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Benign bone tumors in children and adolescents: An overview

Author
John E Tis, MD
Section Editor
William Phillips, MD
Deputy Editor
Mary M Torchia, MD

INTRODUCTION

Benign childhood bone tumors range from static lesions, such as nonossifying fibromas, which remain essentially unchanged throughout childhood, to locally aggressive lesions, such as aneurysmal bone cysts, which continue to expand until treated. (See 'Nonossifying fibroma' below and 'Aneurysmal bone cyst' below.)

Most benign bone tumors have characteristic radiographic features and can be diagnosed with plain radiographs. It is important to be familiar with the radiographic appearance of the most common benign bone tumors. Benign bone tumors often are discovered incidentally, and recognition of benign lesions on plain radiographs can avoid unnecessary advanced imaging and invasive diagnostic studies.

An overview of the presentation, clinical and radiographic features, and management of the most common benign pediatric bone tumors will be presented below. Malignant bone tumors (Ewing sarcoma and osteosarcoma) are discussed separately. (See "Clinical presentation, staging, and prognostic factors of the Ewing sarcoma family of tumors" and "Osteosarcoma: Epidemiology, pathogenesis, clinical presentation, diagnosis, and histology".)

OVERVIEW

Clinical evaluation — Benign bone tumors often are asymptomatic and discovered incidentally during evaluation for trauma or another condition [1]. When they are symptomatic, benign bone tumors may present with localized pain, swelling, deformity, or pathologic fracture. In most cases, the differential diagnosis of these lesions can be narrowed based upon the age of the child, the involved bone, the location of the lesion within the bone, and other general radiographic characteristics (table 1) [2].

History — Certain aspects of the history may be helpful in narrowing the differential diagnosis of a benign-appearing bone tumor. These include (table 1) [1]:

                           

