Updated: 6/21/2021

Chondrosarcoma

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https://upload.orthobullets.com/topic/8023/images/Case E - prox femur - xray - Parsons_moved.png
https://upload.orthobullets.com/topic/8023/images/Case E - prox femur - MRI - Parsons_moved.png
https://upload.orthobullets.com/topic/8023/images/Case F - prox humerus - xray - Parsons_moved.jpg
https://upload.orthobullets.com/topic/8023/images/chondrosarcoma.high power.histology.jpg
https://upload.orthobullets.com/topic/8023/images/clear cell 2.jpg
https://upload.orthobullets.com/topic/8023/images/clear cell.jpg
  • summary
    • Chondrosarcomas are malignant primary bone tumors composed of chondrocytes with variable degrees of malignancy that are most commonly found in the pelvis and proximal femur. Patients typically present after the age of 40, with a progressively painful mass.
    • Diagnosis is made with biopsy showing hypercellular stroma consisting of characteristic "blue-balls" of a cartilage lesion which permeate the bone trabeculae.
    • Treatment is usually wide surgical resection.
  • Epidemiology
    • Demographics
      • typically, chondrosarcomas are found in older patients (40-75 yrs)
      • there is a slight male predominance
    • Anatomic location
      • most common locations include the pelvis, proximal femur, distal femur, scapula
      • tumor location is important for diagnosis as the same histology may be diagnosed as benign in the hand but malignant if located in the long bones
  • Etiology
    • Forms
      • primary chondrosarcoma
        • which includes
          • low-grade, high-grade, dedifferentiated chondrosarcoma
          • clear cell chondrosarcoma (see below)
          • mesenchymal chondrosarcoma (see below)
      • secondary chondrosarcoma
        • arises from benign cartilage lesions including
          • osteochondroma (<1% risk of malignant transfomation)
          • multiple hereditary exostosis (1-10% risk of malignant transformation)
          • enchondromas (1% risk of malignant transformation)
          • Ollier's disease (25-30% risk of malignant transformation)
          • Maffucci's (25-30% risk of malignant transformation)
  • Classification
    • Grade
      • 85% of chondrosarcomas are grade 1 or 2
      • 15% of chondrosarcomas are grade 3 or dedifferentiated chondrosarcoma
        • de-differentiated chondrosarcomas are high grade lesions which develop from low grade chondroid lesions
    • Chondrosarcoma sub-types
      • Clear cell chondrosarcoma
        • malignant immature cartilaginous tumor accounting for <2% of all chondrosarcomas
        • most common in 3rd and 4th decades of life
        • commonly presents with insidious onset of pain
        • presents as an epiphyseal lesion and can be mistaken for low-grade chondroblastoma
        • locally destructive with potential to metastasize
      • Mesenchymal chondrosarcoma
        • chondrosarcoma variant which presents with a biphasic pattern of neoplastic cartilage with associated neoplastic small round blue cell component
        • occurs in younger patients than typical chondrosarcomas
        • may occur at several discontinuous sites at presentation and can occur in the soft tissues
        • treatment includes neo-adjuvant chemotherapy followed by wide surgical resection
  • Presentation
    • Symptoms
      • pain is the most common symptom
      • variable rapidity of presentation dependent on tumor grade and location
      • may present with slowly growing mass or symptoms of bowel/bladder obstruction due to mass effect in the pelvis
      • 50% of de-differentiated chondrosarcomas present with a pathologic fracture
  • Imaging
    • Radiographs
      • views
        • AP and lateral views
      • findings
        • lytic or blastic lesion with reactive thickening of the cortex
        • 85% of patients have significant cortical changes
          • low-grade chondrosarcomas show
            • similar appearance to enchondromas with additional cortical thickening/expansion and endosteal erosion
          • high-grade chondrosarcomas show
            • cortical destruction and a soft tissue mass
        • intra-lesional "popcorn" mineralization may be seen
          • described as rings, arcs, and stipples of mineralization, more prevalent as lesions age
        • de-differentiated chondrosarcomas radiographically show a lower grade chondroid lesion with superimposed highly destructive area consistent with the high grade transformed dedifferentiated chondrosarcoma
        • mesenchymal chondrosarcomas appear similar to Ewing sarcoma with permeative bone destruction and periosteal reaction
    • MRI
      • indications
        • MRI helpful to determine marrow and soft-tissue involvement
      • findings
        • typically dark on T1 and bright on T2 in non-mineralized portions due to high water content
        • heterogeneous and moderate contrast uptake, can be most in fibrovascular connective tissue separating cartilage lobules
