and neoadjuvant treatment for endometriosis is\nbased on medications which induce endometrial at-\nrophy, such as gonadotropin releasing hormone su-\nperactive analogs, or oral estrogen-progestogen for-\nmulations given continuously.\nKey words: umbelical endometriosis, macroscopic\nappearence, pathogenesis, surgical excision, Villar's\nnodule.\nIntroduction\nEndometriosis is defined as the presence of endometrial\ntissue, with its typical stromal and glandular components,\nat anatomical sites other than the uterine cavity. The\novaries are most commonly involved, followed by the\nuterine ligaments, pelvic wall, intestine, bladder and skin. \nCutaneous endometriosis of the umbilical scar, also\nknown as Villar’s nodule, is rare, especially if there is\nno history of abdominal or pelvic surgery or concomi-\ntant endometriosis at other sites.\nVillar’s nodule, which accounts for up to 5% of cases of cu-\ntaneous endometriosis, can occur as a primary lesion re-\nsulting from spontaneous metastatic implantation, or as\nsecondary endometriosis, i.e. following a iatrogenic dis-\nplacement of endometrial tissue during surgical procedures\nsuch as umbilical or inguinal hernia repair or any laparo-\nscopic surgery performed using an umbilical access (2,3).\nOn physical examination, the lesion appears as a solid\nnodule varying in color from red to blue or brown-black,\ndepending on the amount of hemorrhage and the depth\nof penetration of the ectopic endometrial tissue (1). A\ncase of recurrent primary cutaneous endometriosis of\nthe umbilical scar is here reported. \nCase report\nIn February 2013, a 33-year-old nulligravida black African\nwoman referred herself to the Gynecological Surgery Unit\nat the “Ss Annunziata” Hospital in Chieti (Italy) with a ten-\nder and discolored nodule of the umbilical skin. Charac-\nteristically, the lesion showed cyclic hematic discharge\nassociated with evident changes in size and color con-\ncomitantly with the menstrual period.\nSubjective pain was described by the patient as constant\nand not related to any particular activity, but markedly in-\ncreased by the onset of the menstrual period.\nHer past history included surgical excision of a lesion with\nidentical clinical, anatomical and histopathological fea-\ntures, performed in 2006. The nodule was excised with\nfree margins and pathology examination confirmed the le-\nsion nature as cutaneous endometriosis. The patient’s his-\ntory was negative for any other surgery on the umbilical\nskin, including cosmetic procedures such as piercings,\nand she reported no previous pelvic or abdominal surgery.\nHer history was also negative for significant medical prob-\nlems, with the exception of dysmenorrhea, and her family\nhistory was negative for significant related pathology.\nThe patient’s body mass index, blood cell count, liver\nfunction test and renal function test were all in the nor-\nmal range. Her serum CA125 was 77.2 U/ml the day\nbefore the procedure (cut-off 35 U/ml).\nA head-to-toe physical examination showed two red-\ndish-blue nodules with a solid consistency involving the\numbilical skin, moderately tender on palpation (Fig. 1).\nNo other lesions were identified on thorough inspection\nof other cutaneous regions. No pathologically enlarged\nlymph nodes were evident on palpation.\nCase Report\n© CIC Edizioni Internazionali\n\nMultidisciplinary Journal of Women’s Health 2013; 2 (1): 6-8 7\nRecurrence of primary umbilical endometriosis: case report and review of the literature\nThe nodule was excised with wide margins under local\nanesthesia and the specimen was sent for histopatho-\nlogical examination. The macroscopic report described\nan elliptical specimen of skin including two nodules, a\nlateral one with a maximum diameter of 12 mm and a\nmore central one, deeply embedded in the umbilicus,\nwith a maximum diameter of 9 mm. Neither nodule\nshowed superficial hematic scabs. Light microscopy\nwith hematoxylin and eosin (H&E) staining showed a\ntypical area of endometriosis consisting of endometrial-\ntype glands and stroma. Subsequent histological exam-\nination showed evidence of stratified endometrial\nglands and stroma.