Adenomyosis: Common and Uncommon Manifestations on Sonography and Magnetic Resonance Imaging

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1 Image Presentation denomyosis: Common and Uncommon Manifestations on Sonography and Magnetic Resonance Imaging Sheetal Chopra, MS, DN, nna S. Lev-Toaff, MD, Fatih Ors, MD, Diane ergin, MD Objective. The purpose of this presentation is to show the imaging findings of the common and uncommon variants of adenomyosis as seen on sonography and magnetic resonance imaging (MRI). Methods. 3-year database search was performed to identify women who had pelvic sonography and pelvic MRI within a 6-month interval. Images of these cases were retrospectively reviewed. Results. Eighty women were identified. denomyosis was diagnosed on MRI, which was used as the reference standard, in 45 of these women. The correct diagnosis was made on sonography in 73% of the cases. Conclusions. wareness of the spectrum of imaging features of adenomyosis is important to use sonography effectively for diagnosing this entity and to help avoid misdiagnosis. Key words: adenomyosis; magnetic resonance imaging; transvaginal sonography. bbreviations MRI, magnetic resonance imaging; TE, echo time; TR, repetition time; TVS, transvaginal sonography Received November 17, 2005, from Thomas Jefferson University Hospital, Philadelphia, Pennsylvania US. Revision requested December 20, Revised manuscript accepted for publication January 5, ddress correspondence to nna S. Lev-Toaff, MD, Thomas Jefferson University Hospital, 111 S 10th St, 763L Main, Philadelphia, P US. [email protected] denomyosis is an often-overlooked condition that involves the endometrial-myometrial junction. It is a common nonneoplastic gynecologic disease characterized by the migration of endometrial glands from the basal layer of the endometrium into the myometrium, associated with smooth muscle hyperplasia. 1 3 The ectopic glands are located at least 2 to 3 mm below the endometrialmyometrial junction; the associated smooth muscle hyperplasia may produce asymmetric thickening of the uterus. 2 denomyosis typically appears as a diffuse disease process; however, it can also manifest as a focal lesion involving the myometrium. Focal adenomyosis is commonly mistaken for a leiomyoma. 2 Globular uterine enlargement as seen with diffuse adenomyosis is also mistaken for diffuse myomatous uterus. The purpose of this image presentation is to show the common and uncommon features of adenomyosis as seen on sonography and magnetic resonance imaging (MRI) by the merican Institute of Ultrasound in Medicine J Ultrasound Med 2006; 25: /06/$3.50

