Juvenile cystic adenomyoma, a rare diagnostic challenge: Case Reports and literature review

Sushila Arya,Heather R Burks

doi:10.1016/j.xfre.2021.02.002

Abstract

ObjectiveTo report 2 very rare cases of young women who presented with severe dysmenorrhea and a large cystic lesion in the myometrium, which presented a diagnostic dilemma because they were confused with a Müllerian anomaly.DesignCase reports and a literature review.SettingA university-based reproductive endocrinology and infertility clinic in the United States.Patient(s)An 18- and a 16-year-old nulliparous girl presented with worsening of their longstanding pelvic pain, and imaging study results were suggestive of a Müllerian anomaly.Intervention(s)Abdominal and pelvic computed tomography, transvaginal ultrasonography, pelvic magnetic resonance imaging, operative laparoscopy, and excision of a juvenile cystic adenomyoma (JCA).Main Outcome Measure(s)Resolution of the pelvic pain and restoration of normal uterine anatomy after appropriate interventionResult(s)Restoration of normal uterine anatomy, which was confirmed by 3-dimensional ultrasonography for case 1; however, case 2 still had a small remnant of JCA postoperatively.Conclusion(s)Clinical and radiologic examinations may not be useful in differentiating a Müllerian anomaly from other rare abnormalities like JCA. When in doubt, laparoscopy can assist in diagnosing and treating the condition.

Highlights

There is a small population of individuals who have larger cysts within the uterine body, termed as ‘‘cystic adenomyosis.’’ These large myometrial cysts in young women have often been described in the literature using various terms, including juvenile cystic adenomyosis (JCA), cystic myometrial lesions, accessory uterine cavity masses, or juvenile adenomyotic cysts
Case reports of juvenile cystic adenomyoma (JCA) were included if they met the following criteria: if they met the abovementioned criteria used to include a case as JCA, as described by Takeuchi et al [5]; and adolescent girls and women below 30 years who presented with early-onset severe dysmenorrhea and pelvic pain
All cases reported were histopathologically confirmed JCA that presented with severe primary dysmenorrhea and a large, isolated cystic myometrial lesion revealed by pelvic imaging studies

Summary

Objective

To report 2 very rare cases of young women who presented with severe dysmenorrhea and a large cystic lesion in the myometrium, which presented a diagnostic dilemma because they were confused with a Mu€llerian anomaly. Secretory changes and menstrual bleeding within this heterotopic endometrial tissue can result in the formation of tiny hemorrhagic foci (30 years of age with global/diffuse adenomyosis. There is a small population of individuals who have larger cysts within the uterine body, termed as ‘‘cystic adenomyosis.’’ These large myometrial cysts in young women have often been described in the literature using various terms, including juvenile cystic adenomyosis (JCA), cystic myometrial lesions, accessory uterine cavity masses, or juvenile adenomyotic cysts. Takeuchi et al [5] described JCA based on the following diagnostic criteria: age 1 cm in diameter, independent of the uterine cavity, and covered by hypertrophic myometrium, as seen on radiologic images; and association with severe dysmenorrhea [5]

MATERIALS AND METHODS

DISCUSSION

CONCLUSION