Subscribe to RSS
DOI: 10.1055/s-0038-1676125
Ewing's Sarcoma and Primary Osseous Lymphoma: Spectrum of Imaging Appearances
Publication History
Publication Date:
30 January 2019 (online)
Abstract
Ewing's sarcoma (ES) is a rare, highly malignant anaplastic stem cell tumor. Histologically, the tumor consists of uniform densely packed small monomorphic cells with round nuclei. The typical appearance at hematoxylin and eosin (H&E) staining is small blue round cells without any matrix formation. On conventional radiography, ES typically presents as a permeative lesion in the diaphysis of a long bone in a child. A large soft tissue component is another characteristic feature, best depicted by magnetic resonance imaging.
Primary osseous lymphomas are most commonly highly malignant B-cell lymphomas. At H&E histologic staining, the tumor stroma consists of diffuse round-cell infiltrates that resembles the appearance of ES. Although there is no typical imaging appearance of an osseous lymphoma, it should be considered in an adult presenting with a Lodwick grade II or III lesion in the metaphysis or diaphysis of a large long bone, the pelvis, or the vertebral column. Histologic confirmation is mandatory.
-
References
- 1 Freyschmidt J, Ostertag H. Ewing's sarcoma, fibrogenic tumors, giant cell tumor, hemangioma of bone : radiology and pathology [in German]. Radiologe 2016; 56 (06) 520-535
- 2 Peersman B, Vanhoenacker FM, Heyman S. , et al. Ewing's sarcoma: imaging features. JBR-BTR 2007; 90 (05) 368-376
- 3 Jordanov MI, Block JJ, Gonzalez AL, Green NE. Transarticular spread of Ewing sarcoma mimicking septic arthritis. Pediatr Radiol 2009; 39 (04) 381-384
- 4 Ilaslan H, Sundaram M, Unni KK, Dekutoski MB. Primary Ewing's sarcoma of the vertebral column. Skeletal Radiol 2004; 33 (09) 506-513
- 5 Rodallec MH, Feydy A, Larousserie F. , et al. Diagnostic imaging of solitary tumors of the spine: what to do and say. Radiographics 2008; 28 (04) 1019-1041
- 6 Delattre O, Zucman J, Melot T. , et al. The Ewing family of tumors—a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med 1994; 331 (05) 294-299
- 7 Lessnick SL, Ladanyi M. Molecular pathogenesis of Ewing sarcoma: new therapeutic and transcriptional targets. Annu Rev Pathol 2012; 7: 145-159
- 8 Murphey MD, Senchak LT, Mambalam PK, Logie CI, Klassen-Fischer MK, Kransdorf MJ. From the radiologic pathology archives: Ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics 2013; 33 (03) 803-831
- 9 Applebaum MA, Goldsby R, Neuhaus J, DuBois SG. Clinical features and outcomes in patients with Ewing sarcoma and regional lymph node involvement. Pediatr Blood Cancer 2012; 59 (04) 617-620
- 10 Study in localized and disseminated Ewing sarcoma. https://clinicaltrials.gov/ct2/show/NCT00987636 . Accessed September 2, 2018
- 11 Lalam R, Bloem JL, Noebauer-Huhmann IM. , et al. ESSR consensus document for detection, characterization, and referral pathway for tumors and tumorlike lesions of bone. Semin Musculoskelet Radiol 2017; 21 (05) 630-647
- 12 McCarville MB, Chen JY, Coleman JL. , et al. Distinguishing osteomyelitis from Ewing sarcoma on radiography and MRI. AJR Am J Roentgenol 2015; 205 (03) 640-650 ; quiz 651
- 13 Kloth JK, Wolf M, Rehnitz C, Lehner B, Wiedenhöfer B, Weber MA. Radiological diagnostics of spinal tumors. Part 1: general tumor diagnostics and special diagnostics of extradural tumors [in German]. Orthopade 2012; 41 (08) 595-607
- 14 Haeusler J, Ranft A, Boelling T. , et al. The value of local treatment in patients with primary, disseminated, multifocal Ewing sarcoma (PDMES). Cancer 2010; 116 (02) 443-450
- 15 Potratz J, Dirksen U, Jürgens H, Craft A. Ewing sarcoma: clinical state-of-the-art. Pediatr Hematol Oncol 2012; 29 (01) 1-11
- 16 Ladenstein R, Pötschger U, Le Deley MC. , et al. Primary disseminated multifocal Ewing sarcoma: results of the Euro-EWING 99 trial. J Clin Oncol 2010; 28 (20) 3284-3291
- 17 AWMF guideline to Ewing's sarcoma (in children and adolescents) of the German Society of Pediatric and Adolescent Medicine (DGKJ). In: AWMF online (state 2014, valid until June 1, 2019). https://www.awmf.org/uploads/tx_szleitlinien/025-006l_S1_Ewing_Sarkome_Kinder_Jugendliche_2014-06.pdf . Accessed August 12, 2018
- 18 Vilanova JC, Baleato-Gonzalez S, Romero MJ, Carrascoso-Arranz J, Luna A. Assessment of musculoskeletal malignancies with functional MR imaging. Magn Reson Imaging Clin N Am 2016; 24 (01) 239-259
- 19 Paulussen M, Bielack S, Jürgens H, Casali PG. ; ESMO Guidelines Working Group. Ewing's sarcoma of the bone: ESMO clinical recommendations for diagnosis, treatment and follow-up. Ann Oncol 2009; 20 (Suppl. 04) 140-142
- 20 Balamuth NJ, Womer RB. Ewing's sarcoma. Lancet Oncol 2010; 11 (02) 184-192
- 21 Moore DD, Haydon RC. Ewing's sarcoma of bone. Cancer Treat Res 2014; 162: 93-115
- 22 Binesh F, Mirjalili MR, Akhavan A, Navabii H. Primary bony Hodgkin's lymphoma. BMJ Case Rep 2012 2012.
