Arch Gynecol Obstet DOI 10.1007/s00404-014-3291-8

Case Report

Primary umbilical endometriosis: a case report and review of literature Anuradha Ghosh · Sachchidananda Das 

Received: 27 February 2014 / Accepted: 21 May 2014 © Springer-Verlag Berlin Heidelberg 2014

Introduction Endometriosis is defined as presence of endometrial glands and stroma abnormally located outside the uterine cavity. It affects 10–15 % of women of reproductive age and is one of the most important causes of infertility [1, 2]. It may be genital or extragenital. Extragenital endometriosis is less common, but has been described in almost all regions of female body including bowel, bladder, lungs, brain, umbilicus, and surgical scars [3]. Umbilical endometriosis is also known as Villar’s nodule, named after the physician who first described the disease in 1886 [4]. It represents 0.5–1 % of all cases of extragenital endometriosis. It usually occurs secondary to surgical scars [5, 6]. Primary umbilical endometriosis is, however, very rare [4, 7–9].

Case history A 33-year-old woman P1+0 presented with an umbilical nodule of 8 months duration with cyclical pain and swelling coinciding with her period, both symptoms being remitted when the periods were over. The nodule increased in size gradually. She had no other symptoms, like

A. Ghosh  Department of Gynecology and Obstetrics, College of Medicine and Sagore Dutta Hospital, Kolkata, India A. Ghosh (*)  35C, Radha Madhab Dutta Garden Lane, Kolkata 700010, India e-mail: [email protected] S. Das  Park Clinic, Kolkata, India

dysmenorrhoea, menorrhagia, dyspareunia or pelvic pain. She had regular periods with average flow. She had a son aged 5 years delivered vaginally. She had no past history of any surgery. On examination, there was a brown coloured, tender, firm, irreducible nodule of about 3 cm in diameter in the centre of a widened umbilicus. The patient was asked to return with the onset of menstruation when the nodule appeared to be more tender. Ultrasonography of abdomen revealed no intra-abdominal abnormality. Provisional diagnosis was umbilical endometriosis. Excisional biopsy was planned for cure and confirmation of the diagnosis. Under general anaesthesia, a circular incision was made around the umbilical mass leaving 5 mm of the skin attached to the mass. The incision was extended transversely on each side for 1 cm at 3 and 9 O’clock positions. The mass was dissected from skin, subcutaneous tissue and rectus sheath keeping the dissection well outside the mass. The peritoneum was adherent to the mass and a circular piece of adherent peritoneum was removed along with the mass. There was no further extension inside the abdomen. The urachus was ligated, peritoneum was closed, and rectus sheath was repaired with interrupted polypropylene sutures. The transverse transumbilical incision line was closed with absorbable sutures. A few gauze pieces were placed over the umbilicus and a pressure dressing was applied to make it a normal looking inverted umbilicus. In fact when the dressing was removed after 5 days, a well inverted umbilicus was there with nearly no visible scar beyond it. The diagnosis of endometriosis was confirmed histopathologically as the features revealed the presence of endometrial glands and stroma. Postoperative period was uneventful. The patient was followed up for 2 years, and there was no recurrence.

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Discussion The pathogenesis of endometriosis is difficult to understand. The proposed theories are retrograde menstruation [10], the presence of mullerian remnants and differentiation of embryonic coelomic mesothelium into endometrial tissue. Haematogenous or lymphatic dissemination of endometrial cells has also been proposed [7, 11]. Umbilical endometriosis has been reported to be around 0.4–4 % of all patients with endometriosis and accounts for up to 30–40 % cases of cutaneous endometriosis [12, 13]. The occurrence of secondary umbilical endometriosis can be explained by iatrogenic dissemination of endometrial cells during different surgical procedures [12, 13]. In case of primary umbilical endometriosis, it may happen that umbilicus acts as a physiological scar with predilection for endometrial tissue [14]. Umbilical endometriosis is commonly found in patients of reproductive age group. In the review of Victory and coworkers [12], the mean age of diagnosis was 37.7 years, with the youngest being 23 years. Typical symptoms are cyclical swelling, pain and bleeding from umbilicus concomitant with the menstrual bleeding [15]. However, there are case reports [8, 9] like ours which did not present with umbilical bleeding. Cyclical pain and increase in size in the umbilical nodule were the main presenting symptoms in them. The nodule may be flesh coloured [16], brownish [17], dark bluish [18]. It may resemble pigmented tumour [8]. They range in size from 0.5 to 3 cm, but larger masses have been described [15]. Lesions are usually adherent to deep layers and do not increase in size during valsalva manoeuvre [9]. Diagnosis is primarily clinical. USG may be used to determine the extent and echogenicity and for exclusion of pelvic endometriosis. MRI and preoperative laparoscopy may be helpful to exclude pelvic endometriosis [7]. As there was no symptom or sign suggestive of pelvic endometriosis, laparoscopy was not considered essential in our case. Differential diagnosis, include pyogenic granuloma, hernia, residual embryonic tissue, primary or metastatic adenocarcinoma (Sister Joseph’s nodule), nodular melanoma, lipoma, abscess, cyst and cutaneous endosalpingiosis [7]. Umbilical endometriosis should be taken into consideration in the differential diagnosis of umbilical nodule even in young nulliparous female [4]. Surgical excision with wide healthy margin of tissue is recommended [4]. The reasons are 1. Accurate histopathological diagnosis is possible and malignancy can be excluded [16, 17]. 2. Malignant transformation of endometriotic lesions may occur [11, 13, 17, 18].

