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Introduction
  • A benign chondrogenic tumor composed of hyaline cartilage
    • located in the medullary cavity 
    • caused by an abnormality of chondroblast function in the physis
  • Epidemiology
    • incidence
      • 2nd most common benign cartilage lesion (osteochondroma is most common)
    • demographics
      • male:female ratio is 1:1
      • age bracket
        • most common in 20-50 year olds
      • location
        • usually found in the medullary cavity of the diaphysis or metaphysis
        • the most common locations hand (60%) > feet
          • the most common bone tumor in the hand is the enchondroma
        • other locations include distal femur (20%) > proximal humerus (10%) > tibia
        • rare in the pelvis, scapula, ribs
          • suspect chondrosarcoma in these locations
  • Pathophysiology
    • enchondromas represent incomplete endochondral ossification
      • chondroblasts and fragments of epiphyseal cartilage escape from the physis, displace into the metaphysis and proliferate there 
  • Associated conditions
    • solitary enchondroma
    • Ollier's disease (multiple enchondromatosis)  
      • sporadic inheritance with no genetic predisposition
      • skeletal dysplasia with failure of normal endochondral ossification 
      • enchondromas throughout the metaphyses and diaphyses of long bones
        • involved bones are dysplastic, with shortening and bowing
      • risk of malignant transformation <30%
    • Maffucci's syndrome 
      • sporadic inheritance with no genetic predisposition 
      • multiple enchondromas and soft-tissue angiomas   
      • radiographically, enchondromas in Maffucci's syndrome markedly expand the bone and angiomas are seen as small, round calcified phleboliths
      • risk of malignant transformation up to 100% 
      • also has increased risk of visceral malignancies (astrocytoma, GI malignancy)
Classification
 
Enneking Classification of Benign Lesions
Stage Grade Examples
Images 
Stage 1 Latent lesions enchondroma, non-ossifying fibroma  
Stage 2 Active lesions ABC, UBC, chondromyxoid fibroma, chondroblastoma    
Stage 3 Aggressive lesions giant cell tumor of bone  
 
