Abstract

Study Objective Extra-genital endometriosis is rare, with umbilical endometriosis accounting for 0.4-4% of cases. Primary umbilical endometriosis (PUE) is even more uncommon and is not associated with prior surgery. The pathogenesis of this disease is unknown but may be secondary to hematogenous/lymphatic spread or coelomic metaplasia. Here we describe the presentation, diagnosis, and management of a case of PUE presenting along with a 19cm multi-fibroid uterus. Design Case report/literature review. Setting Academic affiliated gynecology practice. Patients or Participants 41yo G0 presented with a 2 year history of increasing pain at her umbilicus associated with odor. Symptoms progressed to cyclic bleeding during menses and gradual disfiguration of umbilicus. Biopsy revealed normal benign tissue. Patient also complained of heavy menstrual bleeding. She had no previous surgical history and medical history was significant for endometrial hyperplasia treated with progesterone. Exam revealed a brown firm, tender, multinodular mass measuring 2cm associated with the umbilicus. MRI showed a T1 hypointense contrast-enhancing 2.4 × 2.1cm mass of the umbilicus extending into the subcutaneous tissue as well as a 19 × 13 × 19cm multi-fibroid uterus. Interventions The umbilicus and margin of subcutaneous tissue were excised by general surgery. Laparoscopic survey did not identify pelvic endometriosis. Robotic assisted hysterectomy was performed with tissue extraction through the umbilical defect. Fascia was repaired with 0 PDS and incision was closed to create a cosmetic umbilical appearance. Measurements and Main Results Umbilical endometriosis was confirmed on pathology. The uterine tissue weighed 1444 grams with benign leiomyoma and secretory endometrium. There was no pelvic endometriosis. There were no perioperative complications. Post-operatively she had relief of umbilical pain and bleeding. Conclusion PUE is a rare condition that should be considered in premenopausal women presenting with painful masses of the umbilicus especially if associated with bleeding at time of menses. Primary umbilical endometriosis can be successfully treated with surgical excision with favorable cosmetic outcome and relief of symptoms.

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