Benign Pathology of Myometrium - Pictorial Review and Clues to Expanding the Differential Diagnosis

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1 Benign Pathology of Myometrium - Pictorial Review and Clues to Expanding the Differential Diagnosis Poster No.: C-1953 Congress: ECR 2016 Type: Educational Exhibit Authors: T. Dionisio, G. Rio, H. S. G. Torrao, V. Mendes, C. P. Vaz ; Braga/PT, , Po/PT, Guimarães/PT Keywords: Education and training, Education, Diagnostic procedure, Ultrasound, MR, CT, Pelvis, Genital / Reproductive system female DOI: /ecr2016/C-1953 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 41

2 Learning objectives - To provide a pictorial review of benign myometrial lesions with pathologic correlation; - To review the clinical manifestations, radiological findings and differential diagnosis of benign myometrial pathology. Background - Although leiomyomas are the most frequent benign tumors of the myometrium, benign pathology of the myometrium includes several other entities; - They should be considered as differential adenomyosis, lipoleiomyomas and uterine contractions. - Moreover, it is important to be able to distinguish these conditions from endometrial and extrauterine disease, in the case of submucosal and suberosal lesions, respectively. - Sometimes the differential diagnosis between all these entities may become a challege. - Furthermore malignancy, such as leiomyosarcoma, must be excluded pre-surgically. - MRI should be the method of choice, as it offers superior contrast and spatial resolution besides the capacity to depict the uterine anatomy. Findings and procedure details MRI Protocol - Benign Myometrial Pathology In our service, it is performed routinely the following protocol for suspected or follow-up of benign lesions of the myometrium: Pelvis Sequences - Axial T1 ( 5mm) Page 2 of 41

3 - Axial T2 ( 5mm) Specific Sequences to the Uterus - Sagittal T2 Body Uterus (4mm ) - Coronal T2 Body Uterus (4mm ) - Axial T2 Body Uterus (4mm ) Post Gadolinium Study - Dynamic study - T1 * SPAIR - usually sagittal plane ( 5 purchases up to 150 sec. ) (2mm ) Normal 0 false false false PT JA X-NONE - Late acquisition - T1 SPIR - usually sagittal and/or axial plane (4mm ) Normal Myometrium - Anatomy - The uterus is divided into three layers - endometrium, myometrium, and serosa or perimetrium. - The middle layer, or myometrium, is the muscular layer and it makes up most of the uterine volume. - The myometrium is composed of mainly smooth muscle cells - It can be separated into: (1) the inner myometrium, or junctional zone (2) the outer myometrium. Page 3 of 41

4 Fig. 1: Normal Myometrium Anatomy References: Serviço de Radiologia, Hospital de Braga - Braga/PT - The junctional zone contains compact smooth muscle with little intercellular matrix, and has a relatively low T2 signal intensity - The outer myometrium has more intercellular matrix and vessels, and less compact smooth muscle, resulting in a higher T2 signal intensity. - These T2 signal intensity differences are best visualized during the reproductive years (Fig. 1). - T1-weighted images typically show poor contrast distinction between the endometrium and the myometrium. LEIOMYOMAS - Benign tumours of myometrial origin Page 4 of 41

5 - The most common solid benign uterine neoplasm - The most common tumors of the female genital tract (~25% of women of reproductive age) - A number of factors are thought to contribute to leyomiomas development, including genetic mutations and ethnicity - Particularly common in the African population - A major risk factor is exposure to unnopposed oestrogens: *Obesity *Early Menarche *Late menopause *Nulliparity *Use of synthetic oestrogens (such as Tamoxifen in breast cancer treatment) - As leiomyomas enlarge, they may outhgrow their blood supply, resulting in different types of degeneration: *cystic degeneration *hyaline or myxoid degeneration *calcification *hemorrhage (red degeneration) Ultrasound - Usually hypoechoic (but can be isooechoic or hyperechoic compared to normal myometrium) - Calcification is seen as echogenic foci with shadowing - Cystic areas of necrosis or degeneration may be seen MRI - T1 Page 5 of 41

6 *non-degenerated fibroids and calcification appear as low to intermediate signal intensity compared with the normal myometrium - T2 *non-degenerated fibroids and calcification appear as low signal intensity *fibroids that have undergone cystic degeneration/necrosis can have a variable appearance, usually appearing high signal on T2 sequences. - T1 C+ (Gd) *variable enhancement is seen with contrast administration *marked high signal intensity with gradual enhancement suggests myxoid degeneration Fig. 3: Typical aspect of leyomioma on MRI. References: Serviço de Radiologia, Hospital de Braga - Braga/PT Page 6 of 41

