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MRI of Rhabdomyosarcoma and Other Soft-Tissue Sarcomas In This copy is for personal use only. To order printed copies, contact [email protected] 1 IMAGING PEDIATRIC MRI of Rhabdomyosarcoma and Other Soft-Tissue Sarcomas in Children Emilio J. Inarejos Clemente, MD María Navallas, MD Soft-tissue sarcomas in children comprise a heterogeneous group Ignasi Barber Martínez de la Torre, PhD of entities with variable manifestation depending on the age of Mariona Suñol, MD the patient and the location of the tumor. MRI is the modality of Josep Munuera del Cerro, PhD choice for evaluating musculoskeletal soft-tissue tumors and plays Ferran Torner, MD a paramount role in both initial diagnosis and assessment of tumor Moira Garraus, MD response during and after treatment. Conventional MRI sequences, Oscar M. Navarro, MD such as T1- and T2-weighted imaging, offer morphologic informa- tion, which is important for localizing the lesion and describing Abbreviations: ADC = apparent diffusion co- anatomic relationships but not accurate for determining its malig- efficient, DCE = dynamic contrast-enhanced, DWI = diffusion-weighted imaging, MPNST = nant or benign nature and may be limited in differentiating tumor malignant peripheral nerve sheath tumor, NF1 = response from therapy-related changes. Advanced multiparametric neurofibromatosis type 1, TIC = time-intensity MRI offers further functional information that can help with these curve tasks by using different imaging sequences and biomarkers. The RadioGraphics 2020; 40:0000–0000 authors present the role of MRI in rhabdomyosarcoma and other https://doi.org/10.1148/rg.2020190119 soft-tissue sarcomas in children, emphasizing a multiparametric Content Codes: approach with focus on the utility and potential added value of From the Departments of Diagnostic Imaging diffusion-weighted imaging (DWI) and dynamic contrast-enhanced (E.J.I.C., M.N., I.B.M.d.l.T., J.M.d.C.), Pathol- MRI in characterization and staging, determination of pretreat- ogy (M.S.), Orthopaedics (F.T.), and Oncology ment extent, and evaluation of tumor response and recurrence after and Haematology (M.G.), Hospital Sant Joan de Déu, Av Sant Joan de Déu 2, 08950 Esplu- treatment. gues de Llobregat (Barcelona), Spain; Depart- ment of Medical Imaging, University of Toronto, ©RSNA, 2020 • radiographics.rsna.org Toronto, Ont, Canada (O.M.N.); and Depart- ment of Diagnostic Imaging, Hospital for Sick Children, Toronto, Ont, Canada (O.M.N.). Pre- sented as an education exhibit at the 2018 RSNA Annual Meeting. Received April 17, 2019; revi- Introduction sion requested July 24 and received September 4; Soft-tissue sarcomas comprise a diverse group of more than 50 accepted September 13. For this journal-based SA-CME activity, the author J.M.d.C. has pro- different histologic tumors of presumed mesenchymal origin, with vided disclosures (see end of article); all other a wide range of biologic and clinical behavior varying from less ag- authors, the editor, and the reviewers have dis- closed no relevant relationships. Address cor- gressive to highly malignant (1). Soft-tissue sarcomas are infrequent respondence to E.J.I.C. (e-mail: emilioinarejos@ neoplasms, with an annual incidence of around five or six cases per gmail.com). 100 000, and account for about 1% of all malignant tumors, which ©RSNA, 2020 represents 7% of all pediatric malignancies (1,2). Soft-tissue sarcomas have variable clinical and imaging manifesta- SA-CME LEARNING OBJECTIVES tions depending on the age of the patient, the location of the tumor, After completing this journal-based SA-CME and the content of the tumor. Imaging is necessary at the time of initial activity, participants will be able to: diagnosis and as part of evaluation of tumor response after treatment. Describe conventional and multipara- Owing to its contrast resolution, MRI is the modality of choice metric MRI techniques used in children for evaluation and characterization of soft-tissue sarcomas, which are with soft-tissue sarcomas. achieved by combining conventional and advanced multiparametric List the most frequent manifestations MRI (3). Conventional MRI techniques can demonstrate tumor of soft-tissue sarcomas in children at conventional and multiparametric MRI. composition, extent, compartmental involvement, and relationship Discuss use of multiparametric MRI with other structures for accurate treatment planning before surgery. for diagnosis of soft-tissue sarcomas However, conventional techniques remain inadequate for differentia- in children, follow-up during and after tion of malignant from benign lesions owing to overlap in the signal treatment, and evaluation of residual dis- intensity characteristics of these tumors and are also limited in as- ease after surgery. sessment of residual or recurrent disease after