•We present a case of atypical adenomyosis with clinical, laboratory, and imaging findings suggestive of a molar pregnancy.•Adenomyosis causes uterine enlargement and may appear cystic on vaginal ultrasound.•Falsely elevated β-hCG in the setting of obesity and hypothyroidism may complicate diagnosing abnormal uterine bleeding

Bedrick, Bronwyn S

Chen, Tiffany

Dicke, Jeffrey

Greenwade, Molly M

Kazarian, Gregory S

Maluf, Horacio

Spies, Nick

Thaker, Premal H

English

Digital Commons@Becker

Washington University School of Medicine Digital Commons@Becker Open Access Publications 5-1-2020 Adenomyosis presenting as a molar pregnancy: A case report Bronwyn S Bedrick Gregory S Kazarian Molly M Greenwade Nick Spies Tiffany Chen See next page for additional authors Follow this and additional works at: https://digitalcommons.wustl.edu/open_access_pubs Authors Bronwyn S Bedrick, Gregory S Kazarian, Molly M Greenwade, Nick Spies, Tiffany Chen, Horacio Maluf, Jeffrey Dicke, and Premal H Thaker Contents lists available at ScienceDirectGynecologic Oncology Reportsjournal homepage: www.elsevier.com/locate/gynorCase reportAdenomyosis presenting as a molar pregnancy: A case reportBronwyn S. Bedricka, Gregory S. Kazariana, Molly M. Greenwadeb, Nick Spiesc, Tiffany Chend,Horacio Malufd, Jeffrey Dickec, Premal H. Thakerb,⁎aWashington University School of Medicine, St. Louis, MO, USAbDivision of Gynecologic Oncology, Washington University School of Medicine, St. Louis, MO, USAc Department of Obstetrics and Gynecology, Washington University School of Medicine, St. Louis, MO, USAdDepartment of Pathology, Washington University School of Medicine, St. Louis, MO, USAA R T I C L E I N F OKeywords:Cystic adenomyosisGestational trophoblastic diseaseHook effectHypothyroidismAbnormal uterine bleeding1. IntroductionAbnormal uterine bleeding (AUB) is a common symptom with abroad differential diagnosis. One cause of AUB is adenomyosis, a con-dition that results from benign proliferation of endometrial glands andstroma into the myometrium. Adenomyosis is characterized by a his-tologically glandular appearance and patchy solid proliferation of en-dometrial glands and stroma, with cystic adenomyosis being lesscommon (Troiano et al., 1998; Dogan et al., 2008; Brosens et al., 2015).Patients with adenomyosis classically present with AUB, pelvic pain,and dysmenorrhea. Due to the non-specific nature of these symptoms, aconclusive diagnosis of adenomyosis often relies on imaging or histo-logical findings. As adenomyosis can affect pre- and postmenopausalwomen, the work-up must exclude pregnancy-related causes of AUB.While there are many possible causes of vaginal bleeding, molarpregnancy is life-threatening and requires rapid evaluation and man-agement. Molar pregnancy, the result of abnormal fertilization andsubsequent aberrant proliferation of an egg, occurs in about 1 in every1000 pregnancies. Clinical findings include vaginal bleeding, rapiduterine growth with uterine size exceeding expected gestational age,ovarian cysts, emesis, anemia, and preeclampsia. Sonographic imagingoften demonstrates a “snowstorm” appearance of the uterus, and me-tastatic lesions may appear on chest imaging (Berkowitz and Goldstein,2009). Given the life-threatening nature of molar pregnancy, it is im-portant to promptly rule out this diagnosis in women of child-bearingage presenting with vaginal bleeding and abdominal pain.Here, we report a case of histologically atypical adenomyosis in awoman presenting with clinical, laboratory, and imaging findingsconcerning for a molar pregnancy.2. CaseA 30-year-old G1P0010 premenopausal female presented to anoutside hospital emergency department after a week of profuse vaginalbleeding with large blood clots, nausea, lightheadedness, diffuse lowerabdominal pain, and a syncopal episode. Her past medical history in-cluded a spontaneous abortion at 11 weeks gestational age at age 18and a BMI of 48.4 kg/m2. Her menstrual history was significant formenarche at age 13 and regular monthly cycles until age 27, when shedeveloped AUB. She denied