7 LEIOMYOMA CLASSIFICATION - Leiomyomas may be classified according to their location, as: *subserosal (beneath the serosa) *intramural (within the substance of the myometrium) *submucosal (projecting into the endometrial cavity) (Fig. 2) Fig. 2: Leiomyomas Classification References: Serviço de Radiologia, Hospital de Braga - Braga/PT - Leiomyomas can involve the cervix (~8%), mas most frequently occur in uterine corpus. SYMPTOMS - Commonly leiomyomas are an incidental finding and are asymptomatic Page 7 of 41

8 % of leiomyomas are symptomatic - symptoms are variable according to the size, location and number os tumors - The most common symptoms are menorrhagia, dysmernorrhea, pressure, urinary freqyency, pain, infertility ou a palpable mass CYSTIC DEGENERATION - Represents ~ 4% of all types of degeneration - Ultrasound shows a hypoechoic or heterogenous uterine mass with cystic areas - MRI reveals a mass with portions of high T2 signal Fig. 4: Leiomyoma with cystic degeneration References: Serviço de Radiologia, Hospital de Braga - Braga/PT Page 8 of 41

9 CALCIFICATION - Seen in approximately 4% of fibroids - Can be confined to the periphery of the fibroid, when it is thought to be secondary to thrombosed veins from previous red degeneration - Calcific degeneration is difficult to distinguish from non-degenerated fibroids on MRI - Areas of calcification can appear as signal voids on MRI Fig. 5: Leiomyoma with calcification References: Serviço de Radiologia, Hospital de Braga - Braga/PT MYXOIDE DEGENERATION - Generally considered uncommon, although reported as high a 50% by some authors - They are filled with a gelatinous material - It can be difficult to differentiate from fibroids that have undergone cysric degeneration - They typically appear as complex cystic masses. Page 9 of 41

10 HYALINE DEGENERATION - It occurs in up to 60% of uterine leiomyomas - It happens when fibroids outgrow their blood supply - As happens with calcifications, hyaline degeneration are difficult to distinguish from nondegenerated fibroids on MRI - Amongst the background low T2 signal of non myxoid components, there are areas of which typical characteristics include: *T2: low signal *T1 C+ (Gd): no enhancement CARNEOUS DEGENERATION - It is the most common form of degeneration of a leiomeyoma during pregnancy (it is a subtype of haemorrhagic infarction of leiomyomas that often occurs during pregnancy) - Patients can present with abdominal pain (particularly during pregnancy), fever and leucocytosis MRI Unusual signal intensity patterns have been described - T1 : *can have peripheral or diffuse high signal intensity *the high signal intensity on T1-weighted images is likely secondary to the proteinaceous content of the blood or the T1-shortening effects of methemoglobin - T2 : *variable signal intensity with or without a low-signal-intensity rim. *when it shows perihperal changes, it may correspond with dilated vessels filled with red blood cells at the periphery of the lesion Page 10 of 41

11 LIPOLEIOMYOMA - Rare benigne tumour if the uterus (incidence of %) - Typically found primarily in obese perimenopausal and postmenopausal patients with typical uterine leiomyomas - Most are asymptomatic - Can cause symptoms similar to classic leiomyomas depending on their size and location (pelvic pain, palpable mass and menstrual abnormalities) - Etiology is not well known, but it is suggested that lipoleiomyomas result from fatty metamorphosis of uterine smooth muscle cells which can proceed to form localized or diffuse mature adipocyte tissue in leiomyoma or in the myometrium rather than fatty degeneration - The tumor consists of long intersecting bundles of bland, smooth muscle cells admixed with nests of mature fat cells and fibrous tissue. - Main differential diagnosis: benign cystic teratoma, malignant degeneration of cystic teratoma, non-teratomatous lipomatous ovarian tumor, benign pelvic lipomas, liposarcomas and lipoblastic lymphadenopathy. - Ultrasound shows a hyperechoic mass with posterior accoustic attenuation - On MRI: T1: hyperintensity T1 FS: hypointensity (saturates out) T2: hyperintensity T2 FS or STIR: hypointensity (saturates out) Page 11 of 41

12 Fig. 6: Lipoleiomyoma References: Serviço de Radiologia, Hospital de Braga - Braga/PT UTERINE CONTRACTION - IT can appear as a myometrial mass that has low signal intensity on T2W imaging and that bulges into the endometrial cavity - The low signal intensity on T2W imaging is secondary to decreased water content in the area of contracted myometrium - Diagnosis is easily made, because myometrial contraction is a transient phenomenon and resolves on subsequent sequences Page 12 of 41

13 Fig. 7: Myometrial Contraction References: Serviço de Radiologia, Hospital de Braga - Braga/PT UTERINE ARTERIOVENOUS MALFORMATION - Can be congenital ou acquired - Acquired UAVMs disease are associated with: *multiple pregnancies *miscarriage *previous surgery (dilation and curettage, termination of pregnancy or caesarean section) Ultrasound: - Areas of subtle myometrial inhomogeneity - Tubular spaces within the myometrium - A intramural uterine, endometrial or cervical mass like region Page 13 of 41

