Primary umbilical endometriosis: Surgical case report

This was a case of a 43-year-old woman, with no history, who presented with a painful hemorrhagic umbilical swelling during the menstrual period associated with dysmenorrhea. On physical examination, we assessed an umbilical swelling of 3 cm painful irreducible on palpation ( Fig 1 -(a)). Abdominal ultrasound revealed a subcutaneous umbilical mass of non-vascularized tissue nature confirmed on Doppler, measuring 20 mm x 20 mm and therefore suggesting umbilical endometriosis. Clinical view of the endometriotic nodule with umbilical reconstruction. Assessment was followed with pelvic MRI which confirm the diagnosis of primary umbilical endometriosis. Imaging showed a multifocal endometriosis with uterine location, deep extra uterine (tubal torus, round ligaments and left utero sacral and right fallopian tube) and superficial (CUL-DE-SAC) and a parietal mass of the umbilical region ( Fig 2 ). The later mass displayed an iso signal on T1 high signal on T2, strongly enhancement after injection of contrast product, containing a cystic cell, measuring 35 × 38 mm ( Fig 2 ). Abdominal mass with iso signal on T1 and high signal on T2, strongly enhanced after injection of contrast product, containing a cystic cell, measuring 35x38 mm on MRI. The patient underwent wide local excision of the endometriotic nodule with umbilical reconstruction ( Fig 1 -(b)). Histology confirmed the diagnosis of umbilical endometriosis, revealing the presence of cutaneous and subcutaneous tissue, endometrial glandular structures lined with regular cells not presenting cytonuclear atypia and surrounded by a chorion cytogenic. Resection margins were clear. Follow up was uneventful and the patient was discharged on post operative day 2. The patient was seen two months after the surgery and found to be asymptomatic with a normal umbilicus. Verbal consent for publication purposes was obtained.

Endometriosis is a defined as metastasis of functional endometrial tissue outside of the endometrial cavity. It is estimated to affect around 190 million of reproductive-aged women worldwide according to World Health Organization. 1 Endometriosis is a benign nevertheless a chronic condition which impacts greatly the quality of life through cyclic discomfort. Catamenial local pain and swelling are the most common complaints, whereas umbilical bleeding was reported less frequently. 2 Pathogenesis of atypical endometriosis location can be explained by retrograde menstruation into the peritoneal cavity during menstruation. 3 However, the exact pathogenesis of primary endometriosis is still unclear. Migrating cell implantation occur commonly in organs or structures within the pelvis, most commonly the ovaries.4 Infrequently migration can occur in the diaphragm, lungs, and anterior abdominal wall. 4 Thus, extra-pelvic sites are rare, more specifically umbilical site which has a prevalence of 0.5–1%. 4 Presentation of endometriosis varies including abdominopelvic pain, dysmenorrhea, and/or heavy menstrual bleeding. 5 Diagnosis is imaging guided and confirmed with histology after surgical excision. Clinically, umbilical endometriosis manifests as an umbilical purple or red swelling with variable diameter, associated with cyclic pain with or without bleeding. 6 Current management of extragenital endometriosis suggest radical surgery with wide local excision. 7 We aim to report the case of umbilical endometriosis and a literature review of the different treatment modalities.

Umbilical localization of endometriosis is an extremely rare entity. Clinical diagnosis is difficult, and it can be confused with other benign and malignant lesions of the umbilicus. Surgical excision is the treatment of choice. Nonetheless therapeutic management can be structured in a case-by-case model considering the rarity of this condition and the therapeutic options available. And finally, recurrence must be anticipated with consideration of the endometriosis condition. Category: Retrospective cohort study

Umbilical localization of endometriosis is an extremely rare and challenging entity considering the limited available current data, and all possible differential diagnosis. 4 Indeed, extra-pelvic endometriosis sites are not common, especially the umbilicus representing 0.5% to 1% of all cases of extragenital endometriosis. 4 Consequently, limited data is available in literature. It usually occurs secondary to surgical scars following laparoscopic or open abdominal surgery, but very rarely presents as primary umbilical endometriosis. 8 , 9 One of the theoretical etiologies of endometriosis is shedding of endometriotic cells which are transported through the lymphatic and vascular system to the umbilicus. 10 In most cases, the clinical manifestation of umbilical endometriosis consists of pigmented, papular or nodular lesion developed at the level of the umbilical depression punctuated by the menstrual cycle. This could be associated with cyclical pain or a bleeding tendency from the umbilicus. This cyclic symptomatology makes it possible to eliminate other diagnostic hypotheses such as melanoma, nevus, umbilical hernia, abscess or lipoma. 11 , 12 In our case, UE diagnosis was suspected on MRI and confirmed on pathological study. Surgical management is currently described as gold standard. 5 Surgical management is made essentially of total resection of the umbilical mass with high caution of any tissue spillage as it can cause disease recurrence. 5 Evaluation for additional endometriosis location is preferable. 5 Laparoscopic approach is recommended as it allows better visual inspection for secondary localization of endometriosis. 2 , 5 Nonetheless, larger cases with associated incidental umbilical hernia were described to undergo open surgery. 13 Medical management corresponds to combined oral contraceptives (COCs) or progestins for management of endometriotic implants decreasing inflammatory effects, or Gonadotropin-releasing hormone for long-course treatment. 5 Moreover, combined medical therapy can optimize pain management especially in cases of differed or unwanted surgical excision. 2 Driri et al. reported a case series of 55 enrolled cases and evaluated women's Quality of life (QoL) with the Short Form-12 questionnaire (SF-12). 2 They reported indeed a predominance of surgical management of 84%. 2 However, the small number of included cases in their cases series undergoing hormonal management restricted comparison between surgical and medical therapy, nonetheless surgical therapy showed to be more promising in women's satisfaction. 2 In fact, restricted evidence is available regarding the effectiveness of hormonal therapy. 7 Finally, non-radical therapies such as thermocoagulation, are not recommended considering the high risk of disease recurrence. 13 Medical therapy was not optioned considering the patient's limited means. Successful en-bloc surgical excision including peritoneum was conducted. Umbilical endometriosis recurrence following surgical resection has been reported to be varying from 5.4% to 27%. 2 , 7 Hirata et al. suggests that recurrences following wide resection including the peritoneum, with or without umbilical reconstruction, considerably reduce recurrence, emphasizing therefore the importance of extensive resection that includes the fascia and peritoneum making surgical therapy the first choice of treatment for umbilical endometriosis. 7 Malignant transformation of the umbilical nodule has been described in literature with a reported risk of malignant transformation to be 3%. 7 , 14 From the few cases of malignant UE transformation were reported one endometrioid adenocarcinoma, one clear cell adenocarcinoma and two adenocarcinomas. In these cases, positron emission tomography (PET) is valuable in confirming diagnosis, showing abnormal fludeoxyglucose accumulation. 7