Introduction
Background
A chondroblastoma is a rare, usually benign, tumor of bone that accounts for approximately 1% of all bone tumors. In 1931, Codman classified it as a chondromatous variant of giant cell tumors, when he described these lesions in the proximal humerus.1 A decade later, Jaffe and Lichtenstein renamed the Codman tumor a benign chondroblastoma to emphasize the chondroblastic genesis of the lesion and to distinguish it from the classic giant cell tumor of bone.2
Pathophysiology
Various theories have been proposed concerning the origin of chondroblastomas. Mii and colleagues described the results of ultrastructural examination of chondroblastomas.3 Their studies showed subcellular, calcium-containing precipitates that are similar to those seen in chondrocytes. Based on these findings, the authors concluded that the tumors are of chondrogenic origin. Aigner and colleagues, however, noted the presence of osteoid matrix–containing type I collagen and the absence of true cartilage matrix production.4 They considered the term chondroblastoma to be a misnomer and believed that the tumor should be reclassified as a bone-forming neoplasm.
Brien and colleagues compared the characteristics of chondroblastoma of bone to chondroblastoma of soft tissue, giant cell tumor of the tendon sheath (GCTTS), and pigmented villonodular synovitis (PVNS).5 On examination of about 15 examples of GCTTS and PVNS, large areas of chondroid differentiation were noted that could not be distinguished from chondroblastoma of bone by either histologic or electron microscopic features. The researchers theorized that chondroblastoma of bone stems from an intraosseous proliferation of tendon sheath cells that have a predilection for chondroid formation. While the exact etiology of chondroblastoma remains uncertain, the presentation, appropriate evaluation, and treatment of patients with the condition have been well described.
Chondroblastomas typically occur in the epiphyses of tubular long bones. The distal femoral and proximal tibial epiphyses are most frequently involved, followed by the proximal humerus, where approximately 18% of chondroblastomas appear.
Frequency
United States
Chondroblastoma accounts for approximately 1% of all bone tumors.
International
International incidence is not reported in current literature.
Mortality/Morbidity
Patients with benign chondroblastoma may limit activities due to pain. Malignant chondroblastomas, which may occur many years after the original lesion (even in the absence of radiation), are extremely rare and are associated with a dismal prognosis.
Race
No racial predilection is recognized.
Sex
The male-to-female ratio is 2:1 in most series.
Age
Approximately 92% of patients presenting with chondroblastoma are younger than 30 years. However, chondroblastomas have been reported to arise in patients as young as 2 years and as old as 83 years. In several large series, most patients were diagnosed in the second decade of life.
Clinical
History
Pain is the most common presenting symptom. It typically is mild and gradually progressive and initially may be attributed to a minor injury. If the lesion is juxta-articular, the patient may complain of joint swelling or diminished range of motion. Usually, constitutional symptoms are lacking. In their series of 70 patients, Turcotte and colleagues found the average duration of symptoms in patients with chondroblastoma to be 20 months.6
Physical
The physical examination is remarkable for localized tenderness in most patients. Soft-tissue swelling, mass, or joint effusion is present in about 20% of cases. Muscular atrophy or decreased joint motion is less common.
Causes
No risk factors are known for chondroblastoma. There have been reports of abnormalities in chromosomes 5 and 8, as well as of p53 mutations, in patients with chondroblastoma. Sj ö gren and colleagues performed cytogenetic analysis of benign and malignant cartilage tumors7 , and while they observed no consistent karyotypic abnormalities, there were recurrent breakpoints seen at 2q35, 3q21-23, and 18q21.
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References
Codman EA. The classic: epiphyseal chondromatous giant cell tumors of the upper end of the humerus. Surg Gynecol Obstet.1931;52:543. Clin Orthop Relat Res. Sep 2006;450:12-6. [Medline].
Jaffe HL, Lichtenstein L. Benign chondroblastoma of bone: a reinterpretation of the so-called calcifying or chondromatous giant cell tumor. Am J Pathol. 1942;18:969-91.
Mii Y, Miyauchi Y, Morishita T, et al. Ultrastructural cytochemical demonstration of proteoglycans and calcium in the extracellular matrix of chondroblastomas. Hum Pathol. Dec 1994;25(12):1290-4. [Medline].
