Chondroblastoma

Jump to: navigation, search

WikiDoc Resources for Chondroblastoma

Articles

Most recent articles on Chondroblastoma

Most cited articles on Chondroblastoma

Review articles on Chondroblastoma

Articles on Chondroblastoma in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Chondroblastoma

Images of Chondroblastoma

Photos of Chondroblastoma

Podcasts & MP3s on Chondroblastoma

Videos on Chondroblastoma

Evidence Based Medicine

Cochrane Collaboration on Chondroblastoma

Bandolier on Chondroblastoma

TRIP on Chondroblastoma

Clinical Trials

Ongoing Trials on Chondroblastoma at Clinical Trials.gov

Trial results on Chondroblastoma

Clinical Trials on Chondroblastoma at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Chondroblastoma

NICE Guidance on Chondroblastoma

NHS PRODIGY Guidance

FDA on Chondroblastoma

CDC on Chondroblastoma

Books

Books on Chondroblastoma

News

Chondroblastoma in the news

Be alerted to news on Chondroblastoma

News trends on Chondroblastoma

Commentary

Blogs on Chondroblastoma

Definitions

Definitions of Chondroblastoma

Patient Resources / Community

Patient resources on Chondroblastoma

Discussion groups on Chondroblastoma

Patient Handouts on Chondroblastoma

Directions to Hospitals Treating Chondroblastoma

Risk calculators and risk factors for Chondroblastoma

Healthcare Provider Resources

Symptoms of Chondroblastoma

Causes & Risk Factors for Chondroblastoma

Diagnostic studies for Chondroblastoma

Treatment of Chondroblastoma

Continuing Medical Education (CME)

CME Programs on Chondroblastoma

International

Chondroblastoma en Espanol

Chondroblastoma en Francais

Business

Chondroblastoma in the Marketplace

Patents on Chondroblastoma

Experimental / Informatics

List of terms related to Chondroblastoma

For patient information click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Rohan A. Bhimani, M.B.B.S., D.N.B., M.Ch.[2]

Synonyms and Keywords: Codman tumor; Codman's tumor; Codman tumour; Codmans tumor; Chondroblastomas; Benign chondroblastoma; Epiphyseal chondroblastoma

Overview

Chondroblastoma is a benign cartilaginous tumor that account for 1% of all bone tumors. Chondroblastoma typically occurs in children and adolescents between 10 and 25 years of age. Male are more commonly affected with chondroblastoma than female. The male to female ratio is approximately 2:1. The epiphysis of the distal femur, proximal tibia, and proximal humerus are the most common sites. In 1928, Codman first described chondroblastoma as epiphyseal chondromatous giant cell tumors of the proximal humerus. The exact pathogenesis of chondroblaroma is not fully understood. Various theories have been postulated such as chondroblastoma arising from osteoid matrix–containing type I collagen and there is absence of true cartilage matrix production (Type II collagen). Recently a specific mutation in the histone 3 gene H3F3B (K36M), was identified in approximately 95% of chondroblastomas. The hallmark of chondroblastoma is pain, limping, tenderness to palpation and restricted range of motion. If left untreated, few patients with chondroblastoma may progress to develop lung metastasis. The mainstay of treatment for chondroblastoma is surgery in form of curettage and bone grafting.

Historical Perspective

  • In 1928, Codman first described chondroblastoma as epiphyseal chondromatous giant cell tumors of the proximal humerus.
  • In 1942, Jaffe and Lichenstein later recognized it as a distinct entity due to its chondorid matrix.[1]

Classification

Chondroblastoma can be classified based on imaging findings.

Enneking (MSTS) Staging System

  • The Enneking surgical staging system (also known as the MSTS system) for benign musculoskeletal tumors based on radiographic characteristics of the tumor host margin.[2]
  • It is widely accepted and routinely used classification.
Stages Description
1 Latent: Well demarcated borders
2 Active: Indistinct borders
3 Aggressive: Indistinct borders

Pathophysiology

Genetics

  • Chondroblastoma may have association with genetic abnormalities on chromosome 5 and 8.
  • Recently a specific mutation in the histone 3 gene H3F3B (K36M), was identified in approximately 95% of chondroblastomas.[8][9]

