From: Subject: eMedicine - Chondroblastoma : Article by Hannah D Morgan, MD Date: Sun, 6 Jul 2003 20:36:44 +0100 MIME-Version: 1.0 Content-Type: multipart/related; boundary="----=_NextPart_000_0000_01C343FE.50515ED0"; type="text/html" X-MimeOLE: Produced By Microsoft MimeOLE V6.00.2800.1165 This is a multi-part message in MIME format. ------=_NextPart_000_0000_01C343FE.50515ED0 Content-Type: text/html; charset="Windows-1252" Content-Transfer-Encoding: quoted-printable Content-Location: http://www.emedicine.com/orthoped/topic469.htm eMedicine - Chondroblastoma : Article by Hannah D = Morgan, MD
 
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Chondroblastoma

Last Updated: = April 17,=20 2002
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Synonyms and related = keywords:=20 CB, bone tumor, giant cell tumor, GCT, benign = chondroblastoma,=20 Codman's tumor, Codman tumor, bone-forming neoplasm, = malignant=20 chondroblastoma, aneurysmal bone cyst =

  AUTHOR INFORMATION=20 Section 1 of = 11   =20
Author=20 Information Introduction=20 = Clinical=20 Differentials=20 Wo= rkup=20 Treatment=20 Medication=20 Follow-up=20 Miscellaneous=20 = Pictures=20 Bibliography
Author: Hannah=20 D Morgan, MD, Acting Instructor, Department of=20 Orthopedics and Sports Medicine, University of Washington

Coauthor(s): Timothy=20 Damron, MD, Associate Professor, Department of=20 Orthopedics, Division of Orthopedic Oncology - Joint = Reconstructive=20 Surgery, State University of New York at Syracuse, Upstate = Medical=20 University

Editor(s): Howard A Chansky, MD, = Associate=20 Professor, Department of Orthopedics and Sports Medicine, = University=20 of Washington Medical Center; Francisco Talavera, = PharmD,=20 PhD, Senior Pharmacy Editor, Pharmacy, eMedicine;=20 Sean P Scully, MD, PhD, Senior Associate=20 Consultant, Department of Orthopedics, Mayo Clinic of = Rochester;=20 Dinesh Patel, MD, Assistant Clinical = Professor of=20 Orthopedic Surgery, Harvard Medical School; Chief of = Arthroscopic=20 Surgery, Department of Orthopedic Surgery, Massachusetts = General=20 Hospital; and Harris Gellman, MD, Private = Practice,=20 Broward Hand Center

Background: A=20 chondroblastoma is a rare, usually benign, tumor of bone, which = accounts=20 for approximately 1% of all bone tumors. In 1931, Codman = classified it as=20 a chondromatous variant of giant cell tumors when he described = these=20 lesions in the proximal humerus. A decade later, Jaffe and = Lichtenstein=20 renamed Codman tumor benign chondroblastoma to emphasize the=20 chondroblastic genesis of the lesion and to distinguish it from = classic=20 giant cell tumor of bone.

Pathophysiology: Various theories have been = proposed=20 concerning the origin of chondroblastomas. Mii et al described the = results=20 of ultrastructural examination of chondroblastomas. These studies = showed=20 subcellular calcium-containing precipitates that are similar to = those seen=20 in chondrocytes. Based on these findings, the authors believe that = the=20 tumors are of chondrogenic origin. Aigner et al, however, noted = the=20 presence of osteoid matrix=96containing type I collagen and the = absence of=20 true cartilage matrix production. They believe that the term=20 chondroblastoma is a misnomer and that the tumor should be = reclassified as=20 a bone-forming neoplasm.=20

Brien et al have compared the characteristics of = chondroblastoma of=20 bone to chondroblastoma of soft tissue, giant cell tumor of the = tendon=20 sheath (GCTTS), and pigmented villonodular synovitis (PVNS). On=20 examination of about 15 examples of GCTTS and PVNS, extensive = areas of=20 chondroid differentiation were noted that could not be = distinguished from=20 chondroblastoma of bone by either histologic or electron = microscopic=20 features. They believe that chondroblastoma of bone stems from an=20 intraosseous proliferation of tendon sheath cells that have a = predilection=20 for chondroid formation. While the exact etiology of = chondroblastoma=20 remains uncertain, the presentation, appropriate evaluation, and = treatment=20 of patients with the tumor have been well described.

