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J KMA Special Issue Bone Tumors Specific in Children Sung Taek Jung, MD Kwang Cheul Jeong, M.D Department of Orthopedic Surgery, Chonnam National University College of Medicine E mail : stjung@chonnam.ac.kr hikcj2005@naver.com J Korean Med Assoc 2006; 49(12): 1068-1081 Abstract Primary bone tumors in pediatric age group are uncommon, and even when they do occur, they are usually benign. The primary malignant tumors that occur predominantly in children are two bone tumors, namely, osteosarcoma and Ewing's sarcoma. An adequate history and physical examination are the first and most important steps in evaluating a patient with a bone tumor. All suspected bone tumors should be evaluated initially with plain roentgenograms. Then the additional diagnostic studies, such as computed tomography(ct), magnetic resonance imaging(mri) and technetium bone scan can be used, if necessary. Biopsy should be the last step in evaluation. Most of benign bone tumors usually do not require treatment other than a periodic follow up evaluation. The optimal treatment of the malignant bone tumor often requires a combination of radiation therapy, chemotherapy, and wide surgical excision or amputation. Early detection of a malignant bone tumor not only may make the difference between life and death but also may allow successful salvage surgery rather than amputation of the limb. Keywords : Pediatric; Done tumor; Benign; Malignant 1068

Bone Tumors Specific in Children Classification of pediatric bone tumors based on tissue of origin Origin Bone origin Cartilaginous origin Fibrous origin Miscellaneous Metastatic tumors Tumor Osteoid osteoma, osteoblastoma, osteosarcoma Osteochondroma, chondroblastoma, chondromyxoid fibroma, enchondroma, periosteal chondroma Nonossifying fibroma, fibrous dysplasia, osteofibrous dysplasia, desmoplastic fibroma Unicameral bone cyst, aneurysmal bone cyst, giant cell tumor, Langerhans cell histiocytosis, Ewing sarcoma, musculoskeletal manifestation of leukemia, lymphoma Neuroblastoma, retinoblastoma, hepatoblastoma A B C (A) Geographic pattern (B) Moth eaten pattern (C) Permeative pattern Different patterns of bone destruction 1069

Jung ST Jeong KC Common locations of pediatric bone tumor Epiphysis Chondroblastoma Brodie's abscess of the epiphysis Giant cell tumor Fibrous dysplasia Diaphysis Fibrous dysplasia Osteofibrous dysplasia Langerhans cell histiocytosis Ewing sarcoma Leukemia, lymphoma Osteoid osteoma Unicameral bone cyst Anterior elements of spine Langerhans cell histiocytosis Leukemia Metastasis Giant cell tumor Pelvis Ewing sarcoma Osteosarcoma Osteochondroma Metastasis Fibrous dysplasia Metaphysis Any tumor Multiple Leukemia (metastasis) Multiple hereditary exostosis Langerhans cell histiocytosis Polyostotic fibrous dysplasia Enchondromatosis Metaphysis Aneurysmal bone cyst Osteoblastoma Osteoid osteoma Rib Fibrous dysplasia Langerhans cell histiocytosis Ewing sarcoma Metastasis (A) Codman triangle (B) Onion skin (C) Sunburst Different patterns of periosteal reaction 1070

Bone Tumors Specific in Children Staging of musculoskeletal tumors Stage Grade Site and Size Metastasis IA Low Intracompartmental None IB Low Extracompartmental None IIA High Intracompartmental None IIB High Extracompartmental None III Any Any Yes From Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin Othop Relat Res 1980; 153: 106 1071

Jung ST Jeong KC Radical Margin Wide Margin Marginal Margin Intralesional Margin Osteoid soteoma(ct) Surgical margins 1072

Bone Tumors Specific in Children Conventional osteosarcoma Conventional osteosarcoma 1073

Jung ST Jeong KC A (A) sessile type Osteochondroma (B) pedunculated type B 1074

Bone Tumors Specific in Children Enchondroma 1075

Jung ST Jeong KC Shepherd's crook deformity of right hip Nonossifying fibroma 1076

