Chronic granulomatous mastitis: An unusual culprit

Transcription

1 Chronic granulomatous mastitis: An unusual culprit Poster No.: C-1389 Congress: ECR 2014 Type: Educational Exhibit Authors: S. Hari, S. Pahwa, N. A. Faizi, H. A. Venkatesh; NEW DELHI/IN Keywords: Inflammation, Diagnostic procedure, Ultrasound, MR, Mammography, Breast DOI: /ecr2014/C-1389 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 16

2 Learning objectives The purpose of our educational exhibit is to: Illustrate the clinical, imaging and pathologicfeatures of chronic granulomatous mastitis (CGM) Describe the spectrum of radiologic findings seen in chronic granulomatous mastitis on mammography, ultrasound (including Shear Wave ElastographySWE) and magnetic resonance imaging (MRI). Describe imaging criteria that help to differentiate CGM from breast cancer, which it mimics clinically and on imaging. Create awareness of this entity amongst young radiologists, surgeons and pathologists and discuss management options. Background Introduction Chronic granulomatous mastitis (CGM), also termed as granulomatous lobular mastitis or granulomatous lobulitis, is a rare, benign,chronic inflammatory disease of the breast, characterized by granulomatous inflammation of the breast lobules.this entity was first described by Kessler and Wolloch in 1972(1). The incidence of this condition is uncertain withonly a few hundred cases reported in the published literature(2). It is important to be aware of CGM as it resembles breast cancerclinically and on imaging. Epidemiology Chronic Granulomatous Mastitis most commonly involves women of child-bearing age, althougha few cases have also been reported in male patients (3). A number of risk factors likeuse of oral contraceptive pills, pregnancy, breast feeding, autoimmune origin, infection with yet unidentified pathogens, breast trauma, hyperprolactinemia, and alpha-1-antitrypsin deficiency have been described, but adefinite etiology has not yet been proven (4). Granulomatous inflammation may be idiopathic (an entity well described in the Western literature), or secondary to other breast diseases as periductal mastitis, tubercular or fungal infections, ruptured cysts, fat necrosis and systemic disorders as sarcoidosis, Wegener's granulomatosis (Figure 1). Most of the cases in developing countries areeither due to breast infections that persist because of inadequate antibiotic therapy or due to endemic infections as tuberculosis. Hence CGM secondary to other causes is not uncommon in the developing world(5,6). Clinical features Page 2 of 16

3 Patients with CGM often present with a hard, palpable breast mass that has been present for a few months, with or without erythematous skin changes. Other symptoms may include pain, skin thickening, sinus formation, nipple retraction and axillary lymphadenopathy (2,7).The disease is classically unilateral, although bilateral involvement has been reported in the literature(4). As the clinical symptoms are of long duration and a hard lump is found on examination, a provisional diagnosis of carcinoma breast is usually made. Images for this section: Fig. 1: Chronic granulomatous mastititis (CGM): etiology Page 3 of 16

4 Findings and procedure details Imaging studies Mammography A number of imaging appearances have been described. A large focal asymmetric density or an irregular ill-defined mass are the most common findings (7) (Figure 2). Multiple small masses, architectural distortion (Figure 7), diffuse increase in the breast density, skin thickening and nipple retraction may also be seen. Mammograms could even be normal, depending on the size and location of the CGM lesion at the time of diagnosis and the density of the surrounding breast parenchyma(2,7). Microcalcification is highly unusual, though cases have been reported in the literature(8). Ultrasound The most common appearanceis an irregular hypoechoic mass associated with multiple fingerlike extensions. Other frequent appearances include single or multiple masses (Figure 8), parenchymal distortion with or without acoustic shadowing and multifocal abscess cavities. Axillary lymphadenopathy and skin thickening are seen in almost 50% of the patients(2,5,7). Fluid clefts or tubular hypoechoic extension in an ill-defined breast lesion are highly suggestive of chronic inflammation in a woman of reproductive age group (Figure 3). Shear Wave Elastography Shear Wave Elastography is very helpful in differentiating CGM from carcinoma. The CGM lesions as well as perilesional stroma are soft on elastography with maximum elastography values less 80 kpa (Figure 4), as is expected for benign lesions(9,10). On the other hand, elastography values for malignant masses are higher. MRI The signal intensity is variable on non-contrastt1w and T2W images.however, T2W hyperintensity points towards a benign etiology (2). Although non-specific, a variety of appearances have been described for CGM on contrast administration: areas of non-mass likeenhancement (segmental or regional), nodularlesions, lesions with peripheral ring like enhancement, homogeneous / heterogeneous enhancing masses or parenchymal distortion(2).the most characteristic finding of CGM is lesions with peripheral, ring-like enhancement that represent micro-abscesses within the breast (Figure 5). Lesions show variable contrastkinetics with some lesions showing rapid enhancement and others showing progressive enhancement (Figure 5). Establishing the diagnosis Page 4 of 16