Frequency:

  • In the US: Chondroblastoma accounts for=20 approximately 1% of all bone tumors.
  • Internationally: International incidence is = not=20 reported in current literature.

Mortality/Morbidity: Patients with benign=20 chondroblastoma may limit activities due to pain. Malignant=20 chondroblastomas, which may occur many years after the original = lesion=20 even in the absence of radiation, are extremely rare and are = associated=20 with a dismal prognosis.=20

Race: No known race predilection exists.=20

Sex: The male-to-female ratio is 2:1 in most = series.=20

Age: Approximately 92% of patients presenting = with=20 chondroblastoma are younger than 30 years. However, = chondroblastomas have=20 been reported to arise in patients as young as 2 years and as old = as 83=20 years. In several large series, most patients were diagnosed in = the second=20 decade of life.

  INTRODUCTION = Section 2 of = 11    =
Author=20 Information Introduction=20 = Clinical=20 Differentials=20 Wo= rkup=20 Treatment=20 Medication=20 Follow-up=20 Miscellaneous=20 = Pictures=20 Bibliography

History: Pain is = the most=20 common presenting symptom. It typically is mild, gradually = progressive,=20 and initially may be attributed to a minor injury. If the lesion = is=20 juxta-articular, the patient may complain of joint swelling or = diminished=20 range of motion. Usually, constitutional symptoms are lacking. In = their=20 series of 70 patients, Turcotte et al found the average duration = of=20 symptoms in patients with chondroblastoma to be 20 months.

Physical: The physical examination is = remarkable for=20 localized tenderness in most patients. Soft tissue swelling, mass, = or=20 joint effusion is present in about 20% of cases. Muscular atrophy = or=20 decreased joint motion is less common.

Causes: No risk factors are known for = chondroblastoma.=20 There have been reports of abnormalities of chromosomes 5 and 8, = as well=20 as of p53 mutations noted in patients with chondroblastoma.

  CLINICAL = Section 3 of = 11   
Author=20 Information Introduction=20 = Clinical=20 Differentials=20 Wo= rkup=20 Treatment=20 Medication=20 Follow-up=20 Miscellaneous=20 = Pictures=20 Bibliography

Chondromyxoid=20 Fibroma
Chondrosarcoma=20
Giant=20 Cell Tumor


Other Problems to be Considered:

Chondromyxoid fibromas, which are found in patients in the same = age=20 group as chondroblastomas, may mimic the latter radiographically = and=20 microscopically. Both lesions tend to have well-circumscribed = lytic areas=20 on radiographs, areas of immature cartilage and giant cells seen=20 microscopically, and peri-lesional marrow edema on MRI = examination.=20 However, chondromyxoid fibromas most often arise from the = metaphysis or=20 metadiaphysis rather than the epiphysis (where 90% of = chondroblastomas are=20 located), are devoid of calcification, and have a characteristic = myxoid=20 pseudolobular pattern of organization as well as more pleomorphic = stellate=20 cells.

Giant cell tumors of bone also may mimic = chondroblastoma, as=20 the epiphyseal location and histologic characteristics may be = quite=20 similar. However, the former are almost exclusively seen in = patients who=20 are skeletally mature, in contrast to the predilection exhibited = by=20 chondroblastoma for patients who are skeletally immature. = Furthermore, the=20 epicenter of a giant cell tumor lies within the metaphysis. On=20 histological examination, giant cell tumors have elongated cells = that are=20 clustered together, in contrast to the round or polygonal cells of = chondroblastomas.

Calcifications and chondroid matrix also = are=20 absent in giant cell tumors. Eosinophilic granuloma is a lesion = found in=20 young patients that may appear rarely as a radiolucent epiphyseal = lesion=20 similar to chondroblastoma. However, the microscopic examination = reveals a=20 more heterogeneous collection of cells, including histiocytes,=20 granulocytes, and eosinophils. Finally, clear cell chondrosarcoma = may have=20 features that overlap with those of chondroblastoma. The presence = of large=20 cells with abundant clear cytoplasm and vesicular nuclei, as well = as=20 malignant chondrocytes, should distinguish this tumor. Typically, = it is an=20 epiphyseal tumor of adulthood.