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Literature review current through: Nov 2016. | This topic last updated: Fri Apr 01 00:00:00 GMT+00:00 2016.
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References
Top
  1. Yildiz C, Erler K, Atesalp AS, Basbozkurt M. Benign bone tumors in children. Curr Opin Pediatr 2003; 15:58.
  2. Wyers MR. Evaluation of pediatric bone lesions. Pediatr Radiol 2010; 40:468.
  3. Springfield DS, Gebhardt MC. Bone and soft tissue tumors. In: Lovell and Winter's Pediatric Orthopaedics, 6th ed, Morrissy RT, Weinstein SL (Eds), Lippincott Williams & Wilkins, Philadelphia 2006. p.493.
  4. Faivre L, Nivelon-Chevallier A, Kottler ML, et al. Mazabraud syndrome in two patients: clinical overlap with McCune-Albright syndrome. Am J Med Genet 2001; 99:132.
  5. Biermann JS. Common benign lesions of bone in children and adolescents. J Pediatr Orthop 2002; 22:268.
  6. Copley L, Dormans JP. Benign pediatric bone tumors. Evaluation and treatment. Pediatr Clin North Am 1996; 43:949.
  7. Kim S, Lee S, Arsenault DA, et al. Pediatric rib lesions: a 13-year experience. J Pediatr Surg 2008; 43:1781.
  8. Wenaden AE, Szyszko TA, Saifuddin A. Imaging of periosteal reactions associated with focal lesions of bone. Clin Radiol 2005; 60:439.
  9. Tachdjian MO. Generalized affectations of the muscular skeletal system. In: Clinical Pediatric Orthopedics The Art of Diagnosis and Principles of Management, Appleton & Lange, Stamford, CT 1997. p.369.
  10. Confavreux CB, Borel O, Lee F, et al. Osteoid osteoma is an osteocalcinoma affecting glucose metabolism. Osteoporos Int 2012; 23:1645.
  11. Kaweblum M, Lehman WB, Bash J, et al. Diagnosis of osteoid osteoma in the child. Orthop Rev 1993; 22:1305.
  12. Orlowski JP, Mercer RD. Osteoid osteoma in children and young adults. Pediatrics 1977; 59:526.
  13. Horvai A, Klein M. Osteoid osteoma. In: WHO Classification of Tumours of Soft Tissue and Bone, 4th ed, Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F (Eds), International Agency for Research on Cancer, Lyons 2013. p.277.
  14. Cohen MD, Harrington TM, Ginsburg WW. Osteoid osteoma: 95 cases and a review of the literature. Semin Arthritis Rheum 1983; 12:265.
  15. Kneisl JS, Simon MA. Medical management compared with operative treatment for osteoid-osteoma. J Bone Joint Surg Am 1992; 74:179.
  16. Greenspan A. Benign bone-forming lesions: osteoma, osteoid osteoma, and osteoblastoma. Clinical, imaging, pathologic, and differential considerations. Skeletal Radiol 1993; 22:485.
  17. Iyer RS, Chapman T, Chew FS. Pediatric bone imaging: diagnostic imaging of osteoid osteoma. AJR Am J Roentgenol 2012; 198:1039.
  18. Spouge AR, Thain LM. Osteoid osteoma: MR imaging revisited. Clin Imaging 2000; 24:19.
  19. Assoun J, Richardi G, Railhac JJ, et al. Osteoid osteoma: MR imaging versus CT. Radiology 1994; 191:217.
  20. Vigorita VJ, Ghelman B. Localization of osteoid osteomas--use of radionuclide scanning and autoimaging in identifying the nidus. Am J Clin Pathol 1983; 79:223.
  21. Papathanassiou ZG, Megas P, Petsas T, et al. Osteoid osteoma: diagnosis and treatment. Orthopedics 2008; 31:1118.
  22. Gasbarrini A, Cappuccio M, Bandiera S, et al. Osteoid osteoma of the mobile spine: surgical outcomes in 81 patients. Spine (Phila Pa 1976) 2011; 36:2089.
  23. Earhart J, Wellman D, Donaldson J, et al. Radiofrequency ablation in the treatment of osteoid osteoma: results and complications. Pediatr Radiol 2013; 43:814.
  24. Aboulafia AJ, Kennon RE, Jelinek JS. Begnign bone tumors of childhood. J Am Acad Orthop Surg 1999; 7:377.
  25. de Andrea CE, Bridge JA, Schiller A. Osteoblastoma. In: WHO Classification of Tumours of Soft Tissue and Bone, 4th ed, Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F (Eds), International Agency for Research on Cancer, Lyons 2013. p.279.
