Enchondromas

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Topic updated on 04/22/13 11:36am
  
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 location is the hand (60%)
          • the most common bone tumor in the hand is the enchondroma
        • other locations include the distal femur (20%), proximal humerus (10%) and tibia
        • rare in the spine and pelvis
  • 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 (multipe 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" stippled calcification and rings 
      • minimal endosteal erosion (<50% width of cortex)
      • cortical expansion and thinning may be present (especially in the hand)  
      • 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
    • findings  
      • increased uptake, but less than chondrosarcoma 
        • because of continued remodeling within the lesion
  • MRI 
    • indications
      • usually not necesary 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 beedle-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  
  • Low grade chondrosarcoma
    • 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 for interval growth (every 3-6 months for 1-2 years, then annually)
        • 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) 
      • technique
        • immobilize until fracture union, followed by currettage and grafting
Complications
  • Malignant transformation
    • risk of transformation of enchondroma to low-grade chondrosarcoma
      • solitary enchondroma 
        • risk of transformation is 1%
      • Ollier's disease (multiple enchondromatosis) 
        • risk of transformation is 25-30%
      • Maffucci's syndrome 
        • risk of transformation is 23-100%
        • also has high risk of fatal visceral malignancy
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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Qbank (4 Questions)

TAG
(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? Topic Review Topic
FIGURES: A   B   C   D    

1. Observation
2. Curretage and bone grafting
3. Thumb amputation
4. Systemic chemotherapy
5. Radiation and intralesional excision

PREFERRED RESPONSE ▶
TAG
(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 A and B respectively. A radiograph of his left knee is shown in Figure C. What is the most likely cause his leg pain? Topic Review Topic
FIGURES: A   B   C   D    

1. Lumbar Disc Herniation
2. Enchondroma
3. Spinal stenosis
4. Vascular claudication
5. Chondrosarcoma

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

1. Resection and internal fixation
2. Chemotherapy followed by wide resection
3. Observation
4. Radiation therapy
5. Palliative care

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

1. Fibrous dysplasia
2. Maffucci syndrome
3. Ollier’s disease
4. McCune-Albright syndrome
5. Multiple hereditary exostoses

PREFERRED RESPONSE ▶



Cases

http://upload.orthobullets.com/cases/1320/enc sad1.jpg http://upload.orthobullets.com/cases/1320/enc sadq.jpg http://upload.orthobullets.com/cases/1320/enc post 1.jpg
HPI - increasing pain and stiffness of the right index finger since over 6 months
poll curretage and bone grafting
11/11/2012
105 responses
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This video demonstrates the histolopathology of Enchondroma / Ollier's Disease...
3/9/2013
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