A Rare Case: Nodular Adenomyosis Can Deceive Cornual Ectopic Pregnancy

Adenomyosis refers to the occurrence of the endo - metrial glands and surrounding stroma within the uter- ine myometrium. A distinction is made between diffuse and focal forms: rare nodular forms and adenomyoma [1,2]. Adenomyoma is a tumor-like lesion of adenomy- osis uteri with structurally complex endometrial glands in a hyperplastic stroma consisting of smooth muscles or leiomyo fibromatous tissue [2,3]. About 1% of the af- fected patients are under 40 years of age, in these cases the reproductive phase of the woman is affected [4]. Ectopic pregnancy (EUG) occurs in about 1.5 to 2.0% of pregnancies and can be a life-threatening event. Such pregnancies cause 6% of all maternal deaths in gyne - cology [5]. 2% of all EUG are cornual/intramural preg - nancies [6]. Ascornual/intramural gravity is a pregnancy implanted in the myometrium and not surrounded by decidua. Risk factors are previous adnexitis and internal genital surgery, in particular homolateral salpingecto - my, and adenomyosis uteri. Cornual/intramural preg - nancies are very rare [7,8]. Case Report A 33-year-old GII/PI patient presented herself with vaginal bleeding in the 6 th week of pregnancy in our emergency department. Sonographically, one could *Corresponding author: Elvin Piriyev, Dr. Med, Department of Obstetrics and Gynaecology, Academic Hospital Cologne- Weyertal, University of Cologne, Germany 1Department of Obstetrics and Gynaecology, Academic Hospital Cologne-Weyertal, University of Cologne, Germany 2Center for Pathology and Cytology, Weyertal, Germany CasE REPoRT Check for updates

Adenomyosis refers to the occurrence of ectopic endome - trial glands and the surrounding stroma within the myo- metrium. A distinction is made between diffuse and focal forms (rare nodular forms and an adenomyoma). Ectopic pregnancy (EUG) occurs in about 1.5 to 2.0% of pregnan - cies and can be a life-threatening event. 2% of all EUG are cornual/intramural ectopic pregnancy. We present the case of a 33-year-old women (GII/PI) who complained about vag- inal bleeding in the 6th week of pregnancy. The next pres- entation took place in 7 + 3 gestational weeks due to con- traction-like pain in the lower abdomen and constant pain in the right lower abdomen. Sonographically we discovered the intrauterine amniotic cavity with an abnormal embryonic development, an intracavitary hematoma and a “thickening” in the right tube angle. After 4 days (8 + 0 weeks of gesta- tion), the patient complained again with constant pain in the right lower abdomen comparable to dysmenorrhea. Sono- graphically, an empty amniotic cavity and a cystic mass in the right-angle of fallopian tube continued to be present. HCG was 6774 mIU/ml. With the high suspicion of a cornu- al ectopic pregnancy, a laparoscopy and a curettage were indexed. Intraoperatively, a protrusion in the right-angle of fallopian tube was present. The finding was cut with a monopolar needle and a chocolate-like secretion emptied. Here, endometrial tissue became apparent. A wedge resec- tion was carried out with the removal of the entire ectopic endometrial tissue. After the histological processing of the preparation, endometriosis with a moderate proliferative activity emerged: Ki 67 of 10%. In the abrasion material a gestational sac, as well as parts of pregnancy-typical endo- metrium were detected. In case of a sonographically seen gestational sac located at the angle of a fallopian tube, one should think of a cornual/interstitial localization of a EUG. However, even in nodular adenomyosis a cystic finding may form as it was proven in our case. Therefore, in order to for- mulate a precise and reliable diagnosis a laparoscopy with tissue removal for histological may be necessary.

Nodular adenomyosis, Cornual pregnancy, Ectopic preg- nancy, Endometriosis ISSN: 2377-9004 DOI: 10.23937/2377-9004/1410166 Piriyev et al. Obstet Gynecol Cases Rev 2020, 7:166 • Page 2 of 4 • detect an intrauterine amniotic cavity corresponding to the week of pregnancy. Thus, physical rest and a fol - low-up were initially recommended (Figure 1). At the time of 7 + 3 gestational weeks the patient presented herself again with the main complaints of contraction-like pain in the lower abdomen, constant pain in the right lower abdomen and persistent vaginal bleeding. Sonographically, an amniotic sac without an embryo and an intracavitary hematoma were found, which gave rise to the suspicion of an abortus incipiens. This ultrasound showed a “thickening” in the right uter- us horn/angle of fallopian tube. The patient reported that she had been suffering from pain of similar nature, described as severe dysmenorrhea in the right lower ab- domen already before pregnancy (Figure 2 and Figure 3). The patient did not want any further therapy at this time. 4 days later, the patient still suffered from constant pain in the right lower abdomen, as in dysmenorrhea. A vaginal examination in the right lower abdomen was very painful for the patient. The β-HCG value at that time was 6774 mIU/ml. Sonographically, an empty am- niotic cavity and a cystic mass at the angle of the right fallopian tube continued to be present. There was an ur- gent suspicion of a cornual pregnancy, so that laparos - copy and simultaneous curettage were indexed (Figure 4 and Figure 5). The patient underwent surgery on the same day. In the curettage, a lot of pregnancy-typical material was obtained. Subsequently, laparoscopy took place. Intra- operatively, a protrusion was present at the angle of the right fallopian tube. Through an incision with a monop - olar needle and chocolate-like secretion was drained off. Also, endometrial-typical tissue emerged. Wedge resection was carried out with removal of the entire endometrial tissue after injection of diluted adrenalin. Amniotic cavity Figure 1: Uterus in sagittal section (6 gestation weeks). Amniotic cavity hematoma Figure 2: Uterus in the sagittal section (7 + 3 gestation weeks). Amniotic cavity thickening Figure 3: Uterus in cross section (7 + 3 SSW). cavity Cyst in rightuterus horn Figure 4: Uterus in sagittal section (8 + 0 gestation weeks). cavity Cyst in rightuterus horn Figure 5: Uterus in cross section (8 + 0 gestation weeks). ISSN: 2377-9004 DOI: 10.23937/2377-9004/1410166 Piriyev et al. Obstet Gynecol Cases Rev 2020, 7:166 • Page 3 of 4 • adenomyoma is destroying the myometrium whereas myomas merely displace the myometrium [11]. If pregnancy is excluded, hormonal therapy can also be performed. Progestins are available in the various application forms. Orally, combined ovulation inhibitors can be used especially in the long-cycle regime as well as progestogen-only preparations. Using a LNG-IUS lo - cally is also worth considering, because there is a very good data situation for Levonorgestrel containing intra- uterine device (LNG-IUS) [12]. The treatment of cornual pregnancy is distinguished in surgical and drug therapy with methotrexate. Howev- er, in cornual pregnancies, wedge resection with remov- al of the entire chorial tissue appears to be the therapy of choice [13].