recent hormonal contraceptive use and waslast sexually active six months ago.Upon initial presentation, laboratory testing revealed an elevatedquantitative β-hCG of 25.0 mIU/mL and a Hgb of 9.6 g/dL. She wasdiagnosed with a spontaneous abortion and acute blood loss anemiaand discharged with plans to repeat labs in 48 h to confirm this diag-nosis. However, her symptoms continued, prompting her return to theemergency department two days later. On return, Hgb had decreased to8.3 g/dL, and β-hCG was 24.0 mIU/mL. Transvaginal ultrasound de-monstrated an enlarged, globular uterus (21.2 × 16.6 × 12.6 cm) witha heterogeneously hyperechoic mass, demonstrating small cystic fociwithin the uterus (12.6 × 14 × 10.6 cm). The mass extended distallyinto the cervix and appeared to invade posteriorly into the myometrium(Fig. 1). Thyroid function tests were also obtained, and TSH was foundto be elevated at 9.59 mU/L with normal T3 and T4. Given her enlargedhttps://doi.org/10.1016/j.gore.2020.100573Received 28 February 2020; Received in revised form 17 April 2020; Accepted 18 April 2020⁎ Corresponding author at: Washington University School of Medicine, 660 South Euclid Avenue, Mail Stop 8064-37-905, St. Louis, MO 63110, USA.E-mail address: thakerp@wustl.edu (P.H. Thaker).Gynecologic Oncology Reports 32 (2020) 100573Available online 23 April 20202352-5789/ © 2020 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).Tuterus, persistently elevated β-hCG, ultrasound findings, and suggestivesymptoms, she was transferred to our gynecologic oncology service forfurther evaluation of a suspected molar pregnancy.On admission, a chest radiograph was obtained that demonstrated anodular opacity within the left lung concerning for metastasis of ge-stational trophoblastic disease. A subsequent CT scan did not supportmetastatic disease to the lungs, but it demonstrated an enlarged uterusand hypovascular mass concerning for molar pregnancy and ovarianchanges concerning for theca lutein cysts.Although the patient’s history appeared to be most concerning formolar pregnancy, her BMI and hence elevated estrogen exposure, raisedour suspicion for other potential causes of AUB, including endometrialhyperplasia, adenomyosis, and malignancy (Templeman et al., 2008).β-hCG levels were repeated and remained elevated at 12.0 mIU/mL.The patient was consented for exam under anesthesia with diagnosticand therapeutic suction dilation and curettage and was counseled re-garding the risks of surgery and the potential need for total abdominalhysterectomy to achieve hemostasis. The patient was not interested infuture fertility. Immediately prior to surgery, the patient had a urine β-hCG test, which was negative.While performing a bimanual exam, manipulation of the cervixprompted profuse, bright red vaginal bleeding, with an estimated bloodloss of 300 cc within minutes. The decision was made to proceed ur-gently with a total abdominal hysterectomy. The ovaries appearedgrossly normal at the time of surgery and were left in situ, and bilateralsalpingectomy was performed. The uterus was grossly inspected by thesurgeon and noted to be enlarged and globular. Bivalving the uterusrevealed poorly defined myometrium, and an endometrial cavity filledwith cystic material. The specimen was sent to pathology for frozensection, but pathology was unable to confirm a histological diagnosisintraoperatively. She received a total of four units of packed red bloodcells. Her post-operative course was routine except for a superficialwound separation. Final pathology reported a 1620.9 g uterus mea-suring 17.5 cm. The endocervix measured 5 × 2 cm and was lined by aherringbone pattern of tan-pink mucosa with no discernible lesions. Themajority of the myometrium, endocervical cavity, and lower uterinesegment demonstrated a spongy trabeculated tan-white homogenouscut surface with no areas of firmness or necrosis. Analysis of permanentsections yielded a diagnosis of cystic adenomyosis of the cervix anduterus (Fig. 2).3. DiscussionAdenomyosis has historically been diagnosed through histologicexamination