  26. McLeod RA, Dahlin DC, Beabout JW. The spectrum of osteoblastoma. AJR Am J Roentgenol 1976; 126:321.
  27. Mirra JM, Cove K, Theros E, et al. A case of osteoblastoma associated with severe systemic toxicity. Am J Surg Pathol 1979; 3:463.
  28. Boriani S, Capanna R, Donati D, et al. Osteoblastoma of the spine. Clin Orthop Relat Res 1992; :37.
  29. Bovee JV, Heymann D, Wuyts W. WHO Classification of Tumours of Soft Tissue and Bone, 4th ed, Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F (Eds), International Agency for Research on Cancer, Lyons 2013. p.250.
  30. Kushner BH, Roberts SS, Friedman DN, et al. Osteochondroma in long-term survivors of high-risk neuroblastoma. Cancer 2015; 121:2090.
  31. Marcovici PA, Berdon WE, Liebling MS. Osteochondromas and growth retardation secondary to externally or internally administered radiation in childhood. Pediatr Radiol 2007; 37:301.
  32. Faraci M, Bagnasco F, Corti P, et al. Osteochondroma after hematopoietic stem cell transplantation in childhood. An Italian study on behalf of the AIEOP-HSCT group. Biol Blood Marrow Transplant 2009; 15:1271.
  33. Pannier S, Legeai-Mallet L. Hereditary multiple exostoses and enchondromatosis. Best Pract Res Clin Rheumatol 2008; 22:45.
  34. Bovée JV. Multiple osteochondromas. Orphanet J Rare Dis 2008; 3:3.
  35. Schmale GA, Conrad EU 3rd, Raskind WH. The natural history of hereditary multiple exostoses. J Bone Joint Surg Am 1994; 76:986.
  36. Wicklund CL, Pauli RM, Johnston D, Hecht JT. Natural history study of hereditary multiple exostoses. Am J Med Genet 1995; 55:43.
  37. Assefa D, Murphy RC, Bergman K, Atlas AB. Three faces of costal exostoses: case series and review of literature. Pediatr Emerg Care 2011; 27:1188.
  38. Khosla A, Parry RL. Costal osteochondroma causing pneumothorax in an adolescent: a case report and review of the literature. J Pediatr Surg 2010; 45:2250.
  39. Imai K, Suga Y, Nagatsuka Y, et al. Pneumothorax caused by costal exostosis. Ann Thorac Cardiovasc Surg 2014; 20:161.
  40. Chawla JK, Jackson M, Munro FD. Spontaneous pneumothoraces in hereditary multiple exostoses. Arch Dis Child 2013; 98:495.
  41. Roach JW, Klatt JW, Faulkner ND. Involvement of the spine in patients with multiple hereditary exostoses. J Bone Joint Surg Am 2009; 91:1942.
  42. Bernard SA, Murphey MD, Flemming DJ, Kransdorf MJ. Improved differentiation of benign osteochondromas from secondary chondrosarcomas with standardized measurement of cartilage cap at CT and MR imaging. Radiology 2010; 255:857.
  43. Garrison RC, Unni KK, McLeod RA, et al. Chondrosarcoma arising in osteochondroma. Cancer 1982; 49:1890.
  44. Ahmed AR, Tan TS, Unni KK, et al. Secondary chondrosarcoma in osteochondroma: report of 107 patients. Clin Orthop Relat Res 2003; :193.
  45. Pierz KA, Womer RB, Dormans JP. Pediatric bone tumors: osteosarcoma ewing's sarcoma, and chondrosarcoma associated with multiple hereditary osteochondromatosis. J Pediatr Orthop 2001; 21:412.
  46. Chin KR, Kharrazi FD, Miller BS, et al. Osteochondromas of the distal aspect of the tibia or fibula. Natural history and treatment. J Bone Joint Surg Am 2000; 82:1269.
  47. Pedrini E, Jennes I, Tremosini M, et al. Genotype-phenotype correlation study in 529 patients with multiple hereditary exostoses: identification of "protective" and "risk" factors. J Bone Joint Surg Am 2011; 93:2294.
  48. Malagón V. Development of hip dysplasia in hereditary multiple exostosis. J Pediatr Orthop 2001; 21:205.
  49. Lucas DR, Bridge JA. Chondromas: enchondroma, periosteal chondroma. In: WHO Classification of Tumours of Soft Tissue and Bone, 4th ed, Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F (Eds), International Agency for Research on Cancer, Lyon 2013. p.252.
  50. Silve C, Jüppner H. Ollier disease. Orphanet J Rare Dis 2006; 1:37.
