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

Abstract

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 M€ ullerian anomaly. Design: Case reports and a literature review. Setting: A 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 M€ ullerian 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 intervention Result(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 M€ ullerian anomaly from other rare abnormalities like JCA. When in doubt, laparoscopy can assist in diagnosing and treating the condition.

Accessory cavitated uterine malformation (ACUM): A scoping review

“…This review thus comprised the largest number of published cases on this relatively unknown disease. In contrast to previously published (narrative) reviews, 34 , 35 , 36 we also included both conservatively and surgically managed cases, thus not only considering those with a histological diagnosis. This approach led to the identification of conservative management as a potential treatment option.…”

Impact of an accessory cavitated uterine mass on fertility: case presentation and review of the literature

“…Recently, controversy has emerged regarding the inclusion of JCA as an entity of ACUM. Previously, JCA has been defined as a cystic lesion >1 cm in diameter; independent of the uterine cavity; covered by hypertrophic myometrium as indicated on radiologic imaging; associated with severe dysmenorrhea; and age <30 years ( 9 ). Previous studies conclude that JCA is a rare variant of adenomyosis rather than a congenital abnormality with distinguishing criteria from ACUM including the presence of denser areas of adenomyosis surrounding the cystic area ( 9 , 10 ).…”

“…Previously, JCA has been defined as a cystic lesion >1 cm in diameter; independent of the uterine cavity; covered by hypertrophic myometrium as indicated on radiologic imaging; associated with severe dysmenorrhea; and age <30 years ( 9 ). Previous studies conclude that JCA is a rare variant of adenomyosis rather than a congenital abnormality with distinguishing criteria from ACUM including the presence of denser areas of adenomyosis surrounding the cystic area ( 9 , 10 ). However, other publications suggest that JCA in young women should be grouped with ACUM to avoid nosological framework overlap and that adenomyosis surrounding may result from increased intra-cystic menstrual pressure ( 2 , 7 , 9 ).…”

“…Previous studies conclude that JCA is a rare variant of adenomyosis rather than a congenital abnormality with distinguishing criteria from ACUM including the presence of denser areas of adenomyosis surrounding the cystic area ( 9 , 10 ). However, other publications suggest that JCA in young women should be grouped with ACUM to avoid nosological framework overlap and that adenomyosis surrounding may result from increased intra-cystic menstrual pressure ( 2 , 7 , 9 ). In support of this argument, both identified entities have similar outcomes after surgical treatment ( 9 , 10 ).…”

“…However, other publications suggest that JCA in young women should be grouped with ACUM to avoid nosological framework overlap and that adenomyosis surrounding may result from increased intra-cystic menstrual pressure ( 2 , 7 , 9 ). In support of this argument, both identified entities have similar outcomes after surgical treatment ( 9 , 10 ).…”