{"paper_id":"dda3a081-c0f5-439c-b55b-78bccf215bae","body_text":"IBIMA Publishing \nInternational Journal of Case Reports in Medicine \n http://www.ibimapublishing.com/journals/IJCRM/ijcr m.html \nVol. 2013 (2013), Article ID 994933, 4 pages \nDOI: 10.5171/2013.994933 \n \n_____________ \n \nCite this Article as : Arianna Pacchiarotti, Giusi Natalia Milazzo, Vinc enzo Gentile, Paola Frati, Chiara Assorgi, \nDonatella Caserta and Massimo Moscarini (2013), “Co ncurrent Spontaneous Umbilical and Abdominal Wall \nEndometriosis Retracting the Surrounding Cutis,” \n International Journal of Case Reports in Medicine, Vol. 2013 \n(2013), Article ID 994933, DOI: 10.5171/2013. 994933 \nCase Report \nConcurrent Spontaneous Umbilical and \nAbdominal Wall Endometriosis Retracting the \nSurrounding Cutis \n \nArianna Pacchiarotti 1, Giusi Natalia Milazzo 1, Vincenzo Gentile 1, Paola Frati 2, Chiara \nAssorgi 1, Donatella Caserta 1 and Massimo Moscarini 1 \n \n1Department of gynecological obstetric and urological sciences, Faculty of Medicine and Dentistry, \n‘Sapienza’ University of Rome, Sant’Andrea Hospital , Rome, Italy \n \n2Department of anatomical, histological, forensic medicine and musculoskeletal sciences, “Sapienza” \nUniversity of Rome, Sant’Andrea Hospital, Rome, Italy \n \nReceived 28 February 2013; Accepted 28 March2013; Published 20 May 2013 \n \nAcademic Editor: Warren G. Foster \n \nCopyright © 2013 Arianna Pacchiarotti, Giusi Natalia  Milazzo, Vincenzo Gentile, Paola Frati, Chiara \nAssorgi, Donatella Caserta and Massimo Moscarini. Di stributed under Creative Commons CC-BY 3.0 \n \nAbstract \n \nEndometriosis consists of extra-uterine functional endometrial tissue. It is usually located in the \npelvis, but it can also be found in other sites. Cu taneous endometriosis is a rare condition and it \ndevelops in the most of cases above gynecological o r obstetric scars, although it may also appear \nspontaneously. We present a 39-year-old woman with umbilical and abdominal dermal nodules \nretracting the surrounding cutis as a clinically ch aracteristic form of spontaneous cutaneous \nendometriosis. The patient had no signs and symptom s of pelvic endometriosis. The \nhistopathological and immunohistochemical examinati ons confirmed the clinical diagnosis. \n \nKeywords : Umbilical endometriosis, cutaneous endometriosis,  spontaneous extrapelvic \nendometriosis. \n \nIntroduction  \n \nEndometriosis is a common gynecological \ndisease, defined as the presence of functional \nendometrial tissue outside the uterine cavity \n(Burney, 2012). Many etiological hypothesis \nhave followed one another over the years, \nfrom the first theory of retrograde \nmenstruation proposed by Sampson in 1927 \n(Sampson, 1927) to the recent theories \ninvolving a stem cell origin (Pacchiarotti, \n2011). Endometriosis generally involves the \novary and the pelvic peritoneum, but in 9-\n12% of cases it reaches extragonadal sites \n(Donnez, 2004), such as bowel, bladder, \nlungs (Kodandapani, 2011) and nerves \n(Pacchiarotti, 2013). Cutaneous \nendometriosis represents less than 5.5% of \nall cases of endometriosis (Din, 2013) and \numbilical endometriosis 0.5-1% of \nextragonadal endometriosis (Kodandapani, \n2011). Cutaneous endometriosis is mostly \nassociated with surgical scars (60% of cases), \nless frequently it occurs spontaneously \n \n\nInternational Journal of Case Reports in Medicine                                                                                               2 \n \n \n \n \n_______________  \n \nArianna Pacchiarotti, Giusi Natalia Milazzo, Vincenzo Gentile, Paola Frati, Chiara Assorgi, Donatella Caserta and \nMassimo Moscarini (2013), International Journal of Case Reports in Medicine, DOI: 10.5171/2013.  