5 Although radiological findings can suggest the diagnosis of CGM in an appropriate clinical setting, the imaging findings are not specific and the diagnosis is best established by histopathology. Tissue biopsy is necessary to rule out malignancy and a stereotactic, ultrasound-guided or vacuum-assisted biopsy can be performed. Image guidance again plays a crucial role here to get adequate tissue samples from representative areas. These samples may be examined for acid fast bacilli and sent for culture. Fluid from the clefts or abscesses can be aspirated forpolymerase chain reaction (PCR) for Mycobacterium tuberculosisdna and also for culture. A general approach to a patient of reproductive age group presenting with a hard, breast lump of long duration is depicted in Figure 9. Histopathology Hematoxylin and Eosin (H&E) stains are used for the histologic analysis. Chronic granulomatous inflammation centered on the lobules is the characteristic finding in granulomatous mastitis. Microabscesses, epithelioid macrophages and multinucleated giant cells (Figure 6) may be demonstrated in tubercular or other chronic infections. Presence of acid fast bacilli, a positive PCR test for M tuberculosis or a positive culture confirms the diagnosis of tuberculosis. Idiopathic CGM is a diagnosis of exclusion and is characterized by presence of non caseatinggranulomas and absence of other systemic illnesses. Treatment Management of CGM is conservative and surgery is reserved for resistant cases. Long antibiotic course is prescribed for cases with pyogenic etiology(2,6). Anti-tubercular drug regimen should be initiated for tubercular granulomatous mastitis. The idiopathic variety and secondary CGM caused by connective tissue diseases can be treated by steroids (5,7) An approach to management of a patient with CGM according to etiology is discussed in Figure 10. Complication and follow up Recurrence is a well-known complication(2). Even a breast carcinoma probably arising from CGM has also been reported in the literature (11). Hence long term follow up is needed. After the initial episode,follow up ultrasound should be performed every 6 months until the disease has resolved. Surveillance with annual mammography is also recommended(2). Images for this section: Page 5 of 16

6 Fig. 2: Mammogram of a 35 year old lady with a hard lump in right breast since 5 months: Mediolateral oblique (MLO) and craniocaudal (CC) reveal asymmetric density in upper, outer quadrant of right breast (arrows). Page 6 of 16

7 Fig. 3: Ultrasound of the same patient as in Figure 2:Extended field of view image (top)depicts ill defined areas of increased echogenicity in the right breast with presence of fluid clefts within(arrows). B mode ultrasound images (bottom)show a round, echogenic mass surrounded by a fluid cleft (curved arrow)- no posterior acoustic shadowing is seen. Page 7 of 16

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9 Fig. 4: Ultrasound doppler (top) and Shear Wave Elastography (SWE)image (bottom) of the same patient as in Figure 2: Internal vascularity is not seen within the lesion, but is increased in the surrounding stroma. SWE image (bottom) reveals that the perilesional stroma is soft (elastography value- less than 80 kpa). Fig. 5: Dynamic contrast enhanced MRI of the same patient as in Figure 2: Multiple ill defined enhancing lesions are seen in the background of enhancing parenchyma in outer half of right breast- one of the lesions is showing peripheral rim enhancement suggestive of a small abscess (arrow). The lesions showed progressive, plateau type of enhancement (Type II enhancement curve) on dynamic post contrast MRI (left). Page 9 of 16