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Arch Gynecol Obstet

3. In early surgery due to the small size of the lesion total umbilical excision may not be necessary [4]. Medical treatment (progesterone, danazol, norethisterone, and GnRh analogue) may be of some help in reducing the size if the umbilical lesion is known to be coexistent with pelvic endometriosis [2, 7, 12]. In case of local excision follow up is vital at least 6 monthly preferably for a period of 2 years and the patient should also be fully informed about the risk of recurrence [4].

Conclusion A primary or spontaneous umbilical endometriosis is rare. Umbilical endometriosis should be suspected in a case of umbilical nodule even in the absence of any clinical features of pelvic endometriosis or history of previous surgery. Diagnosis is mainly clinical and histopathological. Surgery is the mainstay of treatment. Early diagnosis is essential to preserve the umbilicus and to avoid extensive abdominal wall excision. Patient should be counselled about risk of local recurrence. Conflict of interest None.

References 1. Spaziani E, Picchio M, Di Filippo A, De Cristofano C, Ceci F, Stagnitti F (2009) Spontaneous umbilical endometriosis: a case report with one-year follow-up. Clin Exp Obstet Gynecol 36:263–264 2. Rosina P, Pugliarello S, Colato C, Girolomoni G (2008) Endometriosis of umbilical cicatrix: case report and review of the literature. Acta Dermatovenerol Croat 16:218–221 3. Markham SM, Carpenter SE, Rock JA (1989) Extrapelvic endometriosis. Obstet Gynecol Clin North Am 16:193–219 4. Fancellu A, Pinna A, Manca A, Capobianco G, Porcu A (2013) Primary umbilical endometriosis. Case report and discussion on management options. Int J Surg Case Rep 4(12):1145–1148 5. Goldberg JM, Bedaiwy MA (2007) Recurrent umbilical endometriosis after laparoscopic treatment of minimal pelvic endometriosis: a case report. J Reprod Med 52(6):551–552 6. Michowitz M, Baratz M, Stavorovsky M (1983) Endometriosis of the umbilicus. Dermatologica 167(6):326–330 7. Bagade PV, Guirguis MM (2009) Menstruating from the umbilicus as a rare case of primary umbilical endometriosis: a case report. J Med Case Rep 10(3):9326 8. Chatzikokkinou P, Thorfinn J, Angelidis IK, Papa G, Trevisan G (2009) Spontaneous endometriosis in an umbilical skin lesion. Acta Dermatovenerol Alp Panonica Adriat 18(3):126–130 9. Dessy LA, Buccheri EM, Chiummariello S, Gagliardi DN, Onesti MG (2008) Umbilical endometriosis, our experience. In Vivo 22(6):811–815 10. Liu DT, Hitchcock A (1986) Endometriosis: its association with retrograde menstruation, dysmenorrhoea and tubal pathology. Br J Obstet Gynaecol 93:859–862

Arch Gynecol Obstet 11. Mechsner S, Bartley J, Infanger M, Loddenkemper C, Herbel J, Ebert AD (2009) Clinical management and immunohistochemical analysis of umbilical endometriosis. Arch Gynecol Obstet 280(2):235–242 12. Victory R, Diamond MP, Johns DA (2007) Villar’s nodule: a case report and systematic literature review of endometriosis externa of the umbilicus. J Minim Invasive Gynecol 14(1):23–32 13. Kyamidis K, Lora V, Kanitakis J (2011) Spontaneous cutaneous umbilical endometriosis: report of a new case with immunohistochemical study and literature review. Dermatol Online J 17(7):5 14. Yu CY, Perez-Reyes M, Brown JJ, Borrello JA (1994) MR appearance of umbilical endometriosis. J Comput Assist Tomogr 18(2):269–271

15. Latcher JW (1953) Endometriosis of the umbilicus. Am J Obstet Gynecol 66(1):161–168 16. Khaled A, Hammami H, Fazaa B, Zermani R, Ben Jilani S, Kamoun MR (2008) Primary umbilical endometriosis: a rare variant of extragenital endometriosis. Pathologica 100(6):473–475 17. Obata K, Ikoma N, Oomura G, Inoue Y (2013) Clear cell adenocarcinoma arising from umbilical endometriosis. J Obstet Gynaecol Res 39:455–461 18. Elm MK, Twede JV, Turiansky GW (2008) Primary cutaneous endometriosis of the umbilicus: a case report. Cutis 81:124–126

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Primary umbilical endometriosis: a case report and review of literature.

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