  DIFFERENTIALS = Section 4 of = 11   =
Author=20 Information Introduction=20 = Clinical=20 Differentials=20 Wo= rkup=20 Treatment=20 Medication=20 Follow-up=20 Miscellaneous=20 = Pictures=20 Bibliography
Quick Find
Author=20 Information
Introduction
= Clinical
Differentials
Wo= rkup
Treatment
Medication
Follow-up
Miscellaneous
= Pictures
Bibliography

Click=20 for related images.

Related Articles
Chondromyxoid=20 Fibroma

Chondrosarcoma=20

Giant=20 Cell Tumor


Continuing Education =
CME available for this topic. = Click here=20 to take this=20 = CME.

Patient Education
Click here for patient education.=20


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Lab Studies:

  • Laboratory studies are not routinely useful in the workup of = patients with chondroblastoma.

Imaging Studies:

  • Obtain adequate regional radiographs in patients with = suspected=20 chondroblastoma (see Images=20 1-2).
  • A bone scan may be helpful in assessing biological activity = of=20 lesion and in locating other regions of disease involvement that = may=20 occur in entities other than chondroblastoma or in the rare = occurrence=20 of multifocal chondroblastomas (see Image=20 3).
  • Obtain magnetic resonance imaging if any question exists = about the=20 diagnosis or lesion boundaries, including perilesional soft = tissue=20 extension or edema (see Image=20 4).
  • Benign pulmonary metastases occasionally may occur with=20 chondroblastoma. Obtain a chest radiograph in patients with=20 chondroblastoma; computed tomography of the chest may be used to = further=20 evaluate any suspicious areas. These pulmonary lesions are = resectable=20 and curable.

Procedures:

  • If malignancy is not suspected based on clinical and = radiographic=20 features, tissue may be obtained for diagnosis at the time of = surgical=20 treatment of the lesion, and no preceding biopsy is needed. = However,=20 histologic confirmation by frozen section should be sought = before=20 proceeding with definitive treatment. If the lesion appears = atypical on=20 appropriate imaging studies, a needle or incisional biopsy = should be=20 performed before definitive treatment is=20 undertaken.
Histologic Findings:=20 Chondroblastomas are lobulated tumors that consist of=20 grayish-pink soft tissue intermixed with bluish chondroid tissue = and=20 calcifications. The lesions may have many hemorrhagic cystic = areas.=20

The tumors are composed of sheets of neoplastic mononuclear=20 chondroblasts with eosinophilic cytoplasm and grooved nuclei (see = Image=20 5). Interspersed among the mononuclear cells are = osteoclastlike giant=20 cells. The chondroid matrix typically is pink, with the basophilic = matrix=20 seen in hyaline cartilage occurring rarely. Others may have a = spindle-cell=20 component, which represents either spindle-shaped mononuclear = cells or=20 reparative cells of fibroblastic origin. One of the most = characteristic=20 findings in the histologic examination of chondroblastomas is = linear=20 deposition of calcification surrounding individual chondroblasts, = creating=20 a chicken-wire pattern (see Image=20 6). Calcification may be so extensive that the chondrocytes in = the=20 area are not viable. In sections with well-preserved = chondroblasts,=20 mitoses may be seen, but atypical mitoses are not present in = benign=20 chondroblastoma.=20

Cystic changes within chondroblastomas are common. Some = represent=20 secondary aneurysmal bone cysts, which are found in 20-25% of all = patients=20 with chondroblastomas. Other cysts are filled with serous fluid = and are=20 divided into unilocular or multilocular spaces. When these other = cysts are=20 present, the tumors are termed cystic chondroblastomas. Initially, = there=20 was concern that cystic chondroblastomas may have a much higher = recurrence=20 rate than typical chondroblastomas, but later reports have not = confirmed=20 this theory. Less frequently seen histologic findings are cellular = atypia=20 with enlargement and irregularity of chondroblast nuclei = (occurring in 30%=20 of cases); hemosiderin (in 25% of cases); surrounding cortical and = soft=20 tissue permeation (in 5% of cases); myxoid areas (in 2% of cases); = and=20 vascular invasion (in 1% of cases).=20

Immunostaining occasionally can be helpful in confirming the = diagnosis=20 of chondroblastoma. S-100 protein is strongly positive in the = mononuclear=20 cells, although it is absent in multinucleated giant cells and is = present=20 only focally in tumors with a large cystic component. = Chondroblastomas=20 also are positive for vimentin. Reticulin stain reveals a = honeycomb=20 pattern.