  51. Pansuriya TC, van Eijk R, d'Adamo P, et al. Somatic mosaic IDH1 and IDH2 mutations are associated with enchondroma and spindle cell hemangioma in Ollier disease and Maffucci syndrome. Nat Genet 2011; 43:1256.
  52. Amary MF, Damato S, Halai D, et al. Ollier disease and Maffucci syndrome are caused by somatic mosaic mutations of IDH1 and IDH2. Nat Genet 2011; 43:1262.
  53. Bierry G, Kerr DA, Nielsen GP, et al. Enchondromas in children: imaging appearance with pathological correlation. Skeletal Radiol 2012; 41:1223.
  54. D'Angelo L, Massimi L, Narducci A, Di Rocco C. Ollier disease. Childs Nerv Syst 2009; 25:647.
  55. Brien EW, Mirra JM, Kerr R. Benign and malignant cartilage tumors of bone and joint: their anatomic and theoretical basis with an emphasis on radiology, pathology and clinical biology. I. The intramedullary cartilage tumors. Skeletal Radiol 1997; 26:325.
  56. Altay M, Bayrakci K, Yildiz Y, et al. Secondary chondrosarcoma in cartilage bone tumors: report of 32 patients. J Orthop Sci 2007; 12:415.
  57. Liu J, Hudkins PG, Swee RG, Unni KK. Bone sarcomas associated with Ollier's disease. Cancer 1987; 59:1376.
  58. Schwartz HS, Zimmerman NB, Simon MA, et al. The malignant potential of enchondromatosis. J Bone Joint Surg Am 1987; 69:269.
  59. Albregts AE, Rapini RP. Malignancy in Maffucci's syndrome. Dermatol Clin 1995; 13:73.
  60. Verdegaal SH, Bovée JV, Pansuriya TC, et al. Incidence, predictive factors, and prognosis of chondrosarcoma in patients with Ollier disease and Maffucci syndrome: an international multicenter study of 161 patients. Oncologist 2011; 16:1771.
  61. Lewis RJ, Ketcham AS. Maffucci's syndrome: functional and neoplastic significance. Case report and review of the literature. J Bone Joint Surg Am 1973; 55:1465.
  62. Ranger A, Szymczak A. Do intracranial neoplasms differ in Ollier disease and maffucci syndrome? An in-depth analysis of the literature. Neurosurgery 2009; 65:1106.
  63. Brien EW, Mirra JM, Luck JV Jr. Benign and malignant cartilage tumors of bone and joint: their anatomic and theoretical basis with an emphasis on radiology, pathology and clinical biology. II. Juxtacortical cartilage tumors. Skeletal Radiol 1999; 28:1.
  64. Miller SF. Imaging features of juxtacortical chondroma in children. Pediatr Radiol 2014; 44:56.
  65. deSantos LA, Spjut HJ. Periosteal chondroma: a radiographic spectrum. Skeletal Radiol 1981; 6:15.
  66. Bauer TW, Dorfman HD, Latham JT Jr. Periosteal chondroma. A clinicopathologic study of 23 cases. Am J Surg Pathol 1982; 6:631.
  67. Boriani S, Bacchini P, Bertoni F, Campanacci M. Periosteal chondroma. A review of twenty cases. J Bone Joint Surg Am 1983; 65:205.
  68. Kilpatrick SE, Romeo S. Chondroblastoma. In: WHO Classification of Tumours of Soft Tissue and Bone, 4th ed, Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F (Eds), International Agency for Research on Cancer, Lyon 2013. p.262.
  69. Dahlin DC, Ivins JC. Benign chondroblastoma. A study of 125 cases. Cancer 1972; 30:401.
  70. Xu H, Nugent D, Monforte HL, et al. Chondroblastoma of bone in the extremities: a multicenter retrospective study. J Bone Joint Surg Am 2015; 97:925.
  71. Romeo S, Aigner T, Bridge JA. Chondromyxoid fibroma. In: WHO Classification of Tumours of Soft Tissue and Bone, 4th ed, Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F (Eds), International Agency for Research on Cancer, Lyon 2013. p.254.
  72. Gherlinzoni F, Rock M, Picci P. Chondromyxoid fibroma. The experience at the Istituto Ortopedico Rizzoli. J Bone Joint Surg Am 1983; 65:198.
  73. Siegal GP, Bianco P, Dal Cin P. Fibrous dysplasia. In: WHO Classification of Tumours of Soft Tissue and Bone, 4th ed, Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F (Eds), International Agency for Research on Cancer, Lyons 2013. p.352.
  74. Lane JM, Khan SN, O'Connor WJ, et al. Bisphosphonate therapy in fibrous dysplasia. Clin Orthop Relat Res 2001; :6.