\nThe post-operative course was uneventful and the pa-\ntient was given continuous oral estroprogestins for six\nmonths to induce atrophy of other possible microscopic\nfoci of ectopic endometrium. \nDiscussion\nOverall, the estimated incidence of umbilical en-\ndometriosis is 0.5-1% (4,5), and it has a highly variable\nclinical presentation.\nCutaneous endometriosis affects patients with a mean age\nof 35-38 years (6,7) and most commonly involves scars. \nLess than 30% of cutaneous endometriosis occurs in\nthe absence of previous surgery (3-6); in such cases,\nthe site most commonly affected is the umbilicus, fol-\nlowed by the inguinal area and the abdominal wall (8). \nThe clinical features of the lesion described in different\nstudies, e.g. color, consistency and tenderness on pal-\npation, vary widely, depending on the depth of penetra-\ntion of the ectopic endometrial tissue and the amount of\nbleeding (1). Its color reportedly varies from pink to\ndark brown and black, while palpation commonly dis-\ncloses a nodule with a solid consistency and moderate\nto severe tenderness. The nodule can be single or mul-\ntilobed (9).\nMenstrual bleeding into the dermis leads to hemo-\nsiderin deposition, scarring and chronic inflammation.\nThe pathogenesis of spontaneous umbilical en-\ndometriosis is still not well understood. Several hy-\npotheses have been proposed, each one explaining\nsome specific characteristic of different clinical presen-\ntations of endometriosis. Most of the theories lay the\ndevelopment of ectopic endometrial foci either on a\nprimitive lesion of the involved site, arising from tissue\nmetaplasia or from embryonic remnants (the in situ\ntheory), or on the endometrial transplantation from i)\nretrograde menstruation; ii) iatrogenic displacement by\nsurgical procedures; iii) lymphatic or vascular benign\nmetastatic transport (the implantation theory) (10). \nIn contrast to normal, eutopic endometrial tissue, en-\ndometriotic tissue shows, in vitro, the capability, by itself,\nto produce estrogens through the aromatase cycle.\nGenetic (11, 12), hormonal (13) and autoimmune fac-\ntors (14) also play an important role in the pathogene-\nsis of endometriosis.\nThe diagnosis can be suspected on the basis of clinical\nsigns and symptoms collected through a detailed histo-\nry and physical examination, but it must always be con-\nfirmed pathologically through H&E staining. The histo-\nlogical image will typically show irregular glands lined\nby columnar epithelium among a myxoid stroma with\nextravasation of blood into the gland lumina. \nIn the event of doubt, immunocytochemistry stains such\nas vimentin and CD10 are used to confirm the pres-\nence of endometrial-type stroma around endometrial-\ntype glands (15).\nThe treatment of choice is conservative surgical exci-\nsion of the lesion, with sufficient healthy margins to pre-\nvent recurrence, with a subsequent course of medica-\ntion effective in inducing endometrial atrophy (16).\nIt is recommended that surgical excision be performed\nat the end of the menstrual period, when the nodule is\nsmaller and a smaller excision can therefore be per-\nformed (18).\nPostoperative medications to induce endometrial atro-\nphy include continuous oral contraceptives, i.e. given\nwithout the one-week cyclical suspension, or go-\nnadotropin releasing hormone superactive analogs.