There are numerous bone tumors in the pediatric population, with imaging playing an essential role in diagnosis and management. Our understanding of certain bone tumors has rapidly evolved over the past decade with advancements in next-generation genetic sequencing techniques. This increased level of understanding has altered the nomenclature, management approach, and prognosis of certain lesions. We provide a detailed update of bone tumors that occur in the pediatric population with emphasis on the recently released nomenclature provided in the 5th edition of the World Health Organization Classification of Soft Tissue and Bone Tumours. We discuss other mesenchymal tumors of bone, hematopoietic neoplasms of bone, and WHO classification of undifferentiated small round cell sarcomas of bone. We have detailed osteogenic tumors and osteoclastic giant cell-rich tumors, as well as notochordal tumors, chondrogenic tumors, and vascular tumors of the bone in separate manuscripts.
This is a preview of subscription content, access via your institution.
Buy single article
Instant access to the full article PDF.
Price excludes VAT (USA)
Tax calculation will be finalised during checkout.
WHO Classification: of Tumours Editorial Board. WHO Classification of Tumours Editorial Board: Soft Tissue and Bone Tumours. 5th ed. Lyon (France): International Agency for Research on Cancer; 2020.
Choi JH, Ro JY. The 2020 WHO Classification of Tumors of Bone: an updated review. Adv Anat Pathol. 2021;28:119–38.
Anderson WJ, Doyle LA. Updates from the 2020 World Health Organization Classification of Soft Tissue and Bone Tumours. Histopathology. 2021;78:644–57.
Al-Dasuqi K, Cheng R, Moran J, Irshaid L, Maloney E, Porrino J. Update of pediatric bone tumors: osteogenic tumors and osteoclastic giant cell-rich tumors. Skeletal Radiol. https://doi.org/10.1007/s00256-022-04221-3. Accessed 3 Nov 2022.
Lee H, Wang A, Cheng R, Moran J, Al-Dasuqi K, Irshaid L, Maloney E, Porrino J. Update of pediatric bone tumors-notochordal tumors, chondrogenic tumors, and vascular tumors of the bone. Skeletal Radiol. https://doi.org/10.1007/s00256-022-04235-x. Accessed 11 Nov 2022.
Gleason BC, Liegl-Atzwanger B, Kozakewich HP, Connolly S, Gebhardt MC, Fletcher JA, et al. Osteofibrous dysplasia and adamantinoma in children and adolescents: a clinicopathologic reappraisal. Am J Surg Pathol. 2008;32:363–76.
Hazelbag HM, Wessels JW, Mollevangers P, van den Berg E, Molenaar WM, Hogendoorn PC. Cytogenetic analysis of adamantinoma of long bones: further indications for a common histogenesis with osteofibrous dysplasia. Cancer Genet Cytogenet. 1997;97:5–11.
Bone Tumor Pathology, An Issue of Surgical Pathology Clinics, 1st Edition: Edited by Gunnlaugur Petur Nielsen, MD. Elsevier; 240. Accessed 9 Nov 2021.
Park JW, Lee C, Han I, Cho H-S, Kim H-S. Optimal Treatment of Osteofibrous Dysplasia of the Tibia. J Pediatr Orthop. 2018;38:e404–10.
Westacott D, Kannu P, Stimec J, Hopyan S, Howard A. Osteofibrous dysplasia of the tibia in children: outcome without resection. J Pediatr Orthop. 2019;39:e614–21.
Scholfield DW, Sadozai Z, Ghali C, Sumathi V, Douis H, Gaston L, et al. Does osteofibrous dysplasia progress to adamantinoma and how should they be treated? Bone Joint J. 2017;99-B:409–16.
Kitsoulis P, Charchanti A, Paraskevas G, Marini A, Karatzias G. Adamantinoma. Acta Orthop Belg. 2007;73:425–31.
Varvarousis DN, Skandalakis GP, Barbouti A, Papathanakos G, Filis P, Tepelenis K, et al. Adamantinoma: an updated review. In Vivo. 2021;35:3045–52.
Hazelbag HM, Laforga JB, Roels HJL, Hogendoorn PCW. Dedifferentiated adamantinoma with revertant mesenchymal phenotype. Am J Surg Pathol. 2003;27:1530–7.
