Imaging spectrum of angiosarcoma of breast

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1 Imaging spectrum of angiosarcoma of breast Poster No.: C-1097 Congress: ECR 2015 Type: Educational Exhibit Authors: T. Sehgal, S. K. Ramani, N. Nair, A. Patil, M. H. Thakur; Mumbai/ IN Keywords: Neoplasia, Education and training, Diagnostic procedure, Ultrasound, PET-CT, MR, Breast DOI: /ecr2015/C-1097 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 14

2 Learning objectives Angiosarcoma is a rare form of breast cancer with a far worse prognosis than breast carcinoma & is missed and underreported due to lack of familiarity with imaging features which raise a suspicion for angiosarcoma.the aim of this exhibit is to represent the imaging findings(mammography,breast Ultrasonography,color Doppler, MRI,& PET scan)of both primary and secondary angiosarcoma including the lymphedema associated cutaneous angiosarcoma(stewart-treves syndrome) of the breast, and metastases from breast angiosarcoma.not all hyperechoic masses on breast sonography are benign! Images for this section: Fig. 1: Primary Angiosarcoma Case 1 Page 2 of 14

3 Fig. 2: Histopathology Case 1. Fig. 3: Contralateral Breast recurrence.case1. Page 3 of 14

4 Fig. 4: Primary Angiosarcoma:Case 2. Page 4 of 14

5 Fig. 5: Contralateral Breast Recurrence.Case 2. Page 5 of 14

6 Fig. 6: Orbital Metastasis in Bilateral Breast Angiosarcoma. Page 6 of 14

7 Fig. 7: Secondary Angiosarcoma: Case 1. Page 7 of 14

8 Fig. 8: Secondary Angiosarcoma- Increasing skin thickening. Case 1. Page 8 of 14

9 Fig. 9: Secondary Angiosarcoma:Case 2. Page 9 of 14

10 Fig. 10: Chronic Lymphedema -Stewart Treves syndrome. Page 10 of 14

11 Background Angiosarcoma is a rare form of breast cancer with a far worse prognosis than breast carcinoma,which is missed on Imaging,is underreported due to lack of familiarity with imaging features which raise a suspicion for both primary and secondary angiosarcomas. Angiosarcomas are the most common sarcoma of the breast but are still relatively rare. The neoplasm is of vascular origin. The frequency of this rare tumour is 0.04% of primary breast tumours and approximately 8% of breast sarcomas. The estimated incidence of secondary (postirradiation)soft tissue sarcomas of the breast ranges from 0.01% to 0.02% per year. Both primary and secondary breast angiosarcomas carry a prognosis worse than breast carcinoma. They differ, however, in the age of presentation and clinical presentation.primary angiosarcomas of the breast occur sporadically in young women (20-50 years)and usually present as palpable masses. Secondary angiosarcomas occur most frequently after breast conservation therapy with radiation therapy. Mammography appearances are non-specific, most common finding is that of an ill defined noncalcified mass or focal asymmetry.there is absence of spiculations which are generally seen in malignant breast masses. In the case of primary angiosarcoma, the patients are usually young, leading to obscuration of the mass lesion by normal dense parenchyma of breast. Mammograms may appear completely normal in 33% of cases of primary angiosarcoma. Findings and procedure details In this exhibit we present primary breast angiosarcoma, secondary angiosarcomas, as a complication of breast radiation treatments,stewart-treves syndrome seen in chronically lymphedematous arms after axillary treatment, and primary breast angiosarcoma with metastasis. In primary angiosarcomas focal asymmetry was the most common finding on Mammography 3 of 4 cases (fig1,3,6), while one showed no abnormality on mammography(fig4). Sonography showed heterogeneously hyperechoic masses in all primary angiosarcomas(fig 1,3-6) with no vascularity on color Doppler(fig 1,3-5). MRI showed heterogeneously enhancing masses(fig1),non mass enhancement(fig 4),and heterogeneously enhancing mass(fig 5 ). PET scan showed FDG uptake in index breast(fig 4) with contralateral breast recurrence showing no uptake(fig 5). Uptake was noted in contralateral recurrence in one case(fig3). Secondary angiosarcomas showed increasing skin thickening(fig7-9),exophytic mass(fig7), increased breast density and Page 11 of 14

12 focal asymmetry (fig 9). Sonography showed hypoechoic mass(fig 7), mixed echogenic mainly hypoechoic mass(fig 9). Both showed vascularity on color Doppler(fig 7,9).MRI showed enhancing skin thickening with heterogeneously enhancing mass in the retroareolar region showing type II curve (fig 9). Stewart Treves syndrome showed swelling and ulcerations of the involved upper limb(fig 10). Orbital metastasis were noted in bilateral breast angiosarcoma case- uncommon(fig6). Images for this section: Fig. 3: Contralateral Breast recurrence.case1. Page 12 of 14

13 Conclusion The major teaching point of this exhibit are: "Not all hyperechoic lesions are benign"especially in Primary angiosarcoma. Importance of considering primary angiosarcoma on breast sonography finding of heterogenously hyperechoic mass.they do not necessarily show vascularity on color doppler, which varies according to grade of the lesion. Mammography may be normal in primary angiosarcomas and show focal asymmetry in primary angiosarcomas. Serial images showing increase in skin thickening should raise a suspicion for secondary angiosarcoma.vascularity is seen on color doppler in secondary angiosarcomas. Primary angiosarcomas show variable appearances on MRI from masses to non mass enhancement. MRI plays an important role to identify the extent of the lesion & plan surgery. Primary angiosarcomas may show uptake on PET scan as also secondary angiosarcmas. Angiosarcoma of the breast is a rare malignancy with a poor prognosis, even after complete resection, early recognition of angiosarcoma is thus necessary for prompt initiation of appropriate therapy. With increase in the number of breast conservation surgeries & increased survival of patients, development of secondary angiosarcoma as a delayed complication of radiation & long standing edema should also considered on follow-up analysis Personal information References Liberman L, Dershaw DD, Kaufman RJ, Rosen PP. Angiosarcoma of the breast. Radiology 1992;183: Taletha B, Smith, Michael Z. Gilcrease, Lumarie Santiago, Kelly K. Hunt, Wei T. Yang. Imaging Features of Primary Breast Sarcoma. AJR 2012; 198:W386-W393. Page 13 of 14

14 Anna Linda,Chiara Zuiani, Michele Lorenzon, Alessandro Furlan, Rossano Girometti, Viviana Londero, Massimo Bazzocchi. Hyperechoic Lesions of the Breast: Not Always Benign. AJR 2011; 196: Brenner RJ, Pfaff JM. Mammographic features after conservationtherapy for malignant breast disease: serial findings standardizedby regression analysis. Am J Roentgenol 1996;167: Dershaw DW. Mammography in patients with breast cancer treated by breast conservation (lumpectomy with or without radiation).am J Roentgenol 1995;164: Katrina N. Glazebrook1 Maureen J. Magut1 Carol Reynolds. Angiosarcoma of the Breast, AJR 2008; 190: Robert F. Lim,Reginald Goei. Best Cases from the AFIP. Angiosarcoma of the Breast.RadioGraphics,2007;27:S125-S130. Marchal C, Weber B, de Lafontan B, et al. Nine breast angiosarcomas after conservative treatment for breast carcinoma:a survey from French comprehensive Cancer Centers. Int J Radiat Oncol Biol Phys. 1999;44: Page 14 of 14

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