994933 \n(Singh, 2012). Clinical diagnosis can be \ndifficult due to its rarity and the lack of \npathognomonic signs except for its changes \nduring the menstrual cycle. Correct pre-\noperative diagnosis is attained in \napproximately 25% of cases (Din, 2013). \n \nCase Report \n \nA 39-year-old woman reported a blue \nswelling at the navel and two subcutaneous \nnodules with bluish transparency on the \nsuprapubic line.  She described that swellings \nchanged during menstruation. Umbilical \nnodule showed a diameter of about 1.5 cm; it \nwas soft, but not reducible, and dark . Both \nlumps on the pubic line appeared rather \ndeeper, irreducible, in a bluish transparency \nand they formed an area of skin retraction \nwhich joined the two nodules in an \nhorizontal line (Fig. 1). The patient had no \nprevious abdomen surgery neither typical \nendometriosis symptoms, such as \ndysmenorrhea or dyspareunia. Abdominal \nwall ultrasound (US) of the nodules showed \nwell defined anechoic areas (Fig. 2).  \nSuspecting endometriosis, we performed US \ntransvaginal examination, which showed no \nsigns of the disease. Because of pain due to \nthe umbilical lesion, we decided to remove it \nsurgically.  The umbilical nodule was sent for \nhistological examination, which confirmed \nthe presence of a dermal endometriosis (Fig. \n3) and immunohistochemical staining for \nestrogen and progesterone receptors was \npositive.  \n \nDiscussion \n \nThis is a rare case of concomitant \nspontaneous umbilical and abdominal wall \nendometriosis. The most common site of \nspontaneous endometriosis is the umbilicus, \nfollowed by the inguinal area and the \nabdominal wall (Kyamidis, 2011). Cutaneous \nendometriosis could be suspected in women \nof reproductive age when there is a palpable \nabdominal bluish nodule, characterized by \ncyclic pain and swelling. In our case also \nabdominal skin retraction, due to nodules’ \ncyclical bleedings and subsequent fibrosis, \nmay guide the diagnosis. Non specific chronic \nsuppuration (Din, 2013) and bleeding \n(Bagade, 2009) may be another clinical \nmanifestation of umbilical endometriosis. A \nhistory of pelvic pain and sterility may \ncorroborate the hypothesis of endometriosis \n(Kyamidis, 2011).The localization on a \nsurgical scar may be suspicious of a \nsecondary endometriosis (Fernàndez \nVozmediano, 2010). Abdominal wall US is \nuseful to demonstrate the cystic nature of the \nlesion and the MRI and CT can help to assess \nthe extent of the disease and differential \ndiagnosis with hernias (Din, 2013). The \ndifferential diagnosis should include lipoma, \ndermoid cyst, haemangioma, keloid, hernia, \nabscess, pyogenic or foreign granuloma, \nembryological rests, irreducible hernia, \ninclusion cyst, metastatic tumors from intra-\nabdominal malignancy and melanoma (Din, \n2013; Kyamidis, 2011; Singh, 2012).  Fine \nneedle aspiration cytology may be conducted \n(Fernandes, 2011) even if histology is the \nbest diagnostic tool, supported by  \nimmunohistochemical analysis of estrogen \nand progesterone receptors when the \nexcessive fibrosis hides field (Kyamidis, \n2011).The complete excision of the lesion, \nunder local or loco-regional anesthesia, is the \ntreatment of choice and usually curative. \nHormonal therapies are insufficient as sole \ntreatments (Kyamidis, 2011). These latter \nmay be an alternative treatment for little \nendometriomas or may be used before \nsurgery to reduce the size of lesions \n(Kyamidis, 2011). Local recurrence after \nadequate surgical excision is rare. Malignant \ntransformation has been described (Chene, \n2007). Recognizing cutaneous endometriosis \nis very important for a prompt \nendometriosis’ diagnosis, which is generally \ngained with a 6.7-year delay (Burney, 2012). \nAs endometriosis is a progressive disease, \ndelaying diagnosis and treatment would \nincrease the risk of severe pain, distortion of \nthe pelvic anatomy and sterility. \n \n\n3                                                                                    International Journal of Case Reports in Medicine \n \n \n \n_______________  \n \nArianna Pacchiarotti, Giusi Natalia Milazzo, Vincenzo Gentile, Paola Frati, Chiara Assorgi, Donatella Caserta and \nMassimo Moscarini (2013), International Journal of Case Reports in Medicine, DOI: 10.5171/2013.  