2 denomyosis on Sonography and Magnetic Resonance Imaging ackground The prevalence of adenomyosis varies widely depending on the diagnostic criteria and the thoroughness of the pathologic examination. 3 In one study, the diagnostic rate of adenomyosis in the same uteri varied from 31% to 62%, depending on the number of histologic samples obtained. 4 Published reports of the prevalence of adenomyosis in surgically removed uteri vary from 5% to 70%. 3 5 In the past, adenomyosis was diagnosed primarily on pathologic evaluation of hysterectomy specimens. With the advent of transvaginal sonography (TVS) and MRI, specific imaging features of adenomyosis have been published. It has been suggested that TVS allows the diagnosis of adenomyosis with a high degree of accuracy. 6 8 However, clinical experience suggests that the spectrum of imaging features is not as well recognized on sonography as it is on MRI. Clinical spects denomyosis affects mainly premenopausal women, particularly those who are multiparous. 1,9 Symptoms include pelvic pain, dysmenorrhea, menorrhagia, and abnormal uterine bleeding. 2,9 However, these symptoms are nonspecific and can be seen in other common gynecologic disorders such as leiomyoma and endometriosis. The clinical diagnosis of adenomyosis is fraught with error, and until the advent of TVS, the diagnosis was rarely established before surgical exploration. 8,9 Treatment for adenomyosis depends on the patient s symptoms, age, and desire to maintain fertility. Medical therapy for adenomyosis includes administration of hormones and analgesics. Conservative surgical treatment, which is not widely used in the United States, includes endomyometrial ablation, laparoscopic myometrial electrocoagulation, and local excision of the involved myometrium. 10 Hysterectomy remains the most common treatment in severe cases. 2,10 Uterine artery embolization has also been used for adenomyosis. recent study showed that 55% of treated women had clinical improvement after 2 years. 11 Given the difficulty in treating adenomyosis, it is important to recognize this condition as early as possible and to differentiate it from other conditions that it can be mistaken for, such as leiomyomas. ccurate diagnosis may help avoid unnecessary interventions, such as myomectomy undertaken for adenomyosis erroneously diagnosed as leiomyoma. 9 Pathophysiologic Mechanisms The etiology of adenomyosis is poorly understood. 1,9 Proposed mechanisms include a lack of the basement membrane or the presence of a defect in the membrane at the endometrialmyometrial interface, allowing endometrial tissue to grow into the myometrium. 2 The risk factors remain unclear, including hereditary factors, uterine trauma from childbirth or abortion, chronic endometritis, and hyperestrogenemia. 1,2 t pathologic examination, the uterus is enlarged, and the cut surface of the myometrium is trabeculated; unlike leiomyoma, a discrete nodule is not identified. 12 Microscopically, there are endometrial glands within the myometrium. The basal endometrial glands are surrounded by myometrial hypertrophy. 9 Unlike the ectopic endometrium of endometriosis, adenomyotic glands usually do not bleed cyclically because the glandular component of adenomyosis is mostly basal and unresponsive to cyclic changes. 13,14 t pathologic examination, adenomyosis is classified as superficial or deep. 14 Glands and stroma within the inner third of the myometrium characterize superficial adenomyosis; implants in the outer two thirds of myometrium define deep adenomyosis. 13 Superficial adenomyosis may be more amenable to endometrial ablation than deep adenomyosis. 15 The sensitivity of random needle biopsies in diagnosing adenomyosis is low and dependent on the number of biopsies and the depth and extent of mucosal infiltration. 16 Popp et al 16 showed that a single myometrial biopsy identified only 8% to 19% of women with adenomyosis. The sensitivity of random needle biopsy is therefore too low for clinical practice. Materials and Methods 3-year database search identified 80 women who had pelvic MRI and TVS within a 6-month interval. The images were retrospectively reviewed to assess for adenomyosis. On MRI, adenomyosis was defined by diffuse or focal thickening of the junctional zone (inner myometrium) of at least 12 mm with or without high signal intensity myometrial foci on T2-618 J Ultrasound Med 2006; 25:

3 Chopra et al weighted images, representing myometrial cysts. Criteria for adenomyosis on sonography were a globular uterus or asymmetric thickening of the anterior or posterior uterine wall, a poorly defined endometrial-myometrial junction, a focal or diffuse heterogeneous myometrial echo texture, linear striated shadowing, myometrial cysts, and poorly defined foci with an abnormal myometrial echo texture without a discrete mass to suggest a myoma. Initially, the sonograms were reviewed without knowledge of the MRI findings. Later, the MRI findings and sonograms were reviewed together, and the original radiology reports were studied. Magnetic resonance imaging was considered the reference standard for the diagnosis of adenomyosis. Results denomyosis was diagnosed on MRI in 45 of 80 women; it was diagnosed on sonography in 33 (73%) of these 45 cases with the use of the abovementioned criteria. The original interpretations of the sonograms considered adenomyosis in only 18 (40%) of the 45 cases. ll the women with adenomyosis had a history of pelvic pain, abnormal bleeding, or both. Imaging Features The ectopic endometrial glands of adenomyosis are surrounded by whorled, densely packed smooth muscle cells. This stromal reaction forms the basis of the imaging appearance of adenomyosis. 17 The reported sensitivity and specificity of TVS for diagnosing adenomyosis have ranged from 53% to 89% and 67% to 98%, respectively. 3,7,8 Magnetic resonance imaging has reported sensitivity ranging from 78% to 88% and specificity ranging from 67% to 93%. 18,19 Common Features Globular Configuration of the Uterus denomyosis is often responsible for uterine enlargement. 17 globular uterus is defined as generalized rounded enlargement of the uterus without a discrete mass. 3,14 globular configuration not explainable by the presence of leiomyomas often suggests the diagnosis of adenomyosis (Figure 1). 3 symmetric Myometrial Thickness denomyotic uteri tend to be more asymmetric than normal uteri (Figure 2). 13 symmetric thickening of either the anterior or posterior uterine wall has been used as a criterion to increase the specificity of sonography in diagnosing adenomyosis. 3 Diffuse denomyosis denomyosis typically manifests as a diffuse abnormality of the uterus. It is seen as diffuse thickening of the junctional zone (inner Figure 1. Enlarged globular uterus in a 53-year-old perimenopausal woman., Sagittal transvaginal sonogram shows a bulky retroflexed uterus with a mildly heterogeneous echo texture. The uterus measures 9.7 cm long 5 cm anteroposterior 5.2 cm wide; the endometrial echo complex (calipers) is normal., Sagittal T2-weighted fat-suppressed MRI (repetition time [TR], 4000 milliseconds; echo time [TE], 100 milliseconds) shows a thickened junctional zone (arrow) with high signal intensity myometrial cysts (arrowhead), consistent with diffuse adenomyosis. J Ultrasound Med 2006; 25:

4 denomyosis on Sonography and Magnetic Resonance Imaging Figure 2. Diffuse adenomyosis and asymmetric myometrial thickness in a 51-year-old postmenopausal woman., Sagittal transvaginal sonogram shows marked uterine enlargement with asymmetry of the myometrium. The anterior myometrium is grossly thickened compared with the posterior myometrium; the entire thickness of the anterior myometrium cannot be displayed on a single transvaginal sonogram. The endometrial echo complex (calipers) is normal. The uterus, measured transabdominally, is 15 cm long 8.5 cm anteroposterior, 10 cm wide., Sagittal T2-weighted fat-suppressed MRI (TR, 4000 milliseconds; TE, 100 milliseconds) shows marked uterine enlargement. The junctional zone is diffusely thickened (arrows) with scattered myometrial cysts, consistent with diffuse adenomyosis. There is asymmetric thickening of the anterior junctional zone corresponding to the sonographic findings. Figure 3. Heterogeneous myometrial echo texture in a 53-yearold postmenopausal woman. Sagittal transvaginal sonogram shows an enlarged retroflexed uterus with a heterogeneous myometrial echo texture (arrow). The shadowing over the lower uterus is an artifact related to vaginal air. myometrium) measuring greater than 12 mm, with low signal intensity on T2-weighted MR images. 1,6,19 The thickening of the junctional zone corresponds to the smooth muscle hyperplasia accompanying the heterotopic endometrial tissue. 14,15 On TVS, an enlarged uterus with a diffuse heterogeneous echo texture suggests the diagnosis of diffuse adenomyosis (Figure 3). 20 Decreased echogenicity or heterogeneity of the myometrium is seen in approximately 75% of patients. 15 heterogeneous appearance reflects the infiltrative process of islands of heterotropic endometrial tissue that are scattered throughout the myometrium and are poorly demarcated from the surrounding myometrium. 2,15 Myometrial Cysts These are defined as tiny round anechoic spaces (Figure 4), which correlate with fluid-filled dilated endometrial glands in the myometrium. 20 The sizes of myometrial cysts ranged from 1.0 to 5.0 mm. 3,15 Color Doppler sonography is helpful to distinguish between a myometrial cyst and a vascular component. 19 Myometrial cysts are seen as high signal intensity foci on T2-weighted MR images. 1 azot et al 19 reported high sensitivity and specificity for the detection of adenomyosis in the presence of myometrial cysts. Indistinct Endometrial-Myometrial Junction Poor definition of the endometrial-myometrial junction is one of the sonographic diagnostic criteria used for evaluation of adenomyosis. 3 However, this rather subjective criterion does not relate to the severity of adenomyosis. 20 poorly defined endometrial-myometrial junction can lead to an erroneous impression of a thickened endometrium (pseudo endometrial thickening); we have observed this in several cases on TVS (Figure 5). 620 J Ultrasound Med 2006; 25:

5 Chopra et al Figure 4. Myometrial cysts in a 48-year-old woman with a history of menorrhagia., Sagittal transvaginal sonogram shows a heterogeneous echo texture with 2 large myometrial cysts (arrows) involving the anterior and posterior uterine walls. Note that the anterior myometrium is much thicker than the posterior myometrium., Sagittal transvaginal sonogram with power Doppler imaging shows that there is no flow in the myometrial cysts (arrow). C, Sagittal T2-weighted fat-suppressed MRI shows a diffuse thickened junctional zone, consistent with adenomyosis. High signal intensity myometrial cysts (arrows) are shown, corresponding to the sonographic findings. C Figure 5. Pseudo endometrial thickening due to a poorly defined endometrial-myometrial junction in a 48-year-old premenopausal woman with menorrhagia., Sagittal transvaginal sonogram shows an enlarged uterus with a poorly defined endometrial-myometrial junction. The endometrium measured 24 mm in thickness (calipers). Endometrial biopsy had been performed 3 months earlier, and the results were negative., Sagittal fat-suppressed T2-weighted MRI shows marked diffuse thickening of the junctional zone measuring 22 mm (double-headed arrow) with high signal intensity myometrial foci (arrow) and a normal endometrium. J Ultrasound Med 2006; 25:

6 denomyosis on Sonography and Magnetic Resonance Imaging Subendometrial Linear Striations Edge shadows are often seen in extreme cases of muscle hypertrophy, presumably because of whorls of muscle bundles. 3 Lack of a distinct margin and no mass effect or a mild mass effect disproportionate to the size of the abnormal area helps differentiate adenomyosis from uterine fibroids. 3 The finding of linear striations on sonography (Figure 6) has been reported to increase the specificity of sonography in diagnosing adenomyosis. 3 Uncommon Features Focal denomyosis Focal adenomyosis is defined as a localized mass within the myometrium with poorly defined margins that blend with the surrounding myometrium (Figures 7 and 8). 1,2 Two forms of focal adenomyosis 19 are recognized: (1) adenomyoma, defined as a localized, circumscribed form of adenomyosis; and (2) adenomyosis, restricted to 1 uterine wall. Focal adenomyosis is often misdiagnosed as leiomyoma. 9,21 The differentiation between adenomyoma and leiomyoma is essential because the clinical treatment varies with each entity; leiomyoma is well demarcated from the surrounding myometrium and is readily removed at myomectomy, whereas adenomyoma is not. 10 Figure 6. Linear striations, sonographic findings suggestive of adenomyosis, in a 47-year-old woman with pelvic pain and dysmenorrhea., Sagittal transvaginal sonogram shows an enlarged uterus. heterogeneous echo texture involving the anterior myometrium is shown with linear striated shadowing (arrowheads)., Sagittal fat-suppressed T2-weighted MRI shows marked adenomyosis involving the anterior uterine wall manifested as a thickened junctional zone with scattered myometrial cysts (arrows). C, Sagittal T1-weighted gradient echo MRI (TR, 270 milliseconds; TE, 5 milliseconds) obtained after administration of gadolinium dimeglumine shows enhancement of the endometrium and myometrium. Myometrial cysts (arrows) do not show enhancement. C 622 J Ultrasound Med 2006; 25:

7 Chopra et al Figure 7. denomyoma in a 45-year-old woman with a history of irregular menstrual cycles., Sagittal transvaginal sonogram shows mild distortion of the endometrial echo complex with a bulbous area of heterogeneously hypoechoic echo texture in the fundus of the uterus (arrow)., Sagittal fat-suppressed T2-weighted MRI shows thickening of the junctional zone within the fundus of the uterus (arrows) with scattered hyperintense myometrial foci. Figure 8. Focal adenomyosis in a 37-year-old woman with a history of myomas., Sagittal transabdominal sonogram shows a large, well-defined subserosal myoma arising from the anterior uterine corpus (arrow); u indicates uterus., Sagittal transvaginal sonogram shows a poorly defined hypoechoic region (arrow) in the posterior uterine corpus, which corresponds to a focal thickening of the junctional zone seen on MRI. The endometrial echo complex (calipers) is normal. C, Sagittal fat-suppressed T2-weighted MRI shows focal adenomyosis (arrow) in the posterior myometrial wall. The remaining junctional zone is diffusely thin. large subserosal fibroid is noted anteriorly (arrowhead). C J Ultrasound Med 2006; 25:

8 denomyosis on Sonography and Magnetic Resonance Imaging denomyoma generally has a poorly defined border, and on color Doppler sonography, vascularity is seen inside the mass. In contrast, fibroids manifest as hypoechoic masses with welldefined borders. Vascularity in a fibroid is predominately at the periphery of the mass. 2,21 denomyoma may be solitary or multiple and usually arises deep in the myometrium. 22 Magnetic resonance imaging is considered highly accurate and more sensitive than TVS in differentiating adenomyoma from fibroids. 1,2 Polypoid denomyoma n adenomyoma is occasionally located in a submucosal or subserosal position. submucosal adenomyoma manifesting as a polypoid mass protruding into the endometrial cavity has been termed polypoid adenomyoma. 9,23 This lesion usually arises from the uterine corpus, occasionally from the lower uterine segment and cervix. 9,23 It can be seen as a pedunculated (Figure 9) or sessile mass. 9 Its appearance on MRI as an uncommon variant of adenomyosis has been described. 9 Use of the term polypoid adenomyoma has been controversial. lthough some authors have discussed this lesion as a distinct entity, 24 the gynecologic pathologic literature reflects the opinion that the term polypoid adenomyoma refers to a polypoidal form of a circumscribed adenomyotic mass ,25 typical Polypoid denomyoma This is an uncommon variant of polypoid adenomyoma characterized by atypical endometrial glands and often squamous metaplasia and a cellular smooth muscle stroma. 23 efore its delineation, this lesion was often considered malignant. 23 The distinction between the typical and atypical adenomyoma depends on histologic examination because the radiologic findings are similar. 9 Figure 9. Polypoid adenomyomas., Sagittal transvaginal sonogram in a 30-year-old woman with a history of menometrorrhagia shows an indistinct endometrial echo complex with variable thickness; a cyst is shown adjacent to it (arrow). Sonohysterography was performed for further evaluation of these findings., Sagittal image from the sonohysterogram reveals an intracavitary mass containing a cystic focus (arrow). The echo texture of this mass, unlike an endometrial polyp, is similar to that of the myometrium. No endometrial abnormality was seen. C, Transvaginal sonogram in a 39-year-old woman with menorrhagia shows a large prolapsing adenomyoma distending the cervical canal (arrow). The echo texture of the mass is similar to that of the myometrium; it is, therefore, sonographically indistinguishable from a myoma. The arrowhead points to the posterior vaginal wall. C 624 J Ultrasound Med 2006; 25:

9 Chopra et al denomyotic Cyst n adenomyotic cyst is an extremely rare variation of adenomyosis caused by extensive bleeding into the ectopic endometrium. 9,26 The lesion can be entirely within the myometrium (Figure 10), submucosal, or subserosal. t MRI, fluid content has a high signal intensity on T1-weighted images, indicating hemorrhage, surrounded entirely or partly by a hypointense solid wall on T2-weighted images. 9,26 In the presence of multiple myomas, especially central myomas, the sensitivity of sonography for diagnosing adenomyosis was lower In such cases, MRI may prove beneficial, especially before surgical intervention. In our experience, even diffuse adenomyosis was difficult to detect when associated with multiple myomas (Figure 11). Conclusions denomyosis remains under-recognized on TVS in routine clinical practice. However, when specific criteria are sought, the performance of TVS is improved, and the detection of adenomyosis increases. The most common form of adenomyosis is diffuse disease. The focal form is less common and presents a diagnostic challenge on both TVS and MRI. wareness of the typical and atypical imaging features of adenomyosis is important to avoid misdiagnosis. Magnetic resonance imaging is useful for confirming adenomyosis when suspected on TVS, especially when surgical treatment is contemplated. Magnetic resonance imaging is also helpful in the presence of multiple or central myomas, which hinder sonographic diagnosis. Figure 10. Cystic adenomyoma in a 37-year-old woman with a history of infertility., Multiplanar display from 3D TVS of the uterus shows a cystic mass within the right side of the uterine corpus containing low-level echoes. The bottom left image is a coronal plane through the endometrial cavity showing 2 normal cornua. Top left, axial; top right, sagittal; bottom left, coronal., xial T1-weighted gradient echo opposed phase MRI (TR, 225 milliseconds; TE, 2 milliseconds) shows a hyperintense lesion within the myometrium. Lack of a chemical shift artifact around the lesion confirms the absence of fat within. C, xial T2-weighted MRI (TR, 4000 milliseconds; TE, 100 milliseconds) image shows an intermediate to hyperintense signal in the cystic mass (arrow), showing shading, consistent with chronic hemorrhage. C J Ultrasound Med 2006; 25:

10 denomyosis on Sonography and Magnetic Resonance Imaging Figure 11. Multiple fibroids and diffuse adenomyosis in a 50-year-old woman., Sagittal transabdominal sonogram shows a bulky uterus due to multiple fibroids. The endometrial cavity is distorted by the presence of fibroids (arrows)., Sagittal T2-weighted MRI shows diffuse adenomyosis with marked thickening of the junctional zone (arrow) and the presence of multiple fibroids (arrowheads). In the presence of large multiple fibroids, diagnosing adenomyosis on sonography can be a difficult task. References 1. yun JY, Kim SE, Choi G, Ko GY, Jung SE, Choi KH. Diffuse and focal adenomyosis: MR imaging findings. Radiographics 1999; 19: Kuligowska E, Deeds L III, Lu K III. Pelvic pain: overlooked and underdiagnosed gynecologic conditions. Radiographics 2005; 25: tri M, Reinhold C, Mehio R, Chapman W, ret PM. denomyosis: US features with histologic correlation in an in-vitro study. Radiology 2000; 215: ird CC, McElin TW, Manalo-Estrella P. The elusive adenomyosis of the uterus-revisited. m J Obstet Gynecol. 1972; 112: Siedler D, Laing FC, Jeffrey R Jr, Wing VW. Uterine adenomyosis: a difficult sonographic diagnosis. J Ultrasound Med 1987; 6: Dueholm M, Lundorf E, Hansen ES, Sorensen JS, Ledertoug S, Olesen F. Magnetic resonance imaging and transvaginal ultrasonography for the diagnosis of adenomyosis. Fertil Steril 2001; 76: Reinhold C, tri M, Mehio, Zakarian R, ldis E, ret PM. Diffuse uterine adenomyosis: morphologic criteria and diagnostic accuracy of endovaginal sonography. Radiology 1995; 197: Reinhold C, McCarthy S, ret PM, et al. Diffuse adenomyosis: comparison of endovaginal US and MR imaging with histopathologic correlation. Radiology 1996; 199: Tamai K, Togashi K, Ito T, Morisawa N, Fujiwara T, Koyama T. MR imaging findings of adenomyosis: correlation with histopathologic features and diagnostic pitfalls. Radiographics 2005; 25: Wood C. Surgical and medical treatment of adenomyosis. Hum Reprod Update 1998; 4: Pelage JP, Jacob D, Fazel, et al. Midterm results of uterine artery embolization for symptomatic adenomyosis: initial experience. Radiology 2005; 234: Zaloudek C, Norris HJ. Mesenchymal tumors of the uterus. In: Kurman RJ (ed). laustein s Pathology of the Female Genital Tract. 3rd ed. New York, NY: Springer-Verlag; 1987: rosens JJ, de Souza NM, arker FG, Paraschos T, Winston RM. Endovaginal ultrasonography in the diagnosis of adenomyosis uteri: identifying the predictive characteristics. r J Obstet Gynaecol. 1995; 102: Outwater EK, Siegelman ES, Van Deerlin V. denomyosis: current concepts and imaging considerations. JR m J Roentgenol 1998; 170: Reinhold C, Tafazoli F, Mehio, et al. Uterine adenomyosis: endovaginal US and MR imaging features with histopathologic correlation. Radiographics 1999; 19: Popp LW, Schwiedessen JP, Gaetje R. Myometrial biopsy in the diagnosis of adenomyosis uteri. m J Obstet Gynecol 1993; 169: Togashi K, Ozasa H, Konishi I, et al. Enlarged uterus: differentiation between adenomyosis and leiomyoma with MR imaging. Radiology 1989; 171: scher SM, rnold LL, Patt RH, et al. denomyosis: prospective comparison of MR imaging and transvaginal sonography. Radiology 1994; 190: azot M, Cortez, Darai E, et al. Ultrasonography compared with magnetic resonance imaging for the diagnosis of adenomyosis: correlation with histopathology. Hum Reprod 2001; 16: J Ultrasound Med 2006; 25:

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