  75. Mansoori LS, Catel CP, Rothman MS. Bisphosphonate treatment in polyostotic fibrous dysplasia of the cranium: case report and literature review. Endocr Pract 2010; 16:851.
  76. Stevens-Simon C, Stewart J, Nakashima II, White M. Exacerbation of fibrous dysplasia associated with an adolescent pregnancy. J Adolesc Health 1991; 12:403.
  77. Mintz MC, Dalinka MK, Schmidt R. Aneurysmal bone cyst arising in fibrous dysplasia during pregnancy. Radiology 1987; 165:549.
  78. Campanacci M, Laus M. Osteofibrous dysplasia of the tibia and fibula. J Bone Joint Surg Am 1981; 63:367.
  79. Kahn LB. Adamantinoma, osteofibrous dysplasia and differentiated adamantinoma. Skeletal Radiol 2003; 32:245.
  80. McCaffrey M, Letts M, Carpenter B, et al. Osteofibrous dysplasia: a review of the literature and presentation of an additional 3 cases. Am J Orthop (Belle Mead NJ) 2003; 32:479.
  81. Wang JW, Shih CH, Chen WJ. Osteofibrous dysplasia (ossifying fibroma of long bones). A report of four cases and review of the literature. Clin Orthop Relat Res 1992; :235.
  82. Steiner GC. Fibrous cortical defect and nonossifying fibroma of bone. A study of the ultrastructure. Arch Pathol 1974; 97:205.
  83. Nielsen GP, Kyriakos M. Non-ossifying fibroma/benign fibrous histiocytoma of bone. In: WHO Classification of Tumours of Soft Tissue and Bone, 4th ed, Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F (Eds), International Agency for Research on Cancer, Lyons 2013. p.302.
  84. Kalil RK, Araujo ES. Simple bone cyst. In: WHO Classification of Tumours of Soft Tissue and Bone, 4th ed, Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F (Eds), International Agency for Research on Cancer, Lyons 2013. p.350.
  85. Reynolds J. The "fallen fragment sign" in the diagnosis of unicameral bone cysts. Radiology 1969; 92:949.
  86. Cho HS, Oh JH, Kim HS, et al. Unicameral bone cysts: a comparison of injection of steroid and grafting with autologous bone marrow. J Bone Joint Surg Br 2007; 89:222.
  87. Chang CH, Stanton RP, Glutting J. Unicameral bone cysts treated by injection of bone marrow or methylprednisolone. J Bone Joint Surg Br 2002; 84:407.
  88. Wright JG, Yandow S, Donaldson S, et al. A randomized clinical trial comparing intralesional bone marrow and steroid injections for simple bone cysts. J Bone Joint Surg Am 2008; 90:722.
  89. Zhao JG, Ding N, Huang WJ, et al. Interventions for treating simple bone cysts in the long bones of children. Cochrane Database Syst Rev 2014; :CD010847.
  90. Singh S, Dhammi IK, Arora A, Kumar S. Unusually large solitary unicameral bone cyst: case report. J Orthop Sci 2003; 8:599.
  91. Shih HN, Chen YJ, Huang TJ, et al. Semistructural allografting in bone defects after curettage. J Surg Oncol 1998; 68:159.
  92. Donaldson S, Wright JG. Simple bone cysts: better with age? J Pediatr Orthop 2015; 35:108.
  93. Dahlin DC, McLeod RA. Aneurysmal bone cyst and other nonneoplastic conditions. Skeletal Radiol 1982; 8:243.
  94. Nielsen GP, Fletcher JA, Oliveira AM. Aneurysmal bone cyst. In: WHO Classification of Tumours of Soft Tissue and Bone, 4th ed, Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F (Eds), International Agency for Research on Cancer, Lyons 2013. p.348.
  95. Novais EN, Rose PS, Yaszemski MJ, Sim FH. Aneurysmal bone cyst of the cervical spine in children. J Bone Joint Surg Am 2011; 93:1534.
  96. Ibrahim T, Howard AW, Murnaghan ML, Hopyan S. Percutaneous curettage and suction for pediatric extremity aneurysmal bone cysts: is it adequate? J Pediatr Orthop 2012; 32:842.
  97. Skubitz KM, Peltola JC, Santos ER, Cheng EY. Response of Aneurysmal Bone Cyst to Denosumab. Spine (Phila Pa 1976) 2015; 40:E1201.
  98. Lange T, Stehling C, Fröhlich B, et al. Denosumab: a potential new and innovative treatment option for aneurysmal bone cysts. Eur Spine J 2013; 22:1417.
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