Bone Tumors Specific in Children Osteofibrous dysplasia 1077

Jung ST Jeong KC Simple bone cyst 1078

Bone Tumors Specific in Children Ewing's sarcoma 1079

Jung ST Jeong KC Radiol Clin North Am 1981; 19: 715-48 3. Ragsdale BD, Madewell JE, Sweet DE. Radilogic and pathologic analysis of solitary bone lesions: Part periosteal reactions. Radiol Clin North Am 1981; 19: 749-83 4. Springfield DS, Gebghardt MC(2001). Bone and soft tissue tumors. In: Lovell and Winter s Pediatric Orthopaedics, 5th edetion (Morrissy RT, Weinstein SL, eds). Philadelphia: Lippincott Williams & Willinkins, 2001: 507-62 5. Heinrich SD, Scarborough MT, eds. Pediatric Orthopaedic Oncology. Orthop Clin North Am, 1996 6. Jaramillo D, Laor T, Gebhardt MC. Pediatric musculoskeletal neoplasms: evaluation with MR imaging. Magn Reson Imaging Clin N Am 1996; 4: 749-70 7. Mankin HJ, Lange TA, Spanier S. The hazards of the biopsy in patients with malignant primary bone and soft tissue tumors. J Bone Joint Surg Am 1982; 64: 1121-7 8. Mankin Hj, Mankin CJ, Simon MA. The hazards of the biopsy, revisited. Members of the Musculoskeletal Tumor Society. J Bone Joint Surg Am 1996; 78: 656-63 9. Simon MA, Biermann JS. Biopsy of bone and soft tissue lesions. J Bone Joint Surg Am 1993; 75: 616-21 10. Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res 1980; 153: 106-20 11. Huvos AG. Bon tumors: Diagnosis, Treatment, and Prognosis, 2nd ed. Philadelphia: WB Saunders, 1991 12. Greenspan A, Remagen W. Differential Diagnosis of Tumors 1. Enneking W. Musculoskeletal tumor surgery. New York: Churchill Livingstone, 1983 2. Madwell JE, Ragsdale BD, Sweet DE. Radiologic and pathologic analysis of solitary bone lesions: Part Internal margins. and Tumor like Lesions of Bone and Joints. Philadelphia: Lippincott Raven, 1998 13. Himelstein BP, Dormans JP. Malignant bone tumors of childhood. Pediatr Clin North Am 1996; 43: 967-84 1080

Bone Tumors Specific in Children 14. Rougraff BT, Simon MA, Kneisl JS, Greenberg DB, Mankin HJ. Limb salvage compared with amputation for osteosarcoma of the distal end of the femur: a long term ontological, functional, and quality of life study. J Bone Joint Surg Am 1994; 76 : 649-56 15. Copley L, Dormans JP. Benign pediatric bone tumors: Evaluation and treatment. Pediatr Clin North Am 1996; 43: 949-66 16. Dormans JP, Flynn JM. Pathologic fracture associated with tumors and unique conditions of the muscoloskeletal edition. In: Rockwood and Wilkin's Fractures in Children, 5th ed.(beaty JH, Kasser JR, eds) Philadelphia: Lippincott Williams & Wilkins, 2001 17. Dormans JP, Phill SG. Fractures through bone cysts: Unicameral bone cysts, aneurysmal bone cysts, fibrous cortical defects, and nonossifying fibromas, Instr Cours Lect 2002; 51: 457-67 18. Grabias SL, Campbell CJ. Fibrous dysplasia. Orthop Clin North 19. Martinez V, Sissons HA. Aneurysmal bone cyst: a review of 123 cases including primary lesions and those secondary to other bone pathology. Cancer 1988; 61: 2291-304 20. Szendroi M, Arato G, Ezzati A, Huttl K, Szavcsur P. Aneurysmal bone cyst: its pathogenesis based on angiographic, immunohistochemical and eletron microscopic studies. Pathol Oncol Res 1998; 4: 277-81 21. Dormans JP, Hanna BG, Johnston DR, Khurana JS. Surgical treatment and recurrence rate of aneurysmal bone cysts in children. Clin Orthop 2004; 421: 205-11 22. Sessa S, Sommelet D, Lascombes P, Prevot J. Treatment of Langerhans cell histiocytosis in children: experience at the Children's hospital of Nancy. J Bone Joint Surg Am 1994; 76: 1513-25 23. Bernstrand C, Bjork O, Ahstrom L, Henter JI. Intralesional steroids in Langerhans cell histiocytosis of bone. Acta Paediatr 1996; 85: 502-4 Am 1977; 8: 771-83 Peer Reviewer Commentary 1081