\nBoth medications effectively inhibit ovarian function and\ntherefore the cyclical stimulation of the endometrium\n(16-18), while the prevalently progestogenic environ-\nment induced by oral estroprogestins has a direct atro-\nphying effect on the endometrium. \nAlthough endometriosis may recur after the course of\nmedication is stopped, the prognosis is usually from\ngood to excellent, with little or no symptoms at all re-\nmaining after proper treatment. Relapses are uncommon\nif excision is performed with clean and wide margins (1).\nIn conclusion, primary endometriosis is a rare condition\nwhose diagnosis may be sometimes challenging. It can\nbe suspected clinically from the patient’s history, but\nshould be confirmed with histological examination.\nReferences\n1. Kyamidis K, Lora V, Kanitakis J. Spontaneous cutaneous um-\nbilical endometriosis: report of a new case with immuno-\nhistochemical study and literature review. Dermatol Online\nJ 2011; 17(7):5.\nFigure 1. Umbilical endometriosis that presented as a two\nreddish-blue nodules and occupied the deep part of the\numbilical circumference.\n© CIC Edizioni Internazionali\n\nMultidisciplinary Journal of Women’s Health 2013; 2 (1): 6-88\nD. Buca et al.\n2. Douglas C, Rotimi O. Extragenital endometriosis: a clinico-\npathological review of a Glasgow hospital experience with\ncase illustrations. J Obstet Gynaecol 2004; 24(7):804-808.\n3. Agarwal A, Fong YF. Cutaneous endometriosis. Singapore\nMed J 2008; 49(9):704-709.\n4. Rosina P, Pugliarello S, Colato C, Girolomoni G. En-\ndometriosis of umbilical cicatrix: case report and review of\nthe literature. Acta Dermatovenerol Croat 2008; 16(4):218-\n221.\n5. Krumbholz A, Frank U, Norgauer J, Ziemer M. Umbilical en-\ndometriosis. J Dtsch Dermatol Ges 2006; 4(3):239-241.\n6. Fernández-Aceñero MJ, Córdova S. Cutaneous en-\ndometriosis: review of 15 cases diagnosed at a single in-\nstitution. Arch Gynecol Obstet 2011; 283(5):1041-1044.\n7. Victory R, Diamond D, Johns DA. Villar's nodule: a case re-\nport and systematic literature review of endometriosis externa\nof the umbilicus. J Minim Invasive Gyneol 2007; 14(1):23-\n32.\n8. Steck WD, Helwing EB. Cutaneous endometriosis. Clin Ob-\nstet Gynecol 1996; 9(2):373-383.\n9. Weller CV. Endometriosis of the umbilicus. Am J Pathol 1935;\n11(2):281-286.\n10. van der Linden P. Theories on the pathogenesis of en-\ndometriosis. Hum Reprod 1996; 11 Suppl 3:53-65.\n11. Simpson JL, Bischoff FZ, Kamat A, Buster JE, Carson SA.\nGenetics of endometriosis. Obstet Gynecol Clin North Am\n2003; 30(1):21-40.\n12. Vignano P, Somigliana E, Vignali M, Busacca M, Blasio AM.\nGenetics of endometriosis: current status and prospects. Front\nBiosci 2007; 12:3247-3255.\n13. Sinaii N, Cleary SD, Ballweg ML, Nieman LK, Stratton P. High\nrates of autoimmune and endocrine disorders, fibromyalgia,\nchronic fatigue syndrome and atopic diseases among\nwoman with endometriosis: a survey analysis. Hum Reprod\n2002; 17(10):2715-2724.\n14. Mathur A, Peress MR, Williamson HO, Youmans CD,\nManey SA, Garvin AJ at al. Autoimmunity to endometrium\nand ovary in endometriosis. Clin Exp Immunol 1982;\n50(2):259-266.\n15. Potlog-Nahari C, Feldman AL, Stratton P, Koziol DE,\nSegars J, Merino MJ, Nieman LK. CD10 immunohisto-\nchemical staining enhances the histological detection of en-\ndometriosis. Fertil Steril 2004; 82(1):86-92.\n16. Minaidou E, Polymeris A, Vassiliou J, Kondi-Paphiti A, Karout-\nsou E, Katafygiotis P, Papaspyrou E. Primary umbilical en-\ndometriosis: case report and literature review. Clin Exp Obst\nGynecol 2012; 39(4):562-564.\n17. Chatzikokkinou P, Thorfinn J, Angelidis IK, Papa G, Trevisan\nG. Spontaneous endometriosis in an umbilical skin lesion.\nActa Dermatovenerol Alp Panonica Adriat 2009; 18(3):126-\n130.\n18. Bagade PV, Guirguis MM. Menstruating from the umbilicus\nas a rare case of primary umbilical endometriosis:a case re-\nport. J Med Case Rep 2009; 3:9326.\n© CIC Edizioni Internazionali","source_license":"CC0","license_restricted":false}