14 - Prominent parametrial vessels - Color Doppler shows serpiginous anechoic structures within the myometrium with a low resistance (RI ~ ) MRI: - Multiple serpentine flow-related signal voids typically seen in the uterine wall, endometrial cavity, and parametrium on T1 and T2 weighted images - Contrast-enhanced dynamic MR angiography shoes complex serpentine abnormal vessels that enhance as intensely as normal vessels and show early venous return Fig. 8: Uterine Arteriovenous Malformation References: Serviço de Radiologia, Hospital de Braga - Braga/PT ADENOMYOSIS Page 14 of 41

15 - Defined by the presence of ectopic endometrial glands and stroma within the myometrium - Typically affects women of reproductive age and multiparous - More frequent in woman with a history of surgical uterine procedures (caesarian section, dilatation and curettage) - Typically present with menorrhagia and dysmenorrhea or chronic pelvic pain - In 20% of cases is associated with endometriosis Ultrasound - Uterine enlargement - Cystic anechoic spaces or lakes in the myometrium - Uterine wall thickening (anteroposterior asymmetry) - Subendometrial echogenic linear striations - Heterogeneous echo texture - Obscure endometrial/myometrial border - Thickening of the transition zone (thickness of 12 mm or greater has been shown to be associated with adenomyosis) MRI - The most easily recognised feature is thickening of the junctional zone of the uterus to more than 12 mm (diffusely or focally) - T2 * ill-defined region of thickening, often with small high T2 signal regions representing small regions of cystic change *may also have a striated appearance - T1 *foci of high T1 signal are often seen, indicating menstrual haemorrhage into the ectopic endometrial tissues Page 15 of 41

16 - T1 C+ (Gd) *it is usually not indicated for evaluation of adenomyosis *if performed, it shows enhancement of the ectopic endometrial glands Fig. 9: Adenomyosis References: Serviço de Radiologia, Hospital de Braga - Braga/PT ADENOMYOMA - Focal region of adenomyosis resulting in a mass Page 16 of 41

17 - It is difficult to distinguish from a leiomyoma Ultrasound - May be seen as a focal area of myometrical thickening - It may also present as a heterogeneous focal nodule with indistinct margins and cystic spaces MRI - Localized, low-signal-intensity region within the myometrium on both T2-weighted and contrast-enhanced T1-weighted sequences - This region is often continuous with the junctional zone Page 17 of 41

18 Fig. 10: Adenomyoma References: Serviço de Radiologia, Hospital de Braga - Braga/PT DIFFERENTIAL DIAGNOSIS WITH OTHER THAN BENIGN MYOMETRIAL LESIONS LEIOMYOSARCOMA - > 1/3 of uterine sarcomas but only 1.3% of all uterine cancers - The uterus is the commonest location for a leiomyosarcoma Page 18 of 41

19 - Usually present in women in the 5th decade - They are thought to arise de novo (incidence of sarcomatous transformation in benign uterine leiomyomas is reported to be %) - Leiomyosarcomas are often difficult to differentiate from leiomyomas, based on clinical features and even endometrial biopsy or dilatation and fractional curettage. - Recent noinvasive or minimally invasive techiques in leiomyoma management have raised the importance of pretreatment imaging diagnosis of uterine sarcomas - Generally larger and show more rapid growth than leiomyomas MRI - Uterus is often massively enlarged - An irregular margin of a uterine leiomyoma on MRI is suggestive of sarcomatous transformation, but this is not a specific signal! - T1-weighted images: Large infiltrating myometrial mass of heterogeneous hypointensity with irregular and ill-defined margins - T2-weighted images: intermediate-to-high signal intensity, with central hyperintensity indicative of extensive necrosis (present in >50% of cases) - Hemorrhage is common - Foci of calcifications may be present - After contrast administration: early heterogeneous enhancement, due to the areas of necrosis and hemorrhage Tips to Differential Diagnosis The most suggestive features of malignancy are: - Presence of irregular margins - Necrosis - Rapid growth Page 19 of 41

20 Fig. 11: Leiomyoma vc leiomyosarcoma References: Serviço de Radiologia, Hospital de Braga - Braga/PT ADNEXAL LESIONS Page 20 of 41

21 Fig. 12: Leiomyoma vc adnexal lesion References: Serviço de Radiologia, Hospital de Braga - Braga/PT Fig. 13: Leiomyoma vc adnexal lesion Page 21 of 41

22 References: Serviço de Radiologia, Hospital de Braga - Braga/PT Fig. 14: Leiomyoma vc adnexal lesions References: Serviço de Radiologia, Hospital de Braga - Braga/PT Fig. 16: Leiomyoma vc adnexal lesion Page 22 of 41