Kashima TG, Dongre A, Flanagan AM, Hogendoorn PCW, Taylor R, Athanasou NA. Podoplanin expression in adamantinoma of long bones and osteofibrous dysplasia. Virchows Arch. 2011;459:41–6.
Dickson BC, Gortzak Y, Bell RS, Ferguson PC, Howarth DJC, Wunder JS, et al. p63 expression in adamantinoma. Virchows Arch. 2011;459:109–13.
Schutgens EM, Picci P, Baumhoer D, Pollock R, Bovée JVMG, Hogendoorn PCW, et al. Surgical Outcome and oncological survival of osteofibrous dysplasia-like and classic adamantinomas: an international multicenter study of 318 cases. J Bone Joint Surg Am. 2020;102:1703–13.
Khanna M, Delaney D, Tirabosco R, Saifuddin A. Osteofibrous dysplasia, osteofibrous dysplasia-like adamantinoma and adamantinoma: correlation of radiological imaging features with surgical histology and assessment of the use of radiology in contributing to needle biopsy diagnosis. Skeletal Radiol. 2008;37:1077–84.
Unni, KK. Dahlin’s Bone Tumors: General Aspects and Data on 11,087 Cases, ed 5. Philadelphia: Lippincott-Raven; 1996. pp. 463.
Van der Woude H-J, Hazelbag H-M, Bloem JL, Taminiau AHM, Hogendoorn PCW. MRI of adamantinoma of long bones in correlation with histopathology. AJR Am J Roentgenol. 2004;183:1737–44.
Aytekin MN, Öztürk R, Amer K. Epidemiological study of adamantinoma from US surveillance, epidemiology, and end results program: III retrospective analysis. J Oncol. 2020;2020:2809647.
Deng Z, Gong L, Zhang Q, Hao L, Ding Y, Niu X. Outcome of osteofibrous dysplasia-like versus classic adamantinoma of long bones: a single-institution experience. J Orthop Surg Res. 2020;15:268.
Pižem J, Šekoranja D, Zupan A, Boštjančič E, Matjašič A, Mavčič B, et al. FUS-NFATC2 or EWSR1-NFATC2 fusions are present in a large proportion of simple bone cysts. Am J Surg Pathol. 2020;44:1623–34.
Haidar SG, Culliford DJ, Gent ED, Clarke NMP. Distance from the growth plate and Its relation to the outcome of unicameral bone cyst treatment. J Child Orthop. 2011;5:151–6.
Mascard E, Gomez-Brouchet A, Lambot K. Bone cysts: unicameral and aneurysmal bone cyst. Orthop Traumatol Surg Res. 2015;101:S119–127.
Kushchayeva YS, Kushchayev SV, Glushko TY, Tella SH, Teytelboym OM, Collins MT, et al. Fibrous dysplasia for radiologists: beyond ground glass bone matrix. Insights Imaging. 2018;9:1035–56.
Harris WH, Dudley HR, Barry RJ. The natural history of fibrous dysplasia. An orthopaedic, pathological, and roentgenographic study. J Bone Joint Surg Am. 1962;44-A:207–33.
Tafti D, Cecava N. Fibrous Dysplasia [Internet]. StatPearls Publishing. Available from: https://www.ncbi.nlm.nih.gov/books/NBK532947/. Accessed 3 Oct 2022.
Kuznetsov SA, Cherman N, Riminucci M, Collins MT, Robey PG, Bianco P. Age-dependent demise of GNAS-mutated skeletal stem cells and “normalization” of fibrous dysplasia of bone. J Bone Miner Res. 2008;23:1731–40.
Burke AB, Collins MT, Boyce AM. Fibrous dysplasia of bone: craniofacial and dental implications. Oral Dis. 2017;23:697–708.
Javaid MK, Boyce A, Appelman-Dijkstra N, Ong J, Defabianis P, Offiah A, et al. Best practice management guidelines for fibrous dysplasia/McCune-Albright syndrome: a consensus statement from the FD/MAS international consortium. Orphanet J Rare Dis. 2019;14:139.
