Myxoid Containing Tumors Of Soft Tissues: MRI Appearance With Radiologic-Pathologic Correlation

Transcription

1 Myxoid Containing Tumors Of Soft Tissues: MRI Appearance With Radiologic-Pathologic Correlation Poster No.: C-1416 Congress: ECR 2014 Type: Scientific Exhibit Authors: N. Alberti, X. Buy, J. Palussière, M. Kind; Bordeaux/FR Keywords: Musculoskeletal soft tissue, Oncology, MR, Biopsy, Connective tissue disorders, Pathology DOI: /ecr2014/C-1416 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 31

2 Aims and objectives Soft-tissue tumors are mesenchymal tumors for which the classification established and reviewed in 2013 by the World Health Organization (WHO) is based on identification of the line of differentiation formed by tumor cells. The group of myxoid tumors demonstrate significant variability in their biological behavior and pose the problem of determining whether they are benign, or of intermediate behavior or malignant. The patient discovers a painful or painless enlarged mass, and will often relate it to a trauma. 70% of all mesenchymal tumors are found in the limbs and the trunk wall. In the case of soft-tissue sarcoma, an oncological surgery is recommanded with wide resection into healthy tissue. The local recurrence rate at 5 years is 10% to 15% compared with 50% in case of inadequate surgery. The overall survival rate at 2 years is about 75% for all types of sarcoma. The metastatic risk, based on the grading and the histological type, is about 50%. It is currently accepted that image-guided microbiopsy can be performed instead of open biopsy. However, this practice can pose problems for diagnosis and grading, with regard to tumor tissue sampling with a discrepancy's rate of 8% to 25% between pathologists. The combined results of imaging and histology form the basis of decision-making concerning the management of the patient by a multidisciplinary committee. Magnetic Resonance Imaging can help the diagnosis by identifying fluid-like signal of the myxoid component and narrow the differential diagnosis. The puropose of our study is to identify specific MRI features and to correlate with pathology in a large series of histologically proven myxoid soft tissue tumours. Page 2 of 31

3 Methods and materials Retrospectively selected 63 patients with myxoid STT after approval of Ethical Board. All patients underwent MRI before surgery. Definite histological diagnosis was obtained after surgical resection in all cases. Patients' medical, radiological and pathological data were collected and reviewed by two radiologists. The two radiologists were blinded to the histological diagnoses. Discrepancies in interpretation were decided by consensus. Routine MRI protocols included: 1,5T units (Intera Philips Medical System, Eindhoven Holland and Aera Siemens Medical Systems, Erlangen, Germany). T1-weighted sequence (TR/TE = /11-16msec), and T2-weighted sequence (TR/ TE = 1, 800-2,500/54-110msec) in at least 2 orthogonal planes. Section thickness ranged from 3 mm to 5 mm Field of view and use of body or surface coils varied according to the size and location. Gadolinium-complex contrast enhancement was used in all patients; Dotarem (Gadoteric acid, Guerbet, Aulnay s/ Bois, France) and Multihance (Gadobenatedimeglubine, Bracco, Milan, Italy). Images for this section: Page 3 of 31

4 Fig. 1: Imaging analysis Page 4 of 31

5 Fig. 2: Capsule Page 5 of 31

6 Fig. 3: Fat rim Page 6 of 31

7 Fig. 4: Focal oedema Page 7 of 31

8 Fig. 5: Tail sign Page 8 of 31

9 Fig. 6: Myxoid component Page 9 of 31

10 Fig. 7: Fibrous component Page 10 of 31

11 Fig. 8: Necrotic component Page 11 of 31

12 Results 63 patients : 40 male/ 23 female Mean age : 59 years Locations : *inferior limb : 54 * superior limb : 4 * Trunk : 5 A) Myxoma Intramuscular 100% Well defined without capsule 100% fat rim (92%) and peripheral edema 33% Mean length: 89 mm Septas or fine strands: 50% Fluid-like hypert2 (myxoïd component >60%) 100% No intra tumor fat nor necrosis Mild post contrast enhancement B) Myxoid liposarcoma (MLPS) Intermuscular 80% Well defined (95%), with capsule 40% Mean length 128 mm Intra tumoral hypert1 signal (fat): 80% Page 12 of 31

13 * 13/16 : diffuse * 3/16 : focal Fluid-like HyperT2 (myxoide> 60%): 80% Strong enhancement intensity: 75% Tumor heterogeneity+++= round cells liposarcoma C) Low grade fibromyxoid sarcoma (LGFMS) Intramuscular 100% Well defined with capsule 100% length : 68 mm HyperT2 fluid-like signal ( myxoid component < 50%) in 6 cases HypoT2 fibrous-like signal area ++++ No fatty component nor necrosis D) Myxofibrosarcoma (MFS) Ill-defined : 73% Aponevrosis enhancement: Tail sign 70% Superficial and deep location: 50% Mean length: 72 mm hypert2 fluid-like area (myxoid component >50% ): 96% hypot2 fibrous-like area : 100% Necrosis: 80% Complex T2 appearance "triple sign": 80% Page 13 of 31