Aigner T, Loos S, Inwards C, et al. Chondroblastoma is an osteoid-forming, but not cartilage-forming neoplasm. J Pathol. Dec 1999;189(4):463-9. [Medline].
Brien EW, Mirra JM, Ippolito V. Chondroblastoma arising from a nonepiphyseal site. Skeletal Radiol. Apr 1995;24(3):220-2. [Medline].
Turcotte RE, Kurt AM, Sim FH, et al. Chondroblastoma. Hum Pathol. Sep 1993;24(9):944-9. [Medline].
Sjögren H, Orndal C, Tingby O, et al. Cytogenetic and spectral karyotype analyses of benign and malignant cartilage tumours. Int J Oncol. Jun 2004;24(6):1385-91. [Medline].
Springfield DS, Capanna R, Gherlinzoni F, et al. Chondroblastoma. A review of seventy cases. J Bone Joint Surg Am. Jun 1985;67(5):748-55. [Medline].
Kyriakos M, Land VJ, Penning HL, et al. Metastatic chondroblastoma. Report of a fatal case with a review of the literature on atypical, aggressive, and malignant chondroblastoma. Cancer. Apr 15 1985;55(8):1770-89. [Medline].
Ostrowski ML, Johnson ME, Truong LD, et al. Malignant chondroblastoma presenting as a recurrent pelvic tumor with DNA aneuploidy and p53 mutation as supportive evidence of malignancy. Skeletal Radiol. Nov 1999;28(11):644-50. [Medline].
Dahlin DC, Ivins JC. Benign chondroblastoma. A study of 125 cases. Cancer. Aug 1972;30(2):401-13. [Medline].
Dorfman HD, Czerniak B. Cartilage Tumors. In: Bone Tumors. St Louis, Mo: Mosby; 1998:317.
Fanning CV, Sneige NS, Carrasco CH, et al. Fine needle aspiration cytology of chondroblastoma of bone. Cancer. Apr 15 1990;65(8):1847-63. [Medline].
Jambhekar NA, Desai PB, Chitale DA. Benign metastasizing chondroblastoma: a case report. Cancer. Feb 1998;82(4):675-8. [Medline].
Jee WH, Park YK, McCauley TR, et al. Chondroblastoma: MR characteristics with pathologic correlation. J Comput Assist Tomogr. Sep-Oct 1999;23(5):721-6. [Medline].
Khalili K, White LM, Kandel RA, et al. Chondroblastoma with multiple distant soft tissue metastases. Skeletal Radiol. Aug 1997;26(8):493-6. [Medline].
Kurt AM, Turcotte RE, McLeod RA, et al. Chondroblastoma of bone. Orthopedics. Jul 1990;13(7):787-90. [Medline].
Kurt AM, Unni KK, Sim FH, et al. Chondroblastoma of bone. Hum Pathol. Oct 1989;20(10):965-76. [Medline].
Mermelstein LE, Friedlaender GE, Katz LD. Cystic chondroblastoma. Orthopedics. Jan 1997;20(1):69-71. [Medline].
Mirra JM, Ulich TR, Eckardt JJ, et al. "Aggressive" chondroblastoma. Light and ultramicroscopic findings after en bloc resection. Clin Orthop Relat Res. Sep 1983;(178):276-84. [Medline].
Pflueger P, Heinrich SD, Craver R. Chondroblastoma. Orthopedics. Mar 1993;16(3):339-42. [Medline].
Ramappa AJ, Lee FY, Tang P, et al. Chondroblastoma of bone. J Bone Joint Surg Am. Aug 2000;82-A(8):1140-5. [Medline].
Rodgers WB, Mankin HJ. Metastatic malignant chondroblastoma. Am J Orthop. Dec 1996;25(12):846-9. [Medline].
Swarts SJ, Neff JR, Johansson SL, et al. Significance of abnormalities of chromosomes 5 and 8 in chondroblastoma. Clin Orthop Relat Res. Apr 1998;(349):189-93. [Medline].
Weatherall PT, Maale GE, Mendelsohn DB, et al. Chondroblastoma: classic and confusing appearance at MR imaging. Radiology. Feb 1994;190(2):467-74. [Medline].
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Further Reading
Keywords
CB, bone tumor, giant cell tumor, GCT, benign chondroblastoma, Codman's tumor, Codman tumor, bone-forming neoplasm, malignant chondroblastoma, aneurysmal bone cyst
Overview: Chondroblastoma