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Chondrosarcoma

Topic updated on 03/08/13 9:49pm

Introduction
  • Malignant chondrogenic lesions can occur in two forms 
    • primary chondrosarcoma
      • which includes
        • low-grade, high-grade, de-differenitated 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 (25-40% risk of malignant transformation)
        • Maffucci's (100% risk of malignant transformation)
  • Age & location
    • typically, chondrosarcomas are found in older patients (40-75 yrs)
    • there is a slight male predominance
    • most common locations include the pelvis, proximal 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
  • 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
  • Prognosis
    • axial and proximal extremity lesions have a more aggressive course
    • histologic grade correlates with survival
      • Grade I: 90% survival
      • Grade II: 60-70% survival
      • Grade III: 30-50% survival
      • De-differentiated chondrosarcoma: 10% survival
    • increased telomerase activity in chondrosarcoma, as determined by reverse transcriptase-polymerase chain reaction (RT-PCR), has been shown to directly correlate with the rate of recurrence  
  • 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
    • 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
    • lytic or blastic lesion with reactive thickening of the cortex 
      • 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
    • de-differentiated chondrosarcomas radiographically show a lower grade chondroid lesion with superimposed highly destructive area consistent with the high grade transformed dedifferentiated chondrosarcoma  
  • MRI or CT
    • helpful to determine cortical destruction, marrow involvement, and the soft tissue involvement 
  • Bone scan
    • is usually very hot in all grades of chondrosarcoma
Histology
  • Chondrosarcoma
    • needle biopsy is not indicated for cartilage tumors due to difficulties with diagnosis
      • it is often difficult to determine malignancy based on histology alone
    • characteristic histology
      • low-grade chondrosarcomas show
        • few mitotic figures with a bland histologic appearance
        • 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 
    • enchondromas of hand, Ollier's disease, Maffucci's disease, periosteal chondromas, and chondrosarcoma may all have similar histology
  • De-differentiated chondrosarcomas
    • characterized by a bimorphic histology
      • low grade chondroid component
      • high grade spindle cell component (similar histology to osteosarcoma, fibrosarcoma, MFH)  
Treatment
  • Operative
    • intra-lesional curettage 
      • indications
        • Grade 1 lesions 
        • treatment of grade 1 lesions located in the pelvis or axial skeleton is controversial
          • many authors recommend wide excision of all chondrosarcomas (even grade 1) if located in the pelvis
    • wide surgical excision      
      • indications
        • grade 2 or 3 lesions
        • some say grade 1 lesions in pelvis
      • historically, there is no significant role for radiation or chemotherapy in typical intramedullary chondrosarcoma 
    • wide surgical excision combined with multi-agent chemotherapy 
      • indications
        • mesenchymal chondrosarcoma
        • the role of chemotherapy in de-differentiated chondrosarcoma is very controversial
Differentials & Groups
 
Malignant lesion in older patient(1)
 
May have similar chondrogenic histology
 
Sacral lesions in older patients
 
Treated with wide resection alone (2)
Chondrosarcoma
 
 
 
Metastic disease
     
   
Lymphoma
     
   
Myeloma
     
   
MFH
     
   
Secondary sarcoma
           
Enchondroma / Olliers / Marfuccis    
       
Periosteal chondroma    
       
Osteochondroma (MHE)    
       
Parosteal osteosarcoma            
Adamantinoma            
Chordoma        
 
Squamous cell(3)        
 
ASSUMPTIONS: (1) Older patient is > 40 yrs; (2) assuming no impending fracture (3) assuming no squamous cell metastatic disease
 
IBank
 
Location
Xray
Xray
CT
B. Scan
MRI
MRI
Histo(1)
Case A scapula
 
 
 
Case B femur
 
 
 
Case C pelvis
 
 
Case D tibia
 
 
 
Case E prox femur
 
 
 
Case F prox. humerus
 
 
 
Case H pelvis (dedifferentiated)
 
 
 
Case I femur (dedifferentiated)
 
 
 

(1) - histology does not always correspond to case

 


 