Presentation
  • Symptoms
    • asymptomatic, discovered incidentally on radiographs 
      • usually true for enchondromas in long bones and foot
    • pathologic fracture
      • often seen with enchondromas in the hand  
    • pain
      • pain is uncommon
      • when a patient presents with an enchondroma and pain in the adjacent joint, the cause of pain is often unrelated to the tumor
      • unlike enchondroma, most chondrosarcomas have non-mechanical pain (rest pain and nocturnal pain)
  • Physical exam
    • shortening and angular deformities    
      • enchondromas may disrupt the growth plate
    • multiple bluish angiomas in Maffucci's syndrome  
Imaging
  • Radiographs
    • recommended views
      • skeletal survey if polyostotic disease is suspected
    • findings
      • well defined, lucent, central medullary lesions that calcify over time 
      • 1 to 10cm in size
        • metaphyseal location when they first appear
        • appear more diaphyseal as the long bone grows
      • "pop-corn" stippling, arcs, whorls, rings 
      • minimal endosteal erosion (<50% width of cortex)
      • cortical expansion and thinning may be present in hands, feet  
        • but not in long tubular bones (femur, tibia)
      • may have purely lytic appearance (especially in hand) 
      • Ollier's disease
        • enchondromas markedly expand the bone
        • bones are dysplastic, with shortening and bowing  
      • Maffucci's syndrome  
        • enchondromas markedly expand the bone  
        • angiomas are visible as calcified phleboliths  
      • unlike enchondromas, chondrosarcomas display
        • cortical thickening and destruction
        • endosteal erosions and scalloping >50% of the width of the cortex
        • are larger (>5cm)
  • Bone scan 
    • indications
      • to help differentiate chondrosarcoma from enchondroma
      • to identify polyostotic disease
      • rarely adds useful information
        • only 20% have more uptake than ASIS
        • most enchondromas are small, and easily identified as benign
    • findings  
      • increased uptake, but less than chondrosarcoma 
        • because of continued remodeling within the lesion
  • MRI 
    • indications
      • usually not necessary for diagnosis
      • identify size and intramedullary extent and soft tissue extension
      • differentiate from chondrosarcoma
    • findings  
      • lobular and bright on T2-weighted images  
      • no bone marrow edema or periosteal reaction
      • may show steak of cartilage or "sled runner tracks" 
      • medullary fill >90% suggests chondrosarcoma instead
Studies
  • Core needle-biopsy
    • from areas of bone scalloping or lysis
    • prone to sampling error due to tumor heterogeneity
      • chondrosarcomas may contain areas of benign hyaline cartilage
    • often impossible to differentiate from low-grade chondrosarcoma
  • Histology
    • gross appearance
      • blue gray, lobulated hyaline cartilage, with scattered calcifications
    • microscopic
      • solitary lesions in long bones
        • hypocellular with bland, mature hyaline cartilage (blue balls of cartilage) separated by normal marrow     
          • differentiates from chondrosarcoma
        • endochondral ossification encases cartilage with lamellar bone
        • abundant extracellular matrix with no myxoid component
      • solitary lesions in small tubular bones and fibula, Ollier's and Maffucci's syndromes
        • hypercellular, with mild chondrocytic atypia
      • characteristics of chondrocytes
        • small, bland chondroid cells in lacunar spaces 
        • uniform staining nuclei
        • no pleomorphism, mitoses, anaplasia, hyperchromasia or multinucleate cells
    • unlike enchondromas, chondrosarcomas display
      • hypercellularity, with plump nuclei
      • multiple binucleate cells
      • giant cells with clumps of chromatin
Differential
  • Bone infarct
    • "smoke up the chimney" radiographic appearance  
    • MRI does not give high T2 signal
      • enchondromas have high T2 signal because of high water content of cartilage
  • Chondrosarcoma
    • worsening pain
    • large size
    • deep endosteal scalloping >2/3 of cortical thickness
    • periosteal reaction, cortical breakthrough
    • rare in hands, feet, more common in pelvis, scapula, ribs
      • the converse is true for enchondroma
    • radiographic appearance differentiating low grade chondrosarcoma from enchondroma (see below)
Treatment
  • Nonoperative
    • observation
      • indications
        • treatment for vast majority of asymptomatic enchondromas  
      • follow up
        • serial radiographs at 6 months and 12 months to confirm radiographic stability
        • long term follow-up for patients with multiple enchondroma syndromes
  • Operative
    • intralesional curettage and bone grafting  
      • indications
        • lesion that shows any change on serial xrays
        • radiographs suspicious for low-grade chondrosarcoma
        • large lesions at risk for recurrent fracture
      • outcomes
        • local recurrence is unusual
    • immobilization, followed by currettage and bone grafting
      • indications
        • pathologic fracture in small tubular bones (hand lesions) because repeated fractures will usually occur
      • technique
        • immobilize until fracture union, followed by currettage and grafting
Complications
  • Malignant transformation
    • risk of transformation of enchondroma to chondrosarcoma
      • solitary enchondroma 
        • risk of malignant transformation is 1%
      • Ollier's disease                    
        • risk of malignant transformation is 25-30%
      • Maffucci's syndrome 
        • risk of malignant transformation is 25-30%, but up to 100% risk of other visceral and CNS malignancies as well
Image Bank
Case
Location
Xray
CT
Bone scan
MRI
MRI
Histo(1)
Case A prox. humerus
 
 
   
Case B prox. humerus
 
 
 
Case C prox. fibula
 
 
 
Case D prox. humerus
 
 
 
Case E hand
 
 
 
Case F distal femur
 
 
 
Case G prox. humerus
 
 
 
Case H Olliers - hand
 
 
 
Case I Maffucci - hand
 
 
 
Case J Olliers - humerus
 
 
 