of hysterectomy specimens, and thus has been thought tobe a disease of women in their 40 s and 50 s. However, with im-provements in radiologic imaging, adenomyosis is being detected atearlier ages. Cystic adenomyosis also tends to present in youngerwomen, even in adolescents, and with more severe symptoms (Brosenset al., 2015). However, adenomyosis can present diagnostic challenges,as cysts can mimic uterine malformation or ovarian masses (Doganet al., 2008; Brosens et al., 2015), and imaging and histologic featuresmay suggest malignancy (Goldblum et al., 1995; Funaki et al., 2011).Identification of adenomyosis in these cases may be critical as definitivetreatment varies drastically and may eliminate future fertility or induceearlier menopause in young women without malignancy. To ourknowledge, this is the first case report of a presentation of adenomyosispresenting with symptoms, imaging, and laboratory findings con-cerning for molar pregnancy, which further highlights the challenges indiagnosing this condition.Like adenomyosis, the definitive diagnosis for a molar pregnancy isby histology. However, an accurate diagnosis can be made in the settingof classic radiographic appearance, especially with elevated β-hCG le-vels and clinical symptoms such as nausea and vomiting. With 70%sensitivity and 99% specificity, ultrasound can accurately identifycomplete molar pregnancies, which frequently presents as a diffuse,heterogenous echogenic pattern of villi and blood clots, or “snowstorm”appearance (Ross et al., 2018). Additionally, theca lutein cysts arefound in 15–25% of patients with molar pregnancies and can aid indiagnosis. Overall, cystic uterine and ovarian changes are morecommon in the setting of molar pregnancies than in adenomyosis. Inour case, it is likely that the “snowstorm” appearance identified onultrasound represented small cysts associated with cystic adenomyosis,and that the ovarian changes were simply functional cysts.Markedly elevated levels of β-hCG are responsible for many of theclassic signs and symptoms of molar pregnancies including theca luteincysts and nausea and vomiting. While molar pregnancy is frequentlyassociated with β-hCG > 100,000 mIU/mL, our patient’s levels weremarginally elevated. Negative or falsely low levels of β-hCG can occurin the setting of molar pregnancies due to the “hook effect,” in whichthe signaling antibodies used to detect β-hCG are oversaturated andlead to faulty measurements (Tabas et al., 2003). While rare, the hookeffect can lead to delayed therapy and life-threatening complications. Inour case, serial dilutions were not performed because it would not havechanged the initial plan for diagnostic curettage. On the other hand,falsely elevated levels of β-hCG levels can occur in the setting of het-erophile antibodies, in which serum antibodies interfere with the β-hCGimmunoassay. A negative urine β-hCG test in the setting of a positiveserum β-hCG test, as seen with our patient, may indicate the presence ofheterophile antibodies, which are not renally excreted (Check et al.,Fig. 1. Transabdominal ultrasound image from the emergency department demonstrating an enlarged uterus and a heterogeneously hyperechoic mass demonstratingsmall cystic foci within the uterus.B.S. Bedrick, et al. Gynecologic Oncology Reports 32 (2020) 10057321988). Falsely elevated β-hCG levels can be seen with obesity and hy-pothyroidism. Given that hCG and TSH have a common α subunit,elevated TSH levels can falsely increase hCG readings, and this is an-other potential explanation in our patient.This case also illustrates an important lesson in the hemodynamicmanagement of patients with adenomyosis. Given our patient’s profusebleeding with cervical manipulation during the bimanual exam, it isimportant to acknowledge the potential for hemorrhage when ex-amining patients with adenomyosis, especially in the clinical setting.While recent data suggest that the use of uterotonics such as oxytocinprior to dilation and evacuation can reduce blood loss and hemorrhagefrequency (Whitehouse et al., 2019), it is