Accessory and Cavitated Uterine Mass: Is It a Müllerian-Duct Anomaly?

Abstract

Background:

Dysmenorrhea can be primary or secondary. The causes of secondary dysmenorrhea are uterine and vaginal anomalies, menstrual outflow obstructions, endometriosis, adenomyosis and uterine myomas. This article reports on a patient who had a rare but new entity—an accessory and cavitatory uterine mass (ACUM). A review of literature is also provided. The review was performed through a Medline^® for accessory cavitated uterine malformations, juvenile cystic adenomyomas, and uterine-like masses.

Case:

A 30-year-old female presented with chief complaints of chronic pelvic pain and severe dysmenorrhea. She was diagnosed as having rare but new entity known as an accessory and cavitatory uterine mass (ACUM). The mass was removed surgically.

Results:

Case: Histopathology testing of the excised mass revealed that it had a cavity lined with endometrium and surrounded by a muscular layer. The patient's postoperative period was uneventful and her general physical and abdominal condition was normal on discharge. She became totally asymptomatic per close follow-ups for 3 months. Literature search: Sixty-eight cases, including the current case, met inclusion criteria for ACUMs associated with otherwise normal uteri. The average age of women was 25.4 years (range: 14–51 years). The majority had dysmenorrhea or chronic pelvic pain treated elsewhere for many years. All of these patients has their masses excised, and histopathology testing revealed showed that they were cavities lined by endometria and surrounded by muscular layers. Thirty three (48.5%) masses were on the right lateral walls of the uteri adjacent to the round ligaments, 22 (32.3%) were near the attachments of the left round ligaments, 5 (7.3%) were in the broad-ligaments away from the uteri, 3 (4.4%) were subserosal, 2 (2.9%) were in the posterior aspect of the uteri just above the cervices, 1 (1.4%) was on left inguinal subserosal tissue, 1 (1.4%) was on the left anterior wall of the uterus near the uterine artery, and 1 (1.4%) was on the colonic submucosa at the appendiceal foramen.

Conclusions:

ACUM, a rare Müllerian anomaly related to dysfunction of the gubernaculum, should be considered in the differential diagnosis of severe dysmenorrhea and chronic pelvic pain. Magnetic resonance imaging showing an accessory mass, near the attachment of the round ligament, in an otherwise normal uterus with bilateral cornua suggests the diagnosis of ACUM. Excision of the mass is the treatment of choice. ACUM should be considered a newer entity among Müllerian anomalies.