Bone & joints

Chondrosarcoma

Clear cell chondrosarcoma



Last author update: 21 July 2022
Last staff update: 21 July 2022

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PubMed Search: Clear cell chondrosarcoma

Borislav A. Alexiev, M.D.
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Cite this page: Alexiev BA. Clear cell chondrosarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/boneclearcellchondrosarcoma.html. Accessed December 3rd, 2022.
Definition / general
  • Clear cell chondrosarcoma is a low grade malignant cartilaginous epiphyseal neoplasm characterized by lobules of cells with abundant clear cytoplasm
Essential features
  • Epiphyseal location
  • Clear cells with abundant cytoplasm and centrally placed nucleus
  • Presence of woven bone and osteoclast-like giant cells
ICD coding
  • ICD-O: 9242/3 - clear cell chondrosarcoma
  • ICD-11: 2B50.Z & XH7XB9 - chondrosarcoma of bone and articular cartilage of unspecified sites & clear cell chondrosarcoma
Epidemiology
Sites
  • Clear cell chondrosarcoma has a predilection for the epiphyses of long tubular bones (Pathologe 2000;21:449)
  • Proximal femur is the most frequent site of involvement (68%), followed by the proximal humerus (23%) (Skeletal Radiol 2003;32:687)
Etiology
  • Unknown
Diagrams / tables

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Gender and anatomical distribution

Clinical features
Diagnosis
  • Correlation of radiological and clinicopathological features is mandatory in the diagnosis of all bone tumors, including clear cell chondrosarcoma
Radiology description
  • Typical radiolographic manifestation of this tumor is a slow growing epiphyseal or epimetaphyseal osteolytic lesion with a sclerotic border (J Bone Oncol 2019;19:100267, Skeletal Radiol 2003;32:687)
  • Subtle cartilaginous calcifications are seen in > 50% of cases and a periosteal reaction may also be present
  • Lesions in the axial skeleton are typically expansile and destructive, often with soft tissue extension and a lack of mineralization (Skeletal Radiol 2003;32:687)
  • MR imaging is superior to conventional radiographs for demonstrating the intramedullary extent of a lesion as well as soft tissue extension (Skeletal Radiol 2003;32:687)
  • CT images better delineate the presence of cortical destruction and the character of matrix mineralization patterns (Skeletal Radiol 2003;32:687)
  • On T1 weighted sequences, the tumor is typically of low signal intensity (Skeletal Radiol 2002;31:88, Skeletal Radiol 2003;32:687)
  • On T2 weighted sequences, there is a heterogeneous pattern with overall low to intermediate signal intensity (Skeletal Radiol 2002;31:88)
  • Clear cell chondrosarcoma and chondroblastoma may have a very similar radiographic appearance, which prevents a reliable differentiation of the 2 tumors (Skeletal Radiol 2002;31:88)
Radiology images

Contributed by Borislav A. Alexiev, M.D.
Conventional radiography

Conventional radiography

T1 weighted MRI

T1 weighted MRI

MRI STIR sequence

MRI STIR sequence

T2 weighted MRI

T2 weighted MRI

Prognostic factors
  • Prognosis of clear cell chondrosarcoma is excellent when treated adequately with wide surgical resection, with 10 year disease survival approaching 90% (Radiol Case Rep 2015;8:848)
  • Clear cell chondrosarcoma has a tendency for very late recurrence and metastasis 20 years after initial diagnosis
  • Metastases in lungs and other skeletal sites develop in 15 - 20% of cases
  • Bone metastases are as common as pulmonary metastases (Clin Orthop Relat Res 2020;478:2537)
  • Dedifferentiation to high grade sarcoma has been reported (Am J Surg Pathol 2000;24:1079)
Case reports
Treatment
Gross description
Gross images

Contributed by Borislav A. Alexiev, M.D.
Femoral head mass

Femoral head mass

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Borislav A. Alexiev, M.D.
Malignant bone neoplasm

Malignant bone neoplasm

Cells with pale cytoplasm

Cells with pale cytoplasm

Woven bone formation

Woven bone formation

Large round nuclei

Large round nuclei

Cartilaginous component

Cartilaginous component


S100

S100

D2-40

D2-40

AE1 / AE3

AE1 / AE3

H3K36M

H3K36M

Cytology description
  • Low to intermediate cellular smears of clusters and single round or oval tumor cells (Diagn Cytopathol 2021;49:46)
  • Tumor cells with rounded nuclei (sometimes binucleated) and rich vacuolated cytoplasm
  • Low grade cellular atypia
  • Chondroid background matrix
  • Occasional osteoclast-like giant cells in background
Negative stains
Molecular / cytogenetics description
  • Rb pathway is affected in 95% of clear cell chondrosarcomas (Genes Chromosomes Cancer 2012;51:899)
  • 1 of 15 clear cell chondrosarcomas investigated for histone 3.3 mutations (to date) has shown K36M mutations in H3F3B, a highly specific driver mutation for chondroblastoma, suggesting a pathogenetic relation in at least a small subset of tumors (Nat Genet 2013;45:1479)
  • Neither IDH1 nor IDH2 mutations are detected
Sample pathology report
  • Right femoral head, resection:
    • Clear cell chondrosarcoma, low grade (see comment)
    • Margins of resection are negative, with sarcoma closest at 2 cm from the lateral soft tissue margin
    • Comment: The tumor is composed of cells with abundant clear or slightly eosinophilic cytoplasm and large round nuclei with mild pleomorphism and small nucleoli. The cells are arranged in sheets and lobules, admixed with trabeculae of woven bone and scattered osteoclast-like giant cells. Mitotic figures are rare (1/10 high power fields). Areas with hyaline cartilaginous matrix with moderate cellularity and mild nuclear atypia are also present. There is entrapment of pre-existing lamellar bone and invasion of the subchondral bone plate. Immunohistochemically, the tumor cells are strongly positive for S100 and D2-40 and show focal immunoreactivity for keratin AE1 / AE3. Stains for H3K36M and PAX8 are negative.
    • This constellation of morphological and immunohistochemical features strongly supports the diagnosis of clear cell chondrosarcoma. The prognosis of clear cell chondrosarcoma is excellent when treated adequately with wide surgical resection. However, clear cell chondrosarcoma has a tendency for very late recurrence and metastasis 20 years after initial diagnosis.
Differential diagnosis
Board review style question #1

The following tumor is found in the femoral head epiphysis of a 39 year old man. Which is most likely the correct diagnosis?

  1. Chondroblastoma
  2. Chordoma
  3. Clear cell chondrosarcoma
  4. Conventional osteosarcoma
  5. Giant cell tumor of bone
Board review style answer #1
C. Clear cell chondrosarcoma

Comment Here

Reference: Clear cell chondrosarcoma
Board review style question #2
Which of the following is true about clear cell chondrosarcoma?

  1. Clear cell chondrosarcoma has poor prognosis
  2. Clear cell chondrosarcoma is sensitive to radiation and chemotherapy
  3. Late recurrences and even distant metastases may occur
  4. Most clear cell chondrosarcomas have IDH1 or IDH2 mutations
  5. Most often affects the metaphysis of long bones
Board review style answer #2
C. Late recurrences and even distant metastases may occur

Comment Here

Reference: Clear cell chondrosarcoma
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