    • CT
      • indications
        • CT most useful study for low grade cartilage neoplasms to determine cortical involvement
      • findings
        • matrix calcifications more easily identified on CT compared to radiographs
        • deep endosteal scalloping suggests chondrosarcoma instead of enchondroma
        • 90% have cortical breach
    • Bone scan
      • indications
        • can help distinguish (not definitively) chondrosarcoma and enchondroma
        • can determine location of metastastic disease
      • findings
        • usually very hot in all grades of chondrosarcoma
  • Histology
    • Chondrosarcoma
      • needle biopsy is not indicated for cartilage tumors due to difficulties with diagnosis
        • lesions are often heterogenous and difficult to diagnose on histology alone
        • diagnosis requires clinical, pathologic, and radiographic correlation
      • characteristic histology
        • low-grade chondrosarcomas show
          • features similar to enchondroma
          • few mitotic figures with a bland hyaline cartilage appearance
          • hypercellularity and disorganization
          • enlarged chondrocytes with plump multinucleated lacunae
        • high-grade chondrosarcomas show
          • hypercellular stroma consisting of characteristic "blue-balls" of a cartilage lesion which permeate the bone trabeculae
          • benign cartilage appearance and lacunar structure is absent
          • more disorganization, more pleomorphism, and more mitotic figures
      • enchondromas of hand, Ollier's disease, Maffucci's disease, periosteal chondromas, and chondrosarcoma may all have similar histology
    • De-differentiated chondrosarcomas
      • most malignant of cartilage tumors, < 10% long-term survival
      • characterized by a bimorphic histology
        • low grade chondroid component
        • high grade spindle cell component (similar histology to osteosarcoma, fibrosarcoma, MFH)
        • 80% are chondrosarcomas with an extra destructive area
    • Mesenchymal chondrosarcoma
      • biphasic appearance with low grade chondrosarcoma adjacent to small, round blue cells
    • Clear cell chondrosarcoma
      • lobular architecture similar to other cartilage neoplasms, but cells are large and vacuolated
      • typically low to intermediate grade
  • Differential
    • Differential of Chondrosarcoma
      Malignant lesion in older patient
      May have similar chondrogenic histology
      Sacral lesions in older patients
      Treated with wide resection alone 
      Chondrosarcoma
      Metastatic disease
      Lymphoma
      Myeloma
      MFH
      Secondary sarcoma
      Enchondroma / Olliers / Maffuccis
      Periosteal chondroma
      Osteochondroma (MHE)
      Parosteal osteosarcoma
      Adamantinoma
      Chordoma
      Squamous cell
  • Treatment
    • Nonoperative
      • chemotherapy & radiation
        • indications
          • chondrosarcomas are generally resistant to chemotherapy and radiation
    • Operative
      • intra-lesional curettage
        • indications
          • Grade 1 lesions in the extremities (minimal rate of metastasis)
          • treatment of grade 1 lesions located in the pelvis or axial skeleton is controversial (higher rate of local recurrence and metastasis)
            • most authors recommend wide excision of all chondrosarcomas (even grade 1) if located in the pelvis
        • indications
          • grade 2 or 3 lesions
          • grade 1 lesions in pelvis
      • wide surgical excision combined with multi-agent chemotherapy
        • indications
          • mesenchymal chondrosarcoma
          • the role of chemotherapy in de-differentiated chondrosarcoma is very controversial
  • Complications
    • Local recurrence
      • for high-grade lesions, local recurrence associated with distant metastasis and decreased survival
      • grade 1 chondrosarcoma:
        • rare after wide resection with negative margins
        • 5-15% after curettage with adjuvant treatment
      • grade 2 chondrosarcoma:
        • varies depending on resection margins
      • grade 3 chondrosarcoma:
        • 25% local recurrence rate and >30% rate of metastasis
  • Prognosis
    • Histologic grade correlates with survival
      • Grade I
        • 90% 5-year survival
        • grade 1 chondrosarcomas are generally slow growing
      • Grade II
        • 60-70% 5-year survival
      • Grade III
        • 30-50% 5-year survival
      • De-differentiated chondrosarcoma
        • <10% 5-year survival
    • Poor prognostic variables
      • axial and proximal extremity lesions
        • have a more aggressive course
        • pelvic location
      • advanced patient age
      • inadequate surgical margins
    • Increased telomerase activity
      • as determined by reverse transcriptase-polymerase chain reaction (RT-PCR), increased telomerase activity has been shown to directly correlate with the rate of recurrence
    • referral to orthopedic oncologist is necessary for best prognosis