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Qbank (11 Questions)

TAG
(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? Topic Review Topic
FIGURES: A   B        

1. Lymphoma
2. Clear cell chondrosarcoma
3. Chondroblastoma
4. Giant cell tumor
5. Osteosarcoma

PREFERRED RESPONSE ▶
TAG
(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? Topic Review Topic
FIGURES: A   B        

1. Neoadjuvant radiation followed by surgical resection
2. Neoadjuvant chemotherapy followed by surgery followed by adjuvant chemotherapy
3. Wide surgical resection
4. Chemotherapy followed by radiation therapy
5. Radiation therapy

PREFERRED RESPONSE ▶
TAG
(OBQ09.41) What is the preferred definitive treatment for biopsy proven grade 2 chondrosarcoma of the distal femur without evidence of metastases? Topic Review Topic

1. Curettage
2. Marginal excision
3. Wide excision
4. Chemotherapy then wide excision
5. Hip disarticulation amputation

PREFERRED RESPONSE ▶
TAG
(OBQ07.89) Rates of recurrence for patients with chondrosarcoma are most directly related to which of the following? Topic Review Topic

1. Increased telomerase activity
2. Expression of major histocompatibility complex type II
3. Gadolinium uptake on MRI
4. Increased tumor apoptosis on histology
5. Technetium activity on bone scan

PREFERRED RESPONSE ▶
TAG
(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? Topic Review Topic
FIGURES: A   B        

1. Neoadjuvant chemotherapy
2. Neoadjuvant radiation therapy
3. Marginal excision
4. Wide surgical excision and reconstruction
5. Palliative care

PREFERRED RESPONSE ▶
TAG
(OBQ07.273) A 65-year-old man undergoes a biopsy from his pelvis for a suspicious lesion. The biopsy is shown in Figure A. What is the preferred treatment for this condition? Topic Review Topic
FIGURES: A          

1. Observation
2. Wide excision
3. Wide excision plus chemotherapy
4. Radiation therapy
5. Cyrotherapy

PREFERRED RESPONSE ▶
TAG
(OBQ06.23) Which of the following statements regarding dedifferentiated chondrosarcoma is CORRECT? Topic Review Topic

1. Survival rates of 10% at 10 years are common
2. These tumors arise de novo from an intramedullary location
3. Histologically these tumors consist of homogeneous intermediate grade chondroid tissue
4. Treatment involves intra-lesional curettage, cementation, and internal fixation
5. The most common age of presentation is the young adolescent

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TAG
(OBQ05.219) Patients with which of the following conditions have the highest risk of malignant transformation? Topic Review Topic

1. Single enchondroma
2. Single osteochondroma
3. Multiple hereditary exostosis
4. Ollier's disease
5. Maffucci's syndrome

PREFERRED RESPONSE ▶
TAG
(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? Topic Review Topic
FIGURES: A   B   C   D   E  

1. Repeat CT scan in 3 months
2. Neoadjuvant radiation followed by marginal surgical resection followed by adjuvant chemotherapy
3. Marginal surgical resection
4. Wide surgical resection
5. Neoadjuvant chemotherapy followed by marginal surgical resection followed by adjuvant chemotherapy

PREFERRED RESPONSE ▶
TAG
(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? Topic Review Topic
FIGURES: A   B   C   D   E  

1. observation
2. radiation therapy alone
3. wide resection and reconstruction
4. wide resection, reconstruction and chemotherapy
5. wide resection, reconstruction and local radiation therapy.

PREFERRED RESPONSE ▶
TAG
(OBQ04.185) Which of the following tumors is chemotherapy and radiation therapy resistant? Topic Review Topic

1. Ewing's sarcoma
2. Classic intramedullary chondrosarcoma
3. Synovial sarcoma
4. Chordoma
5. Dermatofibrosarcoma protuberans

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Donati D, El Ghoneimy A, Bertoni F, Di Bella C, Mercuri M
J Bone Joint Surg Br. 2005 Nov;87(11):1527-30. PMID: 16260673 (Link to Pubmed)
5/17/2013
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