- 23 Messina C, Christie D, Zucca E, Gospodarowicz M, Ferreri AJ. Primary and secondary bone lymphomas. Cancer Treat Rev 2015; 41 (03) 235-246
- 24 Ludwig K. Musculoskeletal lymphomas [in German]. Radiologe 2002; 42 (12) 988-992
- 25 Demircay E, Hornicek Jr FJ, Mankin HJ, Degroot III H. Malignant lymphoma of bone: a review of 119 patients. Clin Orthop Relat Res 2013; 471 (08) 2684-2690
- 26 Jain A, Alam K, Maheshwari V, Khan R, Nobin H, Narula V. Primary bone lymphomas—clinical cases and review of literature. J Bone Oncol 2013; 2 (03) 132-136
- 27 Chisholm KM, Ohgami RS, Tan B, Hasserjian RP, Weinberg OK. Primary lymphoma of bone in the pediatric and young adult population. Hum Pathol 2017; 60: 1-10
- 28 Fox MG, Marti JK, Bachmann KR, LeGallo RD, Foster WC. Epiphyseal presentation of non-Hodgkin's lymphoma of bone in two pediatric patients—one with primary lymphoma of bone. Skeletal Radiol 2015; 44 (04) 587-595
- 29 Wu H, Zhang L, Shao H. , et al. Prognostic significance of soft tissue extension, international prognostic index, and multifocality in primary bone lymphoma: a single institutional experience. Br J Haematol 2014; 166 (01) 60-68
- 30 Navarro SM, Matcuk GR, Patel DB. , et al. Musculoskeletal imaging findings of hematologic malignancies. Radiographics 2017; 37 (03) 881-900
- 31 Krishnan A, Shirkhoda A, Tehranzadeh J, Armin AR, Irwin R, Les K. Primary bone lymphoma: radiographic-MR imaging correlation. Radiographics 2003; 23 (06) 1371-1383 ; discussion 1384–1387
- 32 Ruzek KA, Wenger DE. The multiple faces of lymphoma of the musculoskeletal system. Skeletal Radiol 2004; 33 (01) 1-8
- 33 Graham TS. The ivory vertebra sign. Radiology 2005; 235 (02) 614-615
- 34 Braun RA, Milito CF, Goldman SM, Fernandes EdeÁ. Ivory vertebra: imaging findings in different diagnoses. Radiol Bras 2016; 49 (02) 117-121
- 35 Lim CY, Ong KO. Imaging of musculoskeletal lymphoma. Cancer Imaging 2013; 13 (04) 448-457
- 36 Murphey MD, Kransdorf MJ. Primary musculoskeletal lymphoma. Radiol Clin North Am 2016; 54 (04) 785-795
- 37 Albano D, Patti C, Lagalla R, Midiri M, Galia M. Whole-body MRI, FDG-PET/CT, and bone marrow biopsy, for the assessment of bone marrow involvement in patients with newly diagnosed lymphoma. J Magn Reson Imaging 2017; 45 (04) 1082-1089
- 38 Balbo-Mussetto A, Saviolo C, Fornari A. , et al. Whole body MRI with qualitative and quantitative analysis of DWI for assessment of bone marrow involvement in lymphoma. Radiol Med (Torino) 2017; 122 (08) 623-632
- 39 Lodwick GS, Wilson AJ, Farrell C, Virtama P, Dittrich F. Determining growth rates of focal lesions of bone from radiographs. Radiology 1980; 134 (03) 577-583
- 40 Lodwick GS, Wilson AJ, Farrell C, Virtama P, Smeltzer FM, Dittrich F. Estimating rate of growth in bone lesions: observer performance and error. Radiology 1980; 134 (03) 585-590
- 41 Siddiqui YS, Khan AQ, Sherwani M. Pathological fractures in primary non-Hodgkin's lymphoma of the bone: a case series with review of the literature. J Clin Diagn Res 2013; 7 (03) 513-517
- 42 Wu H, Bui MM, Leston DG. , et al. Clinical characteristics and prognostic factors of bone lymphomas: focus on the clinical significance of multifocal bone involvement by primary bone large B-cell lymphomas. BMC Cancer 2014; 14: 900
- 43 Dürr HR, Müller PE, Hiller E. , et al. Malignant lymphoma of bone. Arch Orthop Trauma Surg 2002; 122 (01) 10-16