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(OBQ13.34) A 65-year-old male patient presents with pain and swelling in his thigh for 1 year. Radiographs and biopsy findings are seen in Figures A through C. What is the most appropriate treatment?

QID: 4669
FIGURES:
1

Surgery

62%

(3187/5171)

2

Chemotherapy

2%

(93/5171)

3

Radiotherapy

4%

(204/5171)

4

Surgery and chemotherapy

21%

(1076/5171)

5

Surgery, chemotherapy and radiotherapy

11%

(588/5171)

L 3 A

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(OBQ12.142) A 52-year-old Asian male presents with pain in his right hip worse with activity. He is otherwise healthy and denies any history of trauma or constitutional symptoms. Radiographs, CT, and MRI are shown in Figure A, B, and C respectively. A CT guided biopsy is performed and low power and high power histology images are shown in Figure D and E respectively. A CT scan of the chest, abdomen, and pelvis is performed and there is no evidence of metastatic disease. Which of the following is the most appropriate treatment?

QID: 4502
FIGURES:
1

radiation followed by surgical excision followed by chemotherapy

10%

(325/3121)

2

chemotherapy followed by surgical excision followed by chemotherapy

15%

(480/3121)

3

neoadjuvant radiation followed by chemotherapy

3%

(86/3121)

4

wide surgical excision alone

68%

(2107/3121)

5

radical resection and brachytherapy followed by reconstruction

3%

(96/3121)

L 3 A

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(OBQ10.3) A 38-year-old male complains of progressive right groin pain over the past year. Initially, the pain occurred only with weight-bearing, but is now beginning to bother him while sitting and has awoken him from sleep on a few occasions. A radiograph demonstrates a mixed lucent and sclerotic lesion within the femoral head just below the articular surface. An MRI is shown in figure A. A histology slide from an open biopsy is provided in figure B. Which of the following is the most likely diagnosis?

QID: 3091
FIGURES:
1

Lymphoma

4%

(194/4499)

2

Clear cell chondrosarcoma

75%

(3376/4499)

3

Chondroblastoma

16%

(699/4499)

4

Giant cell tumor

4%

(164/4499)

5

Osteosarcoma

1%

(52/4499)

L 2 C

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(OBQ10.136) A 60-year-old female complains of progressive right hip pain. A pelvis radiograph is shown in Figure A and a biopsy specimen is shown in Figure B. What is the most appropriate treatment?

QID: 3187
FIGURES:
1

Neoadjuvant radiation followed by surgical resection

6%

(200/3142)

2

Neoadjuvant chemotherapy followed by surgery followed by adjuvant chemotherapy

12%

(382/3142)

3

Wide surgical resection

74%

(2339/3142)

4

Chemotherapy followed by radiation therapy

4%

(123/3142)

5

Radiation therapy

3%

(83/3142)

L 2 A

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(OBQ09.41) What is the preferred definitive treatment for biopsy proven grade-2 chondrosarcoma of the distal femur without evidence of metastases?