Causes

There are no established causes for chondroblastoma.[10]

Differentiating Chondroblastoma from Other Diseases

  • Chondroblastoma must be differentiated from following bone disorders:
Disease Epiphyseal lesion Benign lesion that may metastasize to lung Diagnosis Treatment is curretage and bone grafting
Chondroblastoma + + Radiology and biopsy +
Giant Cell Tumor + + Radiology and biopsy +
Aneurysmal bone cyst - - Radiology and biopsy +
Osteoblastoma - - Biopsy +
Chondromyxoid Fibroma - - Radiology and biopsy +

Epidemiology and Demographics

  • Chondroblastoma is a rare benign bone tumor accounting for approximately 1% of all benign bone tumors.[11]
  • Adolescents and children are most affected by chondroblastoma.
  • The age distribution of chondroblastoma is between 10-25 years.[12][13]
  • The mean age of the patients with chondroblastoma is 22 years.[12][13]
  • Men are more commonly affected than women, with a 2:1 ratio.
  • There is no racial predilection to chondroblastoma.

Risk Factors

There are no established risk factors for chondroblastoma.

Screening

There is insufficient evidence to recommend routine screening for chondroblastoma.

Natural History, Complications, and Prognosis

  • If left untreated, few patients with chondroblastoma may progress to develop lung metastasis.[14]
  • Common complications of chondroblastoma include:[1][14][15]
    • Pathological fracture
    • Premature epiphyseal closure
      • Limb-length discrepancy
      • Angular deformity
    • Malignant transformation
    • Metastasis to other organs
      • Metastasis most frequently involves the lungs and tends to occur at the time of primary tumor recurrence
      • Other sites of metastasis are secondary bone sites, soft tissue, skin, or the liver
  • Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as excellent.[16]
  • Factors that influence the outcome of the chondroblastoma include:
    • Extent of the tumor
    • Size and location of the tumor
    • Pulmonary metastasis
    • Tumor response to the therapy

Diagnosis

Diagnostic Study of Choice

  • X-ray is the diagnostic study of choice for the diagnosis of chondroblastoma.
  • X-ray findings include:
  • Well-circumscribed eccentric oval or round lytic lesion that usually involves the adjacent bone cortex without periosteal reaction.
  • A sclerotic margin can be observed in some cases.
  • For long bone chondroblastoma the tumor is typically contained to the epiphysis or apophysis but may extend through the epiphyseal plate.
  • A mottled appearance on the radiograph indicates areas of calcification which is commonly associated with skeletally immature patients
  • In cases involving older patients or flat bones, typical radiographic presentation is not as common and may mimic aggressive processes

History and Symptoms

Symptoms of chondroblastoma include:[17][1]

  • Localized bone pain with activity and rest
    • Pain often present for months or years; frequently misdiagnosed as chronic synovitis
  • Localized swelling
  • Decreased range of motion of the affected joint
  • Limp

Physical Examination

  • Patients with chondroblastoma usually appears well.
  • Common physical examination findings of chondroblastoma include:[18]
X-ray of Knee: Chondroblastoma of proximal tibia.Source: Case courtesy of Radswiki, Radiopaedia.org, rID: 11302

Laboratory Findings

There are no diagnostic laboratory findings associated with chondroblastoma.

Electrocardiogram

There are no ECG findings associated with chondroblastoma.

X-ray

  • Three views of affected bone or joint are recommended.
  • X-ray findings include:
    • well-circumscribed epiphyseal lytic lesion with thin rim of sclerotic bone that is sharply demarcated from normal medullary cavity .
    • Lesions often cross physis into metaphysis.
    • Stippled calcifications within the lesion may or may not be present (25%-45%).
    • Cortical expansion may be present.
    • Soft tissue expansion rare.
CT scan of Knee: Chondroblastoma is subchondral, lytic, having geographic appearance, narrow transitional zone and surrounding sclerosis..Source:Case courtesy of Dr Ali Abougazia, Radiopaedia.org, rID: 25725

Chest X-Ray

  • Chest radiograph should be done to look for benign pulmonary metastasis which may occasionally may occur with chondroblastoma.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with chondroblastoma.

