Primary endometriosis of umbilical location

Abstract

Abstract: 

Endometriosis is defined as the presence of functioning endometrial tissue outside the uterine cavity, usually within the pelvis. Exceptionally, secondary extrapelvic involvement is seen involving the skin, related to scars from previous surgeries in patients with a proven pelvic endometriosis.

Primary extrapelvic endometriosis (without pelvic involvement) is exceptional and its prevalence is unknown. There are just a few case reports of perianal, inguinal or other regions involvement. The age of presentation of extrapelvic cases ranges between 30 and 40 years, in contrast to cases with pelvic involvement, between 25 and 30 years.

We present a 35-year-old woman, nulliparous, with no history of previous abdominal surgeries or known pelvic enedometriosis, who consulted in January 2013 for pain in the abdominal wall and umbilical bleeding, coinciding with her last three menstruations. The physical examination reveals a deep retroumbilical induration that is tender on palpation.

The ultrasound of the umbilical region reported: three supraponeurotic nodules, the largest 1 cm in diameter, with clear borders, with posterior acoustic shadow. Inside, mixed image with septa and and finely echogenic (bloody content?), with increased vascularization on Doppler examination. The gynecological ultrasound reported normal internal genitalia, with no signs of endometriosis. Two differential diagnoses arose: cutaneous inclusion cysts or primary umbilical endometriosis. Surgical resection is performed in February 2013. Microscopic examination confirmed the diagnosis of primary umbilical endometriosis. The patient had a daughter by cesarean section in 2016. During the procedure the pelvic cavity was examined and was completely free from of endometriosis.

Extra-pelvic primary endometriosis is exceptional. The ectopic endometrial tissue response to hormonal stimulation explains the pain and the bleeding in the umbilical area, coinciding with menstrual periods. Clinical findings and imaging studies supported the presumptive diagnosis. The patient was successfully treated with surgery. The histopathology confirmed the diagnosis. The patient outcome was satisfactory.