10 Fig. 6: Hematoxylin and Eosin (H and E) staining of tissue biopsy sample from same patient as in Figure 2. Lobulocentric inflammation and granulomas with multinucleated giant cells are seen (arrows). Culture for Mycobacterium tuberculosis was positive and a diagnosis of tubercular chronic granulomatous mastitis was made. Page 10 of 16

11 Fig. 7: Mammogram of a 37 year old lady with multiple episodes of pain and redness in the lower half of right breast: multiple small high density masses are seen in left breast causing architectural distortion (arrows) Page 11 of 16

12 Fig. 8: Ultrasound images of the same patient as in Figure 7: multiple small echogenic masses are present (arrows)with increased vascularity in adjacent breast parenchyma. No posterior acoustic shadowing is seen behind these masses Page 12 of 16

13 Fig. 9: Approach to diagnosis in a patient of reproductive age group presenting with a hard breast lump of chronic duration Page 13 of 16

14 Fig. 10: An approach to management of a patient with CGM according to etiology. Page 14 of 16

15 Conclusion Conclusion Chronic granulomatous mastitis is a benign chronic inflammatory process. Though the idiopathic variety is very rare, CGM due to periductal mastitis, poorly treated pyogenic mastitis, and chronic tubercular or fungal infections is common in developing countries. The clinical and radiologicalfindings are commonly mistaken for. The radiologist plays a crucial role as he / she may be the first one to suggest the diagnosis, and may also acquire tissue samples for pathology from representative areas under image guidance. Histopathological confirmation of CGM combined with exclusion of malignancy and other causes of granulomatous disease is of utmost importance in guiding clinical decision making and preventing inappropriate and unnecessary mastectomies. Personal information References References 1. Kessler E, Wolloch Y. Granulomatous mastitis: a lesion clinically simulating carcinoma. Am J ClinPathol Dec;58(6): Gautier N, Lalonde L, Tran-Thanh D, Khoury ME, David J, Labelle M, et al. Chronic granulomatous mastitis: Imaging, pathology and management. Eur J Radiol Apr;82(4):e165-e Reddy KM, Meyer CER, Nakdjevani A, Shrotria S. Idiopathic granulomatous mastitis in the male breast. Breast J Feb;11(1): Pistolese CA, Di Trapano R, Girardi V, Costanzo E, Di Poce I, Simonetti G. An unusual case of bilateral granulomatous mastitis. Case Rep Radiol. 2013;2013: Seo HRN, Na KY, Yim HE, Kim TH, Kang DK, Oh KK, et al. Differential diagnosis in idiopathic granulomatous mastitis and tuberculous mastitis. J Breast Cancer Mar;15(1): Letourneux C, Diemunsch P, Korganow A-S, Akladios CY, Bellocq J-P, Mathelin C. First report of granulomatous mastitis associated with Sjögren's syndrome. World J SurgOncol. 2013;11(1):268. Page 15 of 16

16 7. Larsen LJH, Peyvandi B, Klipfel N, Grant E, Iyengar G. Granulomatous Lobular Mastitis: Imaging, Diagnosis, and Treatment. Am J Roentgenol Aug;193(2): Limaiem F, Korbi S, Tlili T, Haddad I, Lahmar A, Bouraoui S, et al. Idiopathic granulomatous mastitis mimicking breast cancer: report of two cases. Pathologica Jun;104(3): Berg WA, Cosgrove DO, Doré CJ, Schäfer FKW, Svensson WE, Hooley RJ, et al. Shear-wave elastography improves the specificity of breast US: the BE1 multinational study of 939 masses. Radiology Feb;262(2): Lee EJ, Jung HK, Ko KH, Lee JT, Yoon JH. Diagnostic performances of shear wave elastography: which parameter to use in differential diagnosis of solid breast masses? EurRadiol Jul;23(7): Mazlan L, Suhaimi SNA, Jasmin SJ, Latar NHM, Adzman S, Muhammad R. Breast carcinoma occurring from chronic granulomatous mastitis. Malays J Med Sci MJMS Apr;19(2):82-5. Page 16 of 16