994933 \n \n \n \nFigure 1:  Clinical Appearance of the Lesions before the Surgical Treatment \n \n \n \nFigure 2: US Image of One Dermal Nodule of the Lowe r Abdominal Wall (1.52x0.92 Mm) \n \n \nFigure 3: Histological Examination \n \n \n \n \n \n \n \n \n \n \n \n\nInternational Journal of Case Reports in Medicine                                                                                               4 \n \n \n \n \n_______________  \n \nArianna Pacchiarotti, Giusi Natalia Milazzo, Vincenzo Gentile, Paola Frati, Chiara Assorgi, Donatella Caserta and \nMassimo Moscarini (2013), International Journal of Case Reports in Medicine, DOI: 10.5171/2013.  994933 \nReferences \n \nBagade, P. V. & Guirguis, M. M. (2009). \n\"Menstruating from the Umbilicus as a Rare \nCase of Primary Umbilical Endometriosis: A \nCase Report,\" Journal of Medical Case Reports , \n3: 9326. \n \nBurney, R. O. & Giudice, L. C. (2012). \n\"Pathogenesis and Pathophysiology of \nEndometriosis,\" Fertility and Sterility , 98 (3): \n54-9. \n \nChene, G., Darcha, C., Dechelotte, P., Mage, G. \n& Canis, M. (2007). \"Malignant Degeneration \nof Perineal Endometriosis in Episiotomy \nScar, Case Report and Review of the \nLiterature,\" International Journal of \nGynecological Cancer , 175 (3) : 709-14. \n \nDin, A. H., Verjee, L. S. & Griffiths, M. A. \n(2012). \"Cutaneous Endometriosis: A Plastic \nSurgery Perspective,\" Journal of Plastic, \nReconstructive & Aesthetic Surgery , 66 (1) : \n129-30 \n \nDonnez, J. & Van Langendonckt, A. (2004). \n\"Typical and Subtle Atypical Presentations of \nEndometriosis,\" Current Opinion in Obstetrics \nand Gynecology , 16(5):431-437. \n \nFernandes, H., Marla, N. J., Pailoor, K. & Kini, \nR. (2011). \"Primary Umbilical Endometriosis \n- Diagnosis by Fine Needle Aspiration,\"  \nJournal of Cytology , 28 (4) : 214-6. \n \nFernández Vozmediano, J. M., Armario Hita, J. \nC. & Cuevas Santos, J. (2010). \"Cutaneous \nEndometriosis,\" International Journal of \nDermatology , 49 (1) : 1410-2. \nKodandapani, S., Pai, M. V. & Mathew, M. \n(2011). \"Umbilical Laparoscopic Scar \nEndometriosis,\" Journal of Human \nReproductive Sciences , 4 (3) : 150–152. \n \nKyamidis, K., Lora, V. & Kanitakis, J. (2011). \n\"Spontaneous Cutaneous Umbilical \nEndometriosis: Report of a New Case with \nImmunohistochemical Study and Literature \nReview,\" Dermatology Online Journal , 17 (7) : \n5. \n \nPacchiarotti, A., Caserta, D., Sbracia, M. & \nMoscarini, M. (2011). \"Expression of Oct-4 \nand c-kit Antigens in Endometriosis,\" Fertility \nand Sterility , 95 (3) : 1171-3. \n \nPacchiarotti, A., Milazzo, G. N., Biasiotta, A., \nTruini, A., Antonini, G., Frati, P., Gentile, V., \nCaserta, D. & Moscarini M. (2013). \"Pain in \nthe Upper Anterior-Lateral Part of the Thigh \nin Women Affected by Endometriosis: Study \nof Sensitive Neuropathy,\" Fertility and \nSterility  [epub ahead of print] \n \nSampson, J. A. (1927). 'Peritoneal \nEndometriosis Due to Menstrual \nDissemination of Endometrial Tissue into the \nPeritoneal Cavity,' American Journal of \nObstetrics and Gynecology , 14:442–69. \n \nSingh, A. (2012). \"Umbilical Endometriosis \nMimicking as Papilloma to General Surgeons: \nA Case Report,\" Australasian Medical Journal , \n5 (5) : 272-4.","source_license":"CC0","license_restricted":false}