Jee WH, Choe BY, Kang HS, Suh KJ, Suh JS, Ryu KN, et al. Nonossifying fibroma: characteristics at MR imaging with pathologic correlation. Radiology. 1998;209:197–202.
Samet J, Weinstein J, Fayad LM. MRI and clinical features of Langerhans cell histiocytosis (LCH) in the pelvis and extremities: can LCH really look like anything? Skeletal Radiol. 2016;45:607–13.
Singh J, Rajakulasingam R, Saifuddin A. Langerhans cell histiocytosis of the shoulder girdle, pelvis and extremities: a review of radiographic and MRI features in 85 cases. Skeletal Radiol. 2020;49:1925–37.
Nagy A, Somers GR. Round cell sarcomas: newcomers and diagnostic approaches. Surg Pathol Clin. 2020;13:763–82.
Davis JL, Rudzinski ER. Small round blue cell sarcoma other than ewing sarcoma: what should an oncologist know? Curr Treat Options Oncol. 2020;21:90.
Sbaraglia M, Righi A, Gambarotti M, Dei Tos AP. Ewing sarcoma and Ewing-like tumors. Virchows Arch. 2020;476:109–19.
Tsuda Y, Zhang L, Meyers P, Tap WD, Healey JH, Antonescu CR. The clinical heterogeneity of round cell sarcomas with EWSR1/FUS gene fusions: impact of gene fusion type on clinical features and outcome. Genes Chromosomes Cancer. 2020;59:525–34.
Riggi N, Suvà ML, Stamenkovic I. Ewing’s sarcoma. N Engl J Med. 2021;384:154–64.
Kimbara S, Imamura Y, Kiyota N, Takakura H, Matsumoto S, Koyama T, et al. Secondary CIC-rearranged sarcoma responsive to chemotherapy regimens for Ewing sarcoma: a case report. Mol Clin Oncol. 2021;14:68.
Brady EJ, Hameed M, Tap WD, Hwang S. Imaging features and clinical course of undifferentiated round cell sarcomas with CIC-DUX4 and BCOR-CCNB3 translocations. Skeletal Radiol. 2021;50:521–9.
Sirisena UDN, Rajakulasingam R, Saifuddin A. Imaging of bone and soft tissue BCOR-rearranged sarcoma. Skeletal Radiol. 2021;50:1291–301.
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;33:803–31.
Schaefer I-M, Hornick JL. Diagnostic immunohistochemistry for soft tissue and bone tumors: an update. Adv Anat Pathol. 2018;25:400–12.
Zöllner SK, Amatruda JF, Bauer S, Collaud S, de Álava E, DuBois SG, et al. Ewing Sarcoma-diagnosis, treatment, clinical challenges and future perspectives. J Clin Med. 2021;10:1685.
Diaz-Perez JA, Nielsen GP, Antonescu C, Taylor MS, Lozano-Calderon SA, Rosenberg AE. EWSR1/FUS-NFATc2 rearranged round cell sarcoma: clinicopathological series of 4 cases and literature review. Hum Pathol. 2019;90:45–53.
Antonescu CR, Kao Y-C, Xu B, Fujisawa Y, Chung C, Fletcher CDM, et al. Undifferentiated round cell sarcoma with BCOR internal tandem duplications (ITD) or YWHAE fusions: a clinicopathologic and molecular study. Mod Pathol. 2020;33:1669–77.
Kyriazoglou A, Tourkantoni N, Liontos M, Zagouri F, Mahaira L, Papakosta A, et al. A case series of BCOR sarcomas with a new splice variant of BCOR/CCNB3 fusion gene. In Vivo. 2020;34:2947–54.
Consent for publication
All authors have agreed to approval of the most current version to be published and agree to be accountable for all aspects of the work if questions were to arise related to its accuracy or integrity.
Conflict of interest
The authors declare no competing interests.
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
About this article
Cite this article
Wang, A., Maloney, E., Al-Dasuqi, K. et al. Update of pediatric bone tumors—other mesenchymal tumors of bone, hematopoietic neoplasms of bone, and WHO classification of undifferentiated small round cell sarcomas of bone. Skeletal Radiol 52, 1443–1463 (2023). https://doi.org/10.1007/s00256-023-04286-8