Villar's Nodule — Umbilical Endometriosis

Chia-Sui Weng, M.D., and Yuh-Cheng Yang, M.D.
N Engl J Med 2011; 364:e45May 26, 2011

A 37-year-old woman presented with a brownish tumor protruding from her umbilicus (Panel A), noting that it bled cyclically, in synchronization with her menstrual period. Her medical history was significant for dysmenorrhea and two recent laparoscopic resections of endometriomas. Physical examination was unremarkable aside from the finding of a fixed, anteroflexed uterus and adnexa. Gestrinone, an oral antigonadotropic agent, was administered for 2 months, without improvement. A reconstructed sagittal computed tomographic image showed that the umbilical mass extended to the peritoneum (Panel B, arrowhead). The image also revealed a pelvic cyst with a diameter of 5 cm and a radiologic appearance consistent with another endometrioma (Panel B, arrow). 

Although the patient did not want resection of the pelvic cyst, the umbilical tumor was excised, and histologic examination revealed the presence of endometrial glands and stroma. Endometriosis of the umbilicus, also called Villar's nodule, is a rare presentation of extrapelvic endometriosis. It is hypothesized that endometrial tissue occurs at the umbilicus by means of hematogenous or lymphatic seeding, metaplasia of urachal remnants, or direct seeding from prior laparoscopic procedures. When seen at a follow-up visit at 8 months, the patient continued to have mild dysmenorrhea, but the Villar's nodule had not recurred.

Chia-Sui Weng, M.D.
Yuh-Cheng Yang, M.D.
Mackay Memorial Hospital, Taipei, Taiwan
Umbilical endometriosis, also called Villar’s Nodule,was first described by Villar in1886. It is extremely rare, the incidence being only 0.5-0.1% of all women with extra gonadal or external endometriosis. This condition should not be mistaken for scar endometriosis occurring in a subumbilical incision scar. In the late 19th century, the term endometriosis was coined by Sampson to characterize ectopic tissue,possessing histologic architecture and function of the uterine endometrium. Endometriosis involving the subcutaneous tissues of the umbilicus must be suspected in a woman presenting with localised cyclical pain and swelling, in association with menstruation.

The most common sites for extra gonadal endometriosis are the gastrointestinal tract, especially
the rectum, colon, and the peritoneal surfaces. Ureteral or urinary bladder involvement is also known to occur and could result in cyclical pain and hematuria during menstruation. Pulmonary involvement could manifest as pneumothorax, hemothorax or hemoptysis during menstruation. External endometriosis, involving the subcutaneous tissues, has also been reported in the vicinity of a surgical scar following surgeries such as hysterectomy, hysterotomy, cesarean section, episiotomy and laparoscopy.


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