Dr James Wittig, MD Orthopedic Oncologist
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Bone Tumors
Osteochondroma

James C. Wittig, MD
Sarcoma Surgeon
Orthopedic Oncologist


GENERAL INFORMATION

Osteochondroma is an outgrowth of medullary and cortical bone
A portion of the cartilaginous growth plate grows outward instead of longitudinally and forms the osteochondroma/exostosis (like a branch on a tree)
It consists of bone covered with cartilaginous cap (exostosis)
May be secondary to a growth plate injury (Node of Ranvier injured)
Osteochondromas are benign, non-neoplastic conditions
Hamartomatous anomaly
It can occur as a solitary lesion or as multiple exostoses associated with a hereditary condition known as Multiple Hereditary Exostoses (MHE)
Radiation exposure can also be a cause of multiple osteochondromas
Solitary Osteochondromas are the most common benign bone tumors and constitute 35% of all benign bone tumors and 10% of all bone tumors overall

There are 2 forms
           Pedunculated (with a stalk)
           Sessile (flat without a stalk)

Osteochondromas likely arise from displaced cartilage through periosteal defect and grow at right angles to normal growth plate
Lesions have self-limited growth that ceases after skeletal maturity
Due to endochondral ossification, cartilage cap diminishes in thickness as age increases

Osteochondroma -(most common benign neoplasm of bone that leads to biopsy)

Types:
          Solitary Osetocartilaginous Exostosis
          Hereditary Multiple Exostoses (HME)

Radiographic Subtypes:
         Pedunculated
         Sessile



Multiple Heredity Exostoses (MHE)
Clinical Data:

          Male predominance (3:1)
          Autosomal Dominant inheritance
          There is variability in size and number of ostechondromas (variable penetrance)
          Any portion of the skeleton preformed in cartilage may be involved
          Evident during childhood
          MHE may be bilaterally symmetric
          One side may predominate
          There is a higher incidence of malignant transformation (10-20%) of osteochondromas that
          develop in MHE. Most commonly a secondary low grade chondrosarcoma develops.


CLINICAL PRESENTATION

Signs/Symptoms: Hard swelling for many years
           Symptoms dependent on location/size
           May cause mechanical symptoms from compression of adjacent structures such as tendons,
           nerve or blood vessels
          An overlying bursa may form and result in a bursitis
          Rare vascular injuries and arterial aneurysms from adjacent osteochondromas
          Malignant Transformation: Solitary osteochondroma <1%

Prevalence:
Male>Female 1.8:1

Age:
Usually presents clinically by the third decade of life

Sites:
         Appendicular skeleton: Femur (30%) Tibia (20%) Humerus (2-%) Hand and Foot (10%) 
         Pelvis (5%) Scapula (4%)
         Surface of metaphyseal portions of long tubular bones 
         Knee area 35% of cases


RADIOGRAPHIC PRESENTATION

Plain X-rays:
Projects from bone with narrow (pedunculated) to broad (sessile) stalk
Corticomedullary continuity: Medullary bone continuous with that of osteochondroma and cortex blends with that of osteochondroma
Calcification in cartilaginous cap ("Ring and Arc" and stippled calcifications)
Lobular growth pattern
Long bones:  arise from metaphysis, grows away from epiphysis toward diaphysis,
May be associated with failure of tubulation in Multiple Hereditary Exostosis
Flat bones: tend to be larger and sessile, variable appearance
Cartilage cap thickness is visualized best on MRI, not XR
Bursa may exist external to cartilage cap (seen on MRI)


Plain X-Ray:  Osteochondroma of Proximal Tibia


Proximal Fibula Osteochondroma: Cortical-Medullary Continuity; Ring and Arc Calcifications
(Top arrow)  Calcifications in Cap
(Bottom arrow)  Cortical-Medullary Continuity


MRI of Proximal Fibula Osteochondroma Demonstrates Continuity of Medullary Canal of Fibula with Osteochondroma

 


MRI Demonstrates Stalk and Continuity with Underlying Fibula; Thin Cartilaginous Cap

 



Intraoperative Photograph: Lobular Growth

 



Specimen


Specimen



(arrow) The Cartilage Cap

Plain Xray/MRI: Distal Femur Sessile Osteochondroma

 Xay/MRI: Distal Femur Osteochondroma

 


Gross Specimen Distal Femur Osteochondroma



Osteochondroma of Proximal Femur
(Even though this was a