Case K Olliers - forearm          
(1) - histology does not always correspond to case


 

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(OBQ11.145) A 30-year-old male presents with thumb swelling and pain of 6 months duration. He denies a history of trauma or previous problems with the thumb. Radiographs, MRI, and histology slide are shown in Figures A through D. What is the most appropriate treatment for this patient? Review Topic

QID: 3568
FIGURES:
1

Observation

4%

(46/1229)

2

Curettage and bone grafting

65%

(799/1229)

3

Thumb amputation

24%

(296/1229)

4

Systemic chemotherapy

0%

(4/1229)

5

Radiation and intralesional excision

7%

(80/1229)

Select Answer to see Preferred Response

PREFERRED RESPONSE 2
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(OBQ05.154) Which of the following diseases carries greater than 50% risk of developing a malignancy? Review Topic

QID: 1040
1

Fibrous dysplasia

0%

(6/1257)

2

Maffucci syndrome

82%

(1031/1257)

3

Ollier’s disease

9%

(112/1257)

4

McCune-Albright syndrome

4%

(54/1257)

5

Multiple hereditary exostoses

4%

(48/1257)

Select Answer to see Preferred Response

PREFERRED RESPONSE 2

(OBQ12.101) A 56-year-old male with a history of prostate cancer presents to your clinic complaining of bilateral foot pain (right greater than left) that he relates to his chronic bunion and claw toe deformities. He denies new trauma, fevers, or weight loss. He says that he is able to ambulate but has had progressively increased pain in the great and lesser toes of both feet, with progressive difficulty in wearing shoes. During his radiographic evaluation a lesion is found in the right tibia which is shown in Figure A. What is the next most appropriate step in management of the tibial lesion? Review Topic

QID: 4461
FIGURES:
1

Core needle biopsy of the right tibia

2%

(64/3189)

2

Excisional biopsy of the right tibia

1%

(30/3189)

3

MRI of the right tibia

14%

(434/3189)

4

Reassurance and repeat radiographs in 3-6 months

74%

(2349/3189)

5

Staging with CT of the chest, abdomen, and pelvis, and referral to an orthopaedic oncologist

9%

(294/3189)

Select Answer to see Preferred Response

PREFERRED RESPONSE 4

(OBQ09.263) A 70-year-old woman falls and presents with pelvic pain. Physical exam shows she is able to walk without difficulty. Radiographs of the pelvis and hip are shown in Figure A, B, and C. A biopsy of the lesion in the proximal femur is shown in Figure D. What is the next best step in management of the femoral lesion? Review Topic

QID: 3076
FIGURES:
1

Resection and internal fixation

15%

(340/2293)

2

Chemotherapy followed by wide resection

5%

(106/2293)

3

Observation

78%

(1798/2293)

4

Radiation therapy

2%

(37/2293)

5

Palliative care

0%

(6/2293)

Select Answer to see Preferred Response

PREFERRED RESPONSE 3

(OBQ09.92) A 55-year-old male with a history of diabetes mellitus presents with left leg pain localized to his posterior knee and calf. The pain is worse with prolonged walking and resolves when he rests and remains standing upright. He has a history of chronic low back pain, prior cardiac stenting, and smokes 1 pack of cigarettes daily for the last twenty years. Physical exam is remarkable for 1+ patellar reflexes and an ABI of 0.8. A radiograph of his lumbar spine is shown in Figure A. T2-weighted sagittal and axial MRI images are shown in Figure B and C respectively. A radiograph of his left knee is shown in Figure D. What is the most likely cause his leg pain? Review Topic

QID: 2905
FIGURES:
1

Lumbar Disc Herniation

4%

(46/1287)

2

Enchondroma

4%

(57/1287)

3

Spinal stenosis

7%

(90/1287)

4

Vascular claudication

82%

(1049/1287)

5

Chondrosarcoma

3%

(43/1287)

Select Answer to see Preferred Response

PREFERRED RESPONSE 4
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