The clinical differential diagnosis between nodular adenomyosis in an angle of fallopian tube during preg - nancy and a cornual ectopic pregnancy is very difficult. Sonography can be a valuable tool in diagnosis. In the case of a gestational sac in an angle of fallopian tube, one should think of an interstitial or cornual localiza - tion of a EUG. However, even in nodular adenomyosis a cystic finding may form as it was proven in our case. Therefore, in order to formulate a precise and reliable diagnosis a laparoscopy with tissue removal for histo - logical may be necessary. Conflict of Interest The authors declare that they have no conflict of in - terest. Source of Funding This manuscript was not funded. Informed Consent The patient has consented to this manuscript. Ethics Approval This manuscript was carried out in consensus with our university´s ethics guidelines.

1. Bergeron C, Amant F, Ferenczy A (2006) Pathology and physiopathology of adenomyosis. Best Pract Res Clin Ob- stet Gynaecol 20: 511-521. 2. Nawroth F, Romer T (2015) Diagnostics and therapy of fe- male sterility. De Gruyter. 3. Rabe T, Schweppe KW, Ebert AD, Mlynek-Kersjes ML, Merkle E, et al. (2017) Adenomyosis uteri. J Reprodukti - onsmed Endokrinol 14: 211-218. 4. Devlieger R, D’Hooghe T, Timmerman D (2003) Uterine adenomyosis in the infertility clinic. Hum Reprod Update 9: 139-147. 5. Barnhart KT (2009) Ectopic Pregnancy. N Engl J Med 361: 379-387. The wound was treated with resorbable suture material (Figure 6). Postoperatively, the patient became free of com- plaints. After inconspicuous checks, she was discharged after two days in good health. Histological processing of the obtained material showed following results: I. Abortion material: Gestational sac with hydropic re - gressive changes and part of pregnancy-typical en- dometrium. II. Uterine exudation: An endometriosis with a moder- ate degree of proliferative activity: Ki 67 of 10%. In this case two findings were seen at once: On one hand, there was a pregnancy and subsequent early spontaneous abortion. On the other hand, the patient had a nodular adenomyosis in the right uterine horn, which developed further during pregnancy. The β-HCG value fell to 1288 mIU/ml on the first postoperative day. The further postoperative controls were inconspicuous.

In local adenomyosis, uterine-preserving resection can take place. In nodular adenomyosis, a resection or a volume reduction of adenomyosis can be sought. The adenomyoma should be resected, similar to the man- agement of uterine fibroids, especially in patients who wish to have children with findings > 4 cm [9,10]. How- ever, the procedure of resecting adenomyoma is techni- cally more demanding than extracting myomas, because Uterus righthorn Endometrium Tube Uterus Uterus righthorn Figure 6: Wedge resection was carried out with removal of the entire endometrial tissue. ISSN: 2377-9004 DOI: 10.23937/2377-9004/1410166 Piriyev et al. Obstet Gynecol Cases Rev 2020, 7:166 • Page 4 of 4 • 10. Zepiridis LI, Grimbizis GF, Tarlatzis BC (2016) Infertility and uterine fibroids. Best Pract Res Clin Obstet Gynaecol 34: 66-73. 11. Grimbizis GF, Mikos T, Tarlatzis B (2014) Uterus-sparing operative treatment for adenomyosis. Fertil Steril 101: 472- 487. 12. Romer T, Buhling KJ (2019) Updated consensus recom- mendation on the use of LNG-IUS. Drug Report 1-32. 13. Auslender R, Arodi J, Pascal B, Abramovici H (1983) Inter- stitial pregnancy: Early diagnosis by ultrasonography. Am J Obstet Gynecol 146: 717-718. 6. James AH (2019) Bleeding and the management of hem- orrhagic disorders in pregnancy. Consultative Hemostasis and Thrombosis (Fourth Edition). 7. Lermann J, Muller A, Schulze C, Becker S, Boosz A, et al. (2009) Die Extrauteringraviditat. Frauenheilkunde 383-402. 8. Pritchard JA, MacDonald PC, Gant NF, J Whitridge Willi- ams (1985) Williams obstetrics. (17 th edn), Appleton-Centu- ry Croft, Norwalk, Connecticut, 423-438. 9. Schindler AE (2008) Surgical and drug therapy for endome- triosis/adenomyosis. J Gynakol Endokrinol 18: 18-26.