treatment (3,4). See rsna.org/learning-center-rg. Traditionally, assessment of tumor response in soft-tissue sarco- mas has been achieved by using three-dimensional measurements (based on the European Pediatric Soft-Tissue Sarcoma Study Group [EpSSG] guidelines) and more recently by using one-dimensional 2 May-June 2020 radiographics.rsna.org of and prognosis for these tumors and posttreat- TEACHING POINTS ment imaging are also discussed. Volumetric determination of tumor ADC (ADC histogram analysis) has become more reproducible and a better reflector of lesion heterogeneity than cross-sectional analysis of a single MRI Protocol region of interest (ROI), which may cause selection bias. The MRI protocol is based on a combination of A type I profile is associated with necrosis or a cystic com- anatomic and multiparametric sequences to ad- ponent, type II with benign processes, types III and IV with equately evaluate soft-tissue sarcomas (4,7). Each malignancy, and type V with granulation tissue or fibrosis. sequence provides complementary information for Soft-tissue sarcomas generally manifest with fast enhance- evaluation of a tumor, including characterization, ment and higher slopes of arterial enhancement than benign extension, and response assessment after treatment. lesions, although this pattern is not entirely specific and has Table 2 shows the protocol used at our institution. to be evaluated together with results from DWI and anatomic sequences. Conventional MRI Whole-lesion ADC histogram analysis of embryonal rhabdo- myosarcoma does not usually demonstrate restriction as a Soft-tissue sarcomas do not have a specific MRI consequence of internal necrosis and myxoid component. pattern, showing variable signal intensity on At MRI, triple signal intensity in a soft-tissue mass has been T1- and T2-weighted images (4). The appear- described as a highly suggestive sign of synovial sarcoma. ance with these sequences varies depending on the presence of hemorrhage, necrosis, or myxoid change (4,8,9). Conventional T1-weighted and fluid-sensitive MRI sequences (fat-suppressed measurements (based on the Response Evaluation T2-weighted or short τ inversion-recovery Criteria in Solid Tumors [RECIST]). However, [STIR]) are important for identifying and delin- both of these methods should be used with cau- eating the extent of a tumor. tion, as their interobserver and intermethod vari- Soft-tissue sarcomas are usually isointense to ability can result in different treatment options (5). muscle on T1-weighted images, with variable de- Multiparametric or functional MRI, which gree of hyperintensity to muscle on fluid-sensitive includes techniques such as diffusion-weighted im- images (10). Newer fluid-sensitive sequences, aging (DWI), chemical shift imaging, and dynamic such as spectral presaturation with inversion contrast-enhanced (DCE) MRI, can allow more recovery (SPIR) and Dixon sequences, produce accurate characterization of malignancy, assess- more homogeneous suppression of fat and pro- ment of treatment response, and evaluation of vide better contrast between fluid and surround- postsurgical residual or recurrent disease (Table 1). ing tissues compared with traditional STIR or Multiparametric MRI does not replace fat-suppressed T2-weighted images (11). conventional MRI. It is important to perform a combined analysis of the information provided Multiparametric MRI by conventional sequences (T1- and T2-weighted imaging) together with functional sequences, Diffusion-weighted Imaging.—DWI provides because each may provide some additional value quantitative functional information on tumor cel- in evaluation of a soft-tissue tumor, including lularity, helping in differentiation between benign characterization, determination of extent, and as- and malignant entities as well as improving MRI sessment of response during and after treatment evaluation of treatment response (3,4,12,13). (4,6). In the future, multiparametric MRI may DWI is considered useful in assessing treatment play a paramount role in assessment of tumor response sooner than conventional imaging (4). response and complement the currently used Mean apparent diffusion coefficient (ADC) val- measurement-based guidelines. ues may help differentiate benign and malignant Histologic findings, which are obtained with lesions, whereas changes in ADC tumor hetero- percutaneous or surgical biopsy, are the reference geneity evaluated longitudinally during treatment standard for final diagnosis and tumor grading. may provide another method of characterizing In this article, we review the epidemiologic tumor viability. A precondition for such evaluation features, clinical features, histopathologic find- is a uniform repeatable method of analyzing suf- ings, and conventional MRI and multiparametric ficient
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