14 Images for this section: Fig. 20: Results Page 14 of 31

15 Fig. 9: Myxoma Page 15 of 31

16 Fig. 10: Myxoma Page 16 of 31

17 Fig. 11: MLPS Page 17 of 31

18 Fig. 12: MLPS Page 18 of 31

19 Fig. 14: MLPS Page 19 of 31

20 Fig. 13: Round cells liposarcoma Page 20 of 31

21 Fig. 15: LGFMS Page 21 of 31

22 Fig. 16: LGFMS Page 22 of 31

23 Fig. 17: MFS Page 23 of 31

24 Fig. 18: MFS Page 24 of 31

25 Fig. 19: MFS Page 25 of 31

26 Conclusion Myxoid containing tumors of soft tissues are a heterogeneous group of mesenchymal tumors the diagnosis and management of which can be complex. Thus, MR results give the basis of decision-making by a multidisciplinary committee. The treatment will be radically different according to whether a benign lesion is involved which can be removed simply, or whether it is a sarcoma requiring initial surgery in a specialized environment. The combined results of imaging and histology form the basis of decision-making concerning the management of the patient by a multidisciplinary committee. Images for this section: Fig. 22: Take home messages Page 26 of 31

27 Fig. 21: Take home messages Page 27 of 31

28 Fig. 23: Decision diagram Page 28 of 31

29 Personal information Nicolas Alberti Xavier Buy 1,2 1 1 Jean Palussiere Michele Kind 1 1:Department of Interventional Radiology, Institut Bergonié, Comprehensive Cancer Centre, 229 cours de l'argonne, F Bordeaux, France 2:Department of radiology, HIA Robert Picqué, 351 Route de Toulouse, Villenave d'ornon, France References Coindre J-M. [New WHO classification of tumours of soft tissue and bone]. Ann Pathol Nov;32(5 Suppl):S Fletcher C, Unni K, Mertens F, eds. World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone. IARC Press. Lyon, France; 2002; Kind M, Stock N, Coindre JM. Histology and imaging of soft tissue sarcomas. Eur J Radiol Oct;72(1):6-15. Graadt van Roggen JF, Hogendoorn PC, Fletcher CD. Myxoid tumours of soft tissue. Histopathology Oct;35(4): Allen PW. Myxoid tumors of soft tissues. Pathol Annu. 1980;15(Pt 1): Mackenzie DH. The myxoid tumors of somatic soft tissues. Am. J. Surg. Pathol Jul;5(5): Page 29 of 31

30 Wakely PE Jr. Myxomatous soft tissue tumors: correlation of cytopathology and histopathology. Ann Diagn Pathol Aug;3(4): Peterson KK, Renfrew DL, Feddersen RM, Buckwalter JA, el-khoury GY. Magnetic resonance imaging of myxoid containing tumors. Skeletal Radiol. 1991;20(4): Graadt van Roggen JF, Hogendoorn PC, Fletcher CD. Myxoid tumours of soft tissue. Histopathology Oct;35(4): Cabral CE, Guedes P, Fonseca T, Rezende JF, Cruz Júnior LC, Smith J. Polyostotic fibrous dysplasia associated with intramuscular myxomas: Mazabraud's syndrome. Skeletal Radiol May;27(5): Zoccali C, Teori G, Prencipe U, Erba F. Mazabraud's syndrome: a new case and review of the literature. Int Orthop Jun;33(3): Allen PW. Myxoma is not a single entity: A review of the concept of myxoma. Annals of Diagnostic Pathology avril;4(2): Kindblom LG, Stener B, Angervall L. Intramuscular myxoma. Cancer Nov;34(5): Murphey MD, McRae GA, Fanburg-Smith JC, Temple HT, Levine AM, Aboulafia AJ. Imaging of Soft-Tissue Myxoma with Emphasis on CT and MR and Comparison of Radiologic and Pathologic Findings1. Radiology Oct 1;225(1): Nielsen GP, O'Connell JX, Rosenberg AE. Intramuscular myxoma: a clinicopathologic study of 51 cases with emphasis on hypercellular and hypervascular variants. Am. J. Surg. Pathol Oct;22(10): Hashimoto H, Tsuneyoshi M, Daimaru Y, Enjoji M, Shinohara N. Intramuscular myxoma. A clinicopathologic, immunohistochemical, and electron microscopic study. Cancer Aug 1;58(3): Kransdorf MJ, Murphey MD, Smith SE. Imaging of Soft Tissue Neoplasms in the Adult: Benign Tumors. Semin Musculoskelet Radiol. 1999;3(1): Page 30 of 31

31 Luna A, Martinez S, Bossen E. Magnetic resonance imaging of intramuscular myxoma with histological comparison and a review of the literature. Skeletal Radiol Jan;34(1): Bermejo A, De Bustamante TD, Martinez A, Carrera R, Zabía E, Manjón P. MR Imaging in the Evaluation of Cystic-appearing Soft-Tissue Masses of the Extremities. Radiographics May;33(3): Girish G, Jamadar DA, Landry D, Finlay K, Jacobson JA, Friedman L. Sonography of intramuscular myxomas: the bright rim and bright cap signs. J Ultrasound Med Jul;25(7): ; quiz Hoffman A, Ghadimi MPH, Demicco EG, Creighton CJ, Torres K, Colombo C, et al. Localized and metastatic myxoid/round cell liposarcoma: Clinical and molecular observations. Cancer Feb 7; Moreau L-C, Turcotte R, Ferguson P, Wunder J, Clarkson P, Masri B, et al. Myxoid\round cell liposarcoma (MRCLS) revisited: an analysis of 418 primarily managed cases. Ann. Surg. Oncol Apr;19(4): Page 31 of 31