  WORKUP = Section 5 of = 11   
Author=20 Information Introduction=20 = Clinical=20 Differentials=20 Wo= rkup=20 Treatment=20 Medication=20 Follow-up=20 Miscellaneous=20 = Pictures=20 Bibliography

Medical Care: = Radiation=20 therapy has been employed in the treatment of chondroblastoma but = has=20 essentially no role in the modern treatment of these tumors. = Chemotherapy=20 has not been reported in the treatment of chondroblastoma.

Surgical Care: No evidence suggests that=20 chondroblastoma resolves spontaneously, so surgical treatment is=20 indicated. The most common surgical procedure to treat = chondroblastomas is=20 thorough curettage, with or without autograft or allograft bone = grafting.=20 Other options reported less frequently include substituting=20 polymethylmethacrylate or fat implantation for bone graft, = treating the=20 curetted lesion with chemical cauterization (phenol), liquid = nitrogen=20 cryotherapy, marginal resection, and wide resection.

Consultations: An orthopedic oncologist should = manage=20 large or recurrent chondroblastomas.

Diet: No dietary restrictions are necessary.

Activity: Unless the lesion is particularly = large and=20 creates a risk of pathologic fracture, patients may participate in = activity as tolerated. If an en bloc excision is performed, the = patient's=20 activity may be limited to protect the reconstruction.

  TREATMENT = Section 6 of = 11   
Author=20 Information Introduction=20 = Clinical=20 Differentials=20 Wo= rkup=20 Treatment=20 Medication=20 Follow-up=20 Miscellaneous=20 = Pictures=20 Bibliography

No medical therapy is available = or=20 reported for chondroblastoma.

  MEDICATION = Section 7 of = 11   
Author=20 Information Introduction=20 = Clinical=20 Differentials=20 Wo= rkup=20 Treatment=20 Medication=20 Follow-up=20 Miscellaneous=20 = Pictures=20 Bibliography

Further Outpatient Care:

In/Out Patient Meds:

Complications:

Prognosis:

Patient Education:

  FOLLOW-UP = Section 8 of = 11   
Author=20 Information Introduction=20 = Clinical=20 Differentials=20 Wo= rkup=20 Treatment=20 Medication=20 Follow-up=20 Miscellaneous=20 = Pictures=20 Bibliography

Medical/Legal Pitfalls:

  MISCELLANEOUS = Section 9 of = 11    =
Author=20 Information Introduction=20 = Clinical=20 Differentials=20 Wo= rkup=20 Treatment=20 Medication=20 Follow-up=20 Miscellaneous=20 = Pictures=20 Bibliography

  PICTURES = Section 10 of=20 11   
Author=20 Information Introduction=20 = Clinical=20 Differentials=20 Wo= rkup=20 Treatment=20 Medication=20 Follow-up=20 Miscellaneous=20 = Pictures=20 Bibliography
Caption: = Picture 1. Chondroblastoma. Radiograph of epiphyseal = lesion=20 (hip).
3D"Click<= /TD> 3D"ClickView Full=20 Size Image
3D"ClickeMedicine=20 Zoom View (Interactive!)
Picture = Type:=20 X-RAY
Caption: = Picture 2. Chondroblastoma. Radiograph demonstrating = tumor on=20 both sides of physis (humerus).
3D"Click<= /TD> 3D"ClickView Full=20 Size Image
3D"ClickeMedicine=20 Zoom View (Interactive!)
Picture = Type:=20 X-RAY
Caption: = Picture 3. Chondroblastoma. Bone scan.
3D"Click<= /TD> 3D"ClickView Full=20 Size Image
3D"ClickeMedicine=20 Zoom View (Interactive!)
Picture = Type:=20 X-RAY
Caption: = Picture 4. MRI of a hip showing lobular pattern of=20 chondroblastoma.
3D"Click<= /TD> 3D"ClickView Full=20 Size Image
3D"ClickeMedicine=20 Zoom View (Interactive!)
Picture = Type:=20 MRI
Caption: = Picture 5. Histology of chondroblastoma.
3D"Click<= /TD> 3D"ClickView Full=20 Size Image
3D"ClickeMedicine=20 Zoom View (Interactive!)
Picture = Type:=20 Photo
Caption: = Picture 6. Chondroblastoma histology demonstrating = chicken-wire=20 calcifications.
3D"Click<= /TD> 3D"ClickView Full=20 Size Image
3D"ClickeMedicine=20 Zoom View (Interactive!)
Picture = Type:=20 Photo