unlikely that the use ofuterotonics in our case could have prevented significant hemorrhagegiven the extensive cystic distortion of the uterus and lower uterineoxytocin receptor concentration in a nongravid uterus.4. ConclusionWe report a case of extensive adenomyosis with clinical, imaging,and laboratory findings concerning for molar pregnancy. This casehighlights how hypothyroidism may obfuscate the interpretation of β-hCG levels in reproductive age women and that it should be consideredin the differential diagnosis of an elevated β-hCG. Although rare, cysticadenomyosis should also be considered in women with abnormaluterine bleeding and a “snowstorm” appearing uterus on ultrasound.Finally, this case illustrates how a constellation of symptoms may beexplained by relatively common conditions rather than one relativelymore rare condition.5. Ethics approvalThis retrospective case report was exempt for Institutional ReviewBoard approval.6. ConsentInformed consent was obtained from the patient, which approvedreporting of this case and accompanying images.Author contributionGSK, BSB, NS, MMG, and PHT wrote the main manuscript body.MMG and PHT were involved in patient treatment. TC and HM wereresponsible for reviewing pathology. JD was responsible for reviewingradiology. All authors reviewed the final manuscript.Declaration of Competing InterestThe authors of this case report declare no conflicts pertaining to thecurrent study.ReferencesTroiano, R.N., Flynn, S.D., McCarthy, S., 1998. Cystic adenomyosis of the uterus: MRI. J.Magn. Reson. Imaging 8 (6), 1198–1202.Dogan, E., Gode, F., Saatli, B., Secil, M., 2008. Juvenile cystic adenomyosis mimickinguterine malformation: a case report. Arch. Gynecol. Obstet. 278 (6), 593–595.Brosens, I., Gordts, S., Habiba, M., Benagiano, G., 2015. Uterine cystic adenomyosis: adisease of younger women. J. Pediatr. Adolesc. Gynecol. 28 (6), 420–426.Berkowitz, R.S., Goldstein, D.P., 2009. Clinical practice. Molar pregnancy. N Engl. J. Med.360 (16), 1639–1645.Templeman, C., Marshall, S.F., Ursin, G., Horn-Ross, P.L., Clarke, C.A., Allen, M., et al.,2008. Adenomyosis and endometriosis in the California Teachers Study. Fertil. Steril.90 (2), 415–424.Goldblum, J.R., Clement, P.B., Hart, W.R., 1995. Adenomyosis with sparse glands. Apotential mimic of low-grade endometrial stromal sarcoma. Am. J. Clin. Pathol. 103(2), 218–223.Funaki, K., Fukunishi, H., Maeda, T., Ohbayashi, C., Yamaguchi, S., 2011. Adenomyosiswith extensive glandular proliferation simulating infiltrating malignancy on magneticresonance imaging. Jpn. J. Radiol. 29 (4), 272–275.Ross, J.A., Unipan, A., Clarke, J., Magee, C., Johns, J., 2018. Ultrasound diagnosis ofmolar pregnancy. Ultrasound 26 (3), 153–159.Tabas, J.A., Strehlow, M., Isaacs, E., 2003. A false negative pregnancy test in a patientwith a hydatidiform molar pregnancy. N. Engl. J. Med. 349 (22), 2172–2173.Check, J.H., Nowroozi, K., Chase, J.S., Lauer, C., Elkins, B., Wu, C.H., 1988. False-positivehuman chorionic gonadotropin levels caused by a heterophile antibody with theimmunoradiometric assay. Am. J. Obstet. Gynecol. 158 (1), 99–100.Whitehouse, K., Tschann, M., Soon, R., Davis, J., Micks, E., Salcedo, J., et al., 2019.Effects of prophylactic oxytocin on bleeding outcomes in women undergoing dilationand evacuation: a randomized controlled trial. Obstet. Gynecol. 133 (3), 484–491.Fig. 2. (A) Gross picture of bivalved (ante-roposteriorly) uterus with a uniformly, diffuselycystic cut surface (B) involving the endometrium,myometrium, and lower uterine segment.Hematoxylin and eosin (H&E) histology at 40× (C)shows diffuse cystic adenomyosis. All examinedareas of the adenomyosis shows endometrial glandswith minimal cytologic atypia and mitoses, as illu-strated in the H&E image at 200× in (D).B.S. Bedrick, et al. Gynecologic Oncology Reports 32 (2020) 1005733

Adenomyosis presenting as a molar pregnancy: A case report

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Adenomyosis presenting as a molar pregnancy: A case report

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