QID: 2854
1

Curettage

1%

(30/3121)

2

Marginal excision

2%

(56/3121)

3

Wide excision

85%

(2668/3121)

4

Chemotherapy then wide excision

10%

(326/3121)

5

Hip disarticulation amputation

1%

(25/3121)

L 1 A

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(OBQ07.273) A 65-year-old man undergoes a biopsy from his pelvis for a suspicious lesion. The biopsy is shown in Figures A and B. What is the preferred treatment for this condition?

QID: 934
FIGURES:
1

Observation

3%

(75/2252)

2

Wide excision

67%

(1518/2252)

3

Wide excision plus chemotherapy

23%

(520/2252)

4

Radiation therapy

5%

(118/2252)

5

Cyrotherapy

0%

(6/2252)

L 2 A

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(OBQ07.203) A 72-year-old male presents with increasing thigh pain. Radiographs and high power biopsy are shown in Figures A and B. Staging studies demonstrate no further sites of disease. What is the next step in treatment?

QID: 864
FIGURES:
1

Neoadjuvant chemotherapy

8%

(199/2461)

2

Neoadjuvant radiation therapy

7%

(173/2461)

3

Marginal excision

2%

(61/2461)

4

Wide surgical excision and reconstruction

80%

(1978/2461)

5

Palliative care

1%

(32/2461)

L 2 A

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(OBQ06.23) Which of the following statements regarding dedifferentiated chondrosarcoma is CORRECT?

QID: 34
1

Survival rates of 10% at 10 years are common

66%

(1453/2198)

2

These tumors arise de novo from an intramedullary location

15%

(329/2198)

3

Histologically these tumors consist of homogeneous intermediate grade chondroid tissue

9%

(188/2198)

4

Treatment involves intra-lesional curettage, cementation, and internal fixation

3%

(66/2198)

5

The most common age of presentation is the young adolescent

7%

(148/2198)

L 3 D

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(OBQ05.219) Patients with which of the following conditions have the highest incidence of malignant tumor?

QID: 1105
1

Single enchondroma

0%

(4/811)

2

Single osteochondroma

1%

(6/811)

3

Multiple hereditary exostosis

12%

(94/811)

4

Ollier's disease

8%

(65/811)

5

Maffucci's syndrome

78%

(634/811)

L 2 C

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(OBQ05.227) A 44-year-old male reports a mass at his right iliac crest that bothers him when he wears a belt. He denies constitutional symptoms and has no bowel function disturbance. His ESR and CRP are normal. His chest CT is normal. Pelvis radiograph, CT, and MRI images are shown in Figures A-D. A biopsy is performed with histology shown in Figure E. What is the next most appropriate step in management?

QID: 1113
FIGURES:
1

Repeat CT scan in 3 months

2%

(43/1736)

2

Neoadjuvant radiation followed by marginal surgical resection followed by adjuvant chemotherapy

6%

(100/1736)

3

Marginal surgical resection

17%

(292/1736)

4

Wide surgical resection

66%

(1145/1736)

5

Neoadjuvant chemotherapy followed by marginal surgical resection followed by adjuvant chemotherapy

8%

(138/1736)

L 3 A

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(OBQ04.185) Which of the following tumors is chemotherapy and radiation therapy resistant?

QID: 1290
1

Ewing's sarcoma

6%

(80/1344)

2

Classic intramedullary chondrosarcoma

69%

(927/1344)

3

Synovial sarcoma

7%

(89/1344)

4

Chordoma

11%

(149/1344)

5

Dermatofibrosarcoma protuberans

7%

(93/1344)

L 2 D

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(OBQ04.166) A 79-year-old healthy male has 1 year of progressively worse left hip pain. He denies any significant weight loss but does complain of night pain. Radiograph and MRI are shown in Figures A & B. Bone scan and histology is shown in Figures C-E. What is the definitive treatment of this?

QID: 1271
FIGURES:
1

observation

2%

(21/1215)

2

radiation therapy alone

3%

(35/1215)

3

wide resection and reconstruction

57%

(687/1215)

4

wide resection, reconstruction and chemotherapy

21%

(260/1215)

5

wide resection, reconstruction and local radiation therapy.

17%

(203/1215)

L 3 A

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