  • Aigner T, Loos S, Inwards C: Chondroblastoma is an = Osteoid-Forming,=20 But Not Cartilage-Forming Neoplasm. Journal of Pathology 1999; = 189:=20 463-469[Medline].=20
  • Brien EW, Mirra JM, Ippolito V: Chondroblastoma Arising from = a=20 Nonepiphyseal Site. Skeletal Radiology 1995; 24: 220-222[Medline].=20
  • Codman, EA: Epiphyseal Chondromatous Giant Cell Tumors of = the Upper=20 End of the Humerus. Surgery, Gynecology and Obstetrics 1931; 52: = 543-548.=20
  • Dahlin DC, Ivins JC: Benign chondroblastoma. A study of 125 = cases.=20 Cancer 1972 Aug; 30(2): 401-13[Medline].=20
  • Dorfman HD, Czerniak B: Cartilage Tumors. In: Bone Tumors . = Mosby:=20 St. Louis; 1998: 317.=20
  • Fanning CV, Sneige NS, Carrasco CH: Fine Needle Aspiration = Cytology=20 of Chondroblastoma of Bone. Cancer 1990; 65(8): 1847-1863[Medline].=20
  • Jaffe HL, Lichtenstein L: Benign Chondroblastoma of Bone: A=20 Reinterpretation of the So-called Calcifying or Chondromatous = Giant Cell=20 Tumor. American Journal of Pathology 1942; 18: 969-991.=20
  • Jambhekar NA, Desai PB, Chitale DA: Benign Metastasizing=20 Chondroblastoma: A Case Report. Cancer 1998; 82(4): 675-678[Medline].=20
  • Jee WH, Park YK, MCCauley TR: Chondroblastoma: MR = Characteristics=20 with Pathologic Correlation. Journal of Computer Assisted = Tomography=20 1999; 23(5): 721-726[Medline].=20
  • Khalili K, White LM, Kandel RA: Chondroblastoma with = Multiple=20 Distant Soft Tissue Metastases. Skeletal Radiology 1997; 26: = 493-496[Medline].=20
  • Kurt AM, Turcotte RE, McLeod RA: Chondroblastoma of Bone.=20 Orthopedics 1990; 13(7): 787-790[Medline].=20
  • Kurt AM, Unni KK, Sim FH: Chondroblastoma of Bone. Human = Pathology=20 1989; 20(10): 965-976[Medline].=20
  • Kyriakos M, Land VJ, Penning HL: Metastatic chondroblastoma. = Report=20 of a fatal case with a review of the literature on atypical, = aggressive,=20 and malignant chondroblastoma. Cancer 1985 Apr 15; 55(8): = 1770-89[Medline].=20
  • Mermelstein LE, Friedlaender GE, Katz LD: Cystic = Chondroblastoma.=20 Orthopedics 1997; 20(1): 69-71[Medline].=20
  • Mii Y, Miyauchi Y, Morishita T: Ultrastructural Cytochemical = Demonstration of Proteoglycans and Calcium in the Extracellular = Matrix=20 of Chondroblastomas. Human Pathology 1990; 25(12): 1290-1294[Medline].=20
  • Mirra JM, Ulich TR, Eckardt JJ: "Aggressive" = chondroblastoma. Light=20 and ultramicroscopic findings after en bloc resection. Clin = Orthop 1983=20 Sep; (178): 276-84[Medline].=20
  • Ostrowski ML, Johnson ME, Truong LD: Malignant = Chondroblastoma=20 Presenting as a Recurrent Pelvic Tumor with DNA Aneuploidy and = p53=20 Mutation as Supportive Evidence of Malignancy. Skeletal = Radiology 1999;=20 28: 644-650[Medline].=20
  • Pflueger P, Heinrich SD, Craver R: Chondroblastoma. = Orthopedics=20 1993; 16(3): 339-342[Medline].=20
  • Ramappa AJ, Lee FYI, Tang P: Chondroblastoma of Bone. The = Journal of=20 Bone and Joint Surgery 2000; 82A(8): 1140-1145[Medline].=20
  • Rogers WB, Mankin HJ: Metastatic Malignant Chondroblastoma. = The=20 American Journal of Orthopedics 1996; 25(12): 846-849[Medline].=20
  • Springfield DS, Capanna R, Gherlinzoni F: Chondroblastoma. A = review=20 of seventy cases. J Bone Joint Surg Am 1985 Jun; 67(5): 748-55[Medline].=20
  • Swarts SJ, Neff JR, Johansson SL: Significance of = Abnormalities of=20 Chromosomes 5 and 8 in Chondroblastoma. Clinical Orthopedics and = Related=20 Research 1998; 349: 189-193[Medline].=20
  • Turcotte RE, Kurt AM, Sim FH: Chondroblastoma. Human = Pathology 1993;=20 24(9): 944-949[Medline].=20
  • Weatherall PT, Maale GE, Mendelsohn DB: Chondroblastoma: = Classic and=20 Confusing Appearance at MR Imaging. Radiology 1994; 190: = 467-474[Medline].=20
  • Yamamura S, Sato K, Sugiura H: Inflammatory Reaction in=20 Chondroblastoma. Skeletal Radiology 1996; 25: 371-376[Medline].