CT scan

CT findings for chondroblastoma include:

MRI

  • MRI of chondroblastoma is ideal for the evaluation of transphyseal or transcortical extension, and for demonstrating associated surrounding bone marrow and soft tissue edema, which is observed in a large proportion of cases.
  • MRI Findings include:[19]
MRI scan of Knee: High T2 signals with a thin sclerotic rim and surrounding marrow edema..Source:Case courtesy of Dr Ali Abougazia, Radiopaedia.org, rID: 25725
    • The lesions have signal typical of cartilage:
      • T1: low to intermediate signal
      • T2: intermediate to high signal
    • Focal lobules of low, intermediate, and high signal intensity most likely correspond to calcification, chondroid matrix, and fluid in the lesion.
    • MRI may also reveal an effusion of the joint.
    • Fluid-fluid levels may occasionally be observed presumably due to an associated aneurysmal bone cyst.

Other Imaging Findings

There are no other imaging findings associated with chondroblastoma.

Other Diagnostic Studies

Biopsy

  • Biopsy may be helpful in the diagnosis of chondroblastoma.
  • Biopsy grossly demonstrates the following features:
Histological appearance: Chondroblastoma
  • On histological examination:
    • Chondroblasts arranged in "cobblestone" or "chickenwire" pattern may be present.
    • Scattered multinucleated giant cells with focal areas of chondroid matrix.
    • Occasional multinucleated giant cells may be present
    • Mononuclear stromal cells are distinct, S100+ cells with large central nuclei.
      • Nuclei have longitudinal groove resembling coffee bean.
    • One-third of chonroblastomas have areas of secondary Aneurysmal bone cyst.


Treatment

Medical Therapy

There is no treatment for chondroblastoma; the mainstay of therapy is surgery.

Surgery

Surgery is the mainstay of treatment for chondroblastoma.[17][1][20][21][22]

Extended Intralesional Curettage and Bone Grafting

Indications

  • Symptomatic individuals.

Technique

Radiofrequency Ablation

  • Another procedure that may be used for the management of chondroblastoma is radiofrequency ablation which is less invasive and has less recovery time.
  • Radiofrequency ablation is typically most successful for small chondroblastoma lesions about 1.5 cm.

Surgical Resection

Indications

Recurrence

  • Local recurrence rate is 10-15% after treatment.

Primary Prevention

There are no established measures for the primary prevention of chondroblastoma.

Secondary Prevention

There are no established measures for the secondary prevention of chondroblastoma.

