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Volume 113, Issue 2, Pages 340–341


Akash Shah, B.S., Steven R. Lindheim, M.D., M.M.M., Stephanie J. Estes, M.D.


Reflections on "Decidualized juvenile cystic adenomyoma mimicking a cornual pregnancy" by Kiyak et al.

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Fertility and Sterility

Editorial Office, American Society for Reproductive Medicine

Fertility and Sterility® is an international journal for obstetricians, gynecologists, reproductive endocrinologists, urologists, basic scientists and others who treat and investigate problems of infertility and human reproductive disorders. 


Go to the profile of Tolga Karacan
over 1 year ago

Dear Editor,

We would like to express our sincere gratitude and appreciation for the meaningful commentary written by Akash Shah,  Steven R. Lindheim and  Stephanie J. Estes which has added great significance to our manuscript entitled "Decidualized Juvenile Cystic Adenomyoma Mimicking a Cornual Pregnancy”. As mentioned in the commentary, a physician should always "be prepared" to seek rational solutions for challenges which may be encountered during clinical practice, similar to scouts in different regions of the world who stand ready in mind, body and heart for whatever challenges that may be awaiting. We hope our presentation of the surgical management of a rarely encountered entity, juvenile cystic adenomyoma, will contribute to the current knowledge on this pathology.

Adenomyosis is defined as the presence of ectopic endometrial glands and stroma in the myometrium with hyperplasia of surrounding smooth muscle cells. Adenomyosis may be diffuse or focal (1). Despite the growing body of evidence concerning the treatment options in subjects with adenomyosis, the etiology of this disorder has not been clearly elucidated yet. There are two commonly accepted hypotheses proposed for the pathogenesis of adenomyosis (2). The first hypothesis is the invagination of endometrial glands into the myometrium as a result of hyperestrogenism, hyperperistaltism or tissue damage and repair mechanisms. The second proposed hypothesis is the metaplasia of embryonic pluripotent Mullerian remnants within the myometrium into endometrial tissue leading to adenomyosis.

The diagnostic criteria for JCA suggested by Takeuchi et al. include 1) age <30 years; 2) a cystic lesion ≥1 cm in diameter which is independent from the uterine cavity and is surrounded by hypertrophic myometrium on diagnostic images; 3) and association with severe dysmenorrhea (3). Takeuchi and colleagues also suggested that JCA may be a variant of focal adenomyosis presenting in younger women. Acien et al. proposed that the entity previously defined as JCA could in fact be a new type of Mullerian anomaly defined as an accessory cavitated uterine mass (ACUM), which may result from duplication or persistence of ectopic ductal Mullerian tissue following gubernaculum dysfunction. In such patients, the uterine cavity and fallopian tubes are normal.

Before a diagnosis of JCA can be made, obstructive type Mullerian anomalies of the uterus need to be excluded. The differential diagnosis should also include hematometra complicating the non-communicating horn of a unicornuate uterus, fibroids with lipoid or hemorrhagic degeneration, congenital uterine cysts and intrauterine hydrosalpinges.

Transvaginal ultrasound is the primary imaging technique used in the evaluation of gynecologic pathologies. However, a gynecologist may not always be able to reach the correct diagnosis with only ultrasonography, as was in the present case. Considering the high HCG level and a cystic cavity with decidualization in the right cornual area, we were focused on the diagnosis of a cornual pregnancy; however, if an MRI had been performed before surgery the correct diagnosis might have been reached preoperatively. Upon laparoscopic exploration, an ectopic pregnancy was observed within the left fallopian tube rather than in the right cornual area. The possibility of a simultaneous bilateral ectopic pregnancy involving both the left fallopian tube and the right cornual area was excluded as the right tube appeared normal. Upon these findings, the right cornual mass was thought to be a fibroid and its resection was planned for a definitive diagnosis. As an incision was made over the mass, a pseudo-capsule could not be visualized around the mass.  Consequently, it was decided that the mass was most probably an area of focal adenomyosis. A surgical technique similar to the enucleation of fibroids has been defined previously for focal adenomyotic lesions; however because these lesions lack a pseudocapsule, their enucleation and excision is more complicated than fibroids (4). In such cases, division of the adenomyotic mass into two halves before its excision helps in better identification of the tumor borders. The nodular structure of adenomyotic tissue and the circular arrangement of the surrounding smooth muscle layer also help to differentiate the pathological tissue from healthy myometrium. The difference in the colour and texture of healthy and pathological tissues is evident even under 2 dimensional laparoscopic visualization.  When the base of the nodular tissue is reached, continuation of careful dissection along the myometrial border allows total resection of the mass with preservation of intact myometrium. 

The laparoscopic bisection of a mass without a definite pathological diagnosis may be a matter of concern for disease spread in cases of malignancy. However, the risk of sarcoma in subjects with uterine fibroids is extremely low, particularly in women < 30 years of age (5). Since adenomyosis is a benign disease, we believe this technique does not pose a significant risk for tumor spread in such patients. Multilayer repair of the myometrial defect following resection of the mass is crucial in reducing the risk of uterine rupture during ensuing pregnancies.

We also recommend obtaining the informed consents of patients covering all possible surgical options prior to surgery in cases with complicated clinical presentations. We believe that a comprehensive informed consent will facilitate safer treatment of unexpected pathologies which can be encountered during surgery. We declare ‘daima hazır!(always ready!)‘ to be our motto as Turkish gynecological surgeons and aim to be prepared for challenges at all times with the spirit of serving the community in accordance with the principles of scouting all over the world.


Kiyak Huseyin1 M.D.; Seckin Doga Kerem1 M.D.; Karakis Lale2 M.D.; Karacan Tolga3 M.D.; Ozyurek Sefik Eser3 M.D.; Asoglu Resit Mehmet2 M.D.

1Department of Obstetrics and Gynecology, Kanuni Sultan Suleyman Research and Education Hospital, University of Health Sciences, Istanbul, Turkey.

2Department of Obstetrics and Gynecology, Bahceci Woman Health Center Reproductive Endocrinology and Infertility, Istanbul, Turkey

2Department of Obstetrics and Gynecology, Bagcilar Research and Education Hospital, University of Health Sciences, Istanbul, Turkey.



  1. Garcia-Solares J, Donnez J, Donnez O, Dolmans MM. Pathogenesis of uterine adenomyosis: invagination or metaplasia? Fertility and sterility. 2018;109(3):371-9.
  2. Vannuccini S, Tosti C, Carmona F, Huang SJ, Chapron C, Guo SW, et al. Pathogenesis of adenomyosis: an update on molecular mechanisms. Reproductive biomedicine online. 2017;35(5):592-601.
  3. Takeuchi H, Kitade M, Kikuchi I, Kumakiri J, Kuroda K, Jinushi M. Diagnosis, laparoscopic management, and histopathologic findings of juvenile cystic adenomyoma: a review of nine cases. Fertility and sterility. 2010;94(3):862-8.
  4. Acien P, Bataller A, Fernandez F, Acien MI, Rodriguez JM, Mayol MJ. New cases of accessory and cavitated uterine masses (ACUM): a significant cause of severe dysmenorrhea and recurrent pelvic pain in young women. Human reproduction (Oxford, England). 2012;27(3):683-94.
  5. Kundu S, Zachen M, Hertel H, Hillemanns P, Soergel P. Sarcoma Risk in Uterine Surgery in a Tertiary University Hospital in Germany. International journal of gynecological cancer: official journal of the International Gynecological Cancer Society. 2017;27(5):961-6.