23 References: Serviço de Radiologia, Hospital de Braga - Braga/PT Fig. 17: Leiomyoma vc adnexal lesion References: Serviço de Radiologia, Hospital de Braga - Braga/PT Fig. 18: Leiomyoma vc adnexal lesion References: Serviço de Radiologia, Hospital de Braga - Braga/PT Page 23 of 41

24 ENDOMETRIAL LESIONS Fig. 15: Leiomyoma vc endometryal lesion References: Serviço de Radiologia, Hospital de Braga - Braga/PT Images for this section: Page 24 of 41

25 Fig. 1: Normal Myometrium Anatomy Serviço de Radiologia, Hospital de Braga - Braga/PT Page 25 of 41

26 Fig. 2: Leiomyomas Classification Serviço de Radiologia, Hospital de Braga - Braga/PT Page 26 of 41

27 Fig. 3: Typical aspect of leyomioma on MRI. Serviço de Radiologia, Hospital de Braga - Braga/PT Page 27 of 41

28 Fig. 4: Leiomyoma with cystic degeneration Serviço de Radiologia, Hospital de Braga - Braga/PT Fig. 5: Leiomyoma with calcification Page 28 of 41

29 Serviço de Radiologia, Hospital de Braga - Braga/PT Fig. 6: Lipoleiomyoma Serviço de Radiologia, Hospital de Braga - Braga/PT Page 29 of 41

30 Fig. 7: Myometrial Contraction Serviço de Radiologia, Hospital de Braga - Braga/PT Page 30 of 41

31 Fig. 8: Uterine Arteriovenous Malformation Serviço de Radiologia, Hospital de Braga - Braga/PT Page 31 of 41

32 Fig. 9: Adenomyosis Serviço de Radiologia, Hospital de Braga - Braga/PT Page 32 of 41

33 Fig. 10: Adenomyoma Serviço de Radiologia, Hospital de Braga - Braga/PT Page 33 of 41

34 Fig. 11: Leiomyoma vc leiomyosarcoma Serviço de Radiologia, Hospital de Braga - Braga/PT Page 34 of 41

35 Fig. 12: Leiomyoma vc adnexal lesion Serviço de Radiologia, Hospital de Braga - Braga/PT Page 35 of 41

36 Fig. 13: Leiomyoma vc adnexal lesion Serviço de Radiologia, Hospital de Braga - Braga/PT Fig. 14: Leiomyoma vc adnexal lesions Serviço de Radiologia, Hospital de Braga - Braga/PT Page 36 of 41

37 Fig. 15: Leiomyoma vc endometryal lesion Serviço de Radiologia, Hospital de Braga - Braga/PT Fig. 16: Leiomyoma vc adnexal lesion Page 37 of 41

38 Serviço de Radiologia, Hospital de Braga - Braga/PT Fig. 17: Leiomyoma vc adnexal lesion Serviço de Radiologia, Hospital de Braga - Braga/PT Page 38 of 41

39 Fig. 18: Leiomyoma vc adnexal lesion Serviço de Radiologia, Hospital de Braga - Braga/PT Page 39 of 41

40 Conclusion - Leiomyomas are the commonest uterine neoplasms; - Althought they are frequently assymptomatic, they may cause pelvic pain, menometrorrhagia and infertility and are the commonest indication for hysterectomy; - To know the differential diagnoses of benign pathology of the myometrium and their respective ultrasound and MRI findings, becomes indispensable, considering that leiomyomas may mimic a range of other pelvic disorders; - MRI is the method of choice, but radiologists should be aware of typical and unusual radiological findings of the different benign myometrial lesions and their mimics, to enable prompt diagnosis and and correct therapeutic strategy. Personal information References (1) Manjunatha HK, Ramaswamy AS, Kumar BS, Kumar SPA, Krishna L. Lipoleiomyoma of uterus in a postmenopausal woman. Journal of Mid-Life Health. 2010;1(2): doi: / (2) Santos P, Cunha TM. Uterine sarcomas: clinical presentation and MRI features. Diagn Interv Radiol Oct 28. (3) Sala E, Wakely S, Senior E, Lomas D. American Jounral of Roentgenology. 2007; 188: (4) Al-Shekaili KR, Bhatnagar G, Ramadhan FA, Al-Zadjali N. Arteriovenous malformation of uterus. Indian J Pathol Microbiol 2011, 54: Page 40 of 41

41 (5) Teixeira Arildo Corrêa, Urban Linei A. B. D., Zapparoli Mauricio, Pereira Caroline, Millani Thaís Cristina Cleto, Passos Ana Paula. Degenerating cystic uterine fibroid mimics an ovarian cyst in a pregnant patient: a case report. Radiol Bras [Internet] Aug (6) Sakhel K, Abuhamad A. J Ultrasound Med 2012; 31: Page 41 of 41

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