References

  1. 1.0 1.1 1.2 1.3 Fitzgerald, Judd; Broehm, Cory; Chafey, David; Treme, Gehron (2014). "Chondroblastoma of the Knee Treated with Resection and Osteochondral Allograft Reconstruction". Case Reports in Orthopedics. 2014: 1–7. doi:10.1155/2014/543959. ISSN 2090-6749.
  2. Jawad MU, Scully SP (2010). "In brief: classifications in brief: enneking classification: benign and malignant tumors of the musculoskeletal system". Clin Orthop Relat Res. 468 (7): 2000–2. doi:10.1007/s11999-010-1315-7. PMC 2882012. PMID 20333492.
  3. Peabody, Terrance (2014). Orthopaedic oncology : primary and metastatic tumors of the skeletal system. Cham: Springer. ISBN 9783319073224.
  4. Mii Y, Miyauchi Y, Morishita T, Miura S, Honoki K, Aoki M; et al. (1994). "Ultrastructural cytochemical demonstration of proteoglycans and calcium in the extracellular matrix of chondroblastomas". Hum Pathol. 25 (12): 1290–4. PMID 8001922.
  5. Aigner T, Loos S, Inwards C, Perris R, Perissinotto D, Unni KK; et al. (1999). "Chondroblastoma is an osteoid-forming, but not cartilage-forming neoplasm". J Pathol. 189 (4): 463–9. doi:10.1002/(SICI)1096-9896(199912)189:4<463::AID-PATH476>3.0.CO;2-N. PMID 10629544.
  6. Brien EW, Mirra JM, Ippolito V (1995). "Chondroblastoma arising from a nonepiphyseal site". Skeletal Radiol. 24 (3): 220–2. PMID 7610417.
  7. Punit A, Nadkarni S, Doomra T (2014). "Chondroblastoma of Diaphysis of Radius in a Seven Year Old Child". J Orthop Case Rep. 4 (3): 32–5. doi:10.13107/jocr.2250-0685.191. PMC 4719322. PMID 27298978.
  8. Amary MF, Berisha F, Mozela R, Gibbons R, Guttridge A, O'Donnell P; et al. (2016). "The H3F3 K36M mutant antibody is a sensitive and specific marker for the diagnosis of chondroblastoma". Histopathology. 69 (1): 121–7. doi:10.1111/his.12945. PMID 26844533.
  9. Behjati S, Tarpey PS, Presneau N, Scheipl S, Pillay N, Van Loo P; et al. (2013). "Distinct H3F3A and H3F3B driver mutations define chondroblastoma and giant cell tumor of bone". Nat Genet. 45 (12): 1479–82. doi:10.1038/ng.2814. PMC 3839851. PMID 24162739.
  10. Peabody, Terrance (2014). Orthopaedic oncology : primary and metastatic tumors of the skeletal system. Cham: Springer. ISBN 9783319073224.
  11. Dahlin DC, Ivins JC (1972). "Benign chondroblastoma. A study of 125 cases". Cancer. 30 (2): 401–13. PMID 5051664.
  12. 12.0 12.1 Bulanov DV, Semenova LA, Makhson AN, Bulycheva IV (2007). "[Chondrosarcoma of the larynx]". Arkh Patol. 69 (6): 50–2. PMID 18290384.
  13. 13.0 13.1 Turcotte RE, Kurt AM, Sim FH, Unni KK, McLeod RA (1993). "Chondroblastoma". Hum Pathol. 24 (9): 944–9. PMID 8253461.
  14. 14.0 14.1 Bloem JL, Mulder JD (1985). "Chondroblastoma: a clinical and radiological study of 104 cases". Skeletal Radiol. 14 (1): 1–9. PMID 4023729.
  15. Brien EW, Mirra JM, Kerr R (1997). "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. 26 (6): 325–53. PMID 9229417.
  16. Caterini R, Manili M, Spinelli M, Santori FS, Ippolito E (1992). "Epiphyseal chondroblastoma of bone. Long-term effects on skeletal growth and articular function in 15 cases treated surgically". Arch Orthop Trauma Surg. 111 (6): 327–32. PMID 1449941.
  17. 17.0 17.1 Tonogai, Ichiro; Takahashi, Mitsuhiko; Manabe, Hiroaki; Nishisho, Toshihiko; Iwamoto, Seiji; Takao, Shoichiro; Kagawa, Seiko; Kudo, Eiji; Yasui, Natsuo (2013). "A Massive Chondroblastoma in the Proximal Humerus Simulating Malignant Bone Tumors". Case Reports in Orthopedics. 2013: 1–5. doi:10.1155/2013/673576. ISSN 2090-6749.
  18. Peabody, Terrance (2014). Orthopaedic oncology : primary and metastatic tumors of the skeletal system. Cham: Springer. ISBN 9783319073224.
  19. Tiwari M, Chaturvedi H, Patel V, Matti R (2016). "Chondroblastoma of the Medial Malleolus: A Case Report of A Rare Tumor at an Extremely Uncommon Site". J Orthop Case Rep. 6 (1): 29–32. doi:10.13107/jocr.2250-0685.370. PMC 4845405. PMID 27299121.
  20. Xu H, Nugent D, Monforte HL, Binitie OT, Ding Y, Letson GD; et al. (2015). "Chondroblastoma of bone in the extremities: a multicenter retrospective study". J Bone Joint Surg Am. 97 (11): 925–31. doi:10.2106/JBJS.N.00992. PMID 26041854.
  21. Cho HS, Park YK, Oh JH, Lee JH, Han I, Kim HS (2016). "Proximal Tibia Chondroblastoma Treated With Curettage and Bone Graft and Cement Use". Orthopedics. 39 (1): e80–5. doi:10.3928/01477447-20151222-04. PMID 26726978.
  22. Lehner B, Witte D, Weiss S (2011). "Clinical and radiological long-term results after operative treatment of chondroblastoma". Arch Orthop Trauma Surg. 131 (1): 45–52. doi:10.1007/s00402-010-1099-y. PMID 20364261.



Linked-in.jpg