  BIBLIOGRAPHY = Section 11 of=20 11   =
Author=20 Information Introduction=20 = Clinical=20 Differentials=20 Wo= rkup=20 Treatment=20 Medication=20 Follow-up=20 Miscellaneous=20 = Pictures=20 Bibliography
NOTE: =
Medicine is a = constantly=20 changing science and not all therapies are clearly = established. New=20 research changes drug and treatment therapies daily. The = authors,=20 editors, and publisher of this journal have used their best = efforts=20 to provide information that is up-to-date and accurate and = is=20 generally accepted within medical standards at the time of=20 publication. However, as medical science is constantly = changing and=20 human error is always possible, the authors, editors, = and=20 publisher or any other party involved with the publication = of this=20 article do not warrant the information in this article is = accurate=20 or complete, nor are they responsible for omissions or = errors in the=20 article or for the results of using this information. The = reader=20 should confirm the information in this article from other = sources=20 prior to use. In particular, all drug doses, indications, = and=20 contraindications should be confirmed in the package = insert. FULL = DISCLAIMER=20

Cho= ndroblastoma=20 excerpt

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ORTHOPEDIC SURGERY=0A= - Medical Reference
 
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Table of = Contents
Alphabetical Index = of Topics

Biomechanics
Elbow
Foot And Ankle
Hand And Upper = Extremity
Hip
Knee



Neoplasms
Pediatrics
Shoulder
Spine
Systemic Diseases
Trauma
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Dennis P Grogan, MD
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Carlos J Lavernia, MD
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Nancy J Taubenheim, DPT
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Amer Suleman, MD
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Michael S Clarke, MD
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Timothy Damron, MD
James K DeOrio, MD
John S Early, = MD
Dennis P Grogan, MD
William J Hozack, MD
William L Jaffe, = MD
Mordechai (Morton) Kamel, MD
James F Kellam, MD
Mininder S = Kocher, MD, MPH
Jegan Krishnan, MBBS, FRACS, PhD
Cato T Laurencin, = MD, PhD
Mark D Lazarus, MD
M Mike Malek, MD, FACS
Phillip J = Marone, MD
Charles T Mehlman, DO, MPH
Peter M Murray, MD
N Ake = Nystrom, MD, PhD
A Lee Osterman, MD
Albert W Pearsall IV, = MD
Daniel Riew, MD
Lee H Riley III, MD
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Thomas R Hunt III, MD
Shepard R Hurwitz, MD
Pekka A Mooar, = MD
N Ake Nystrom, MD, PhD
Sean P Scully, MD, PhD
William O = Shaffer, MD
George H Thompson, MD
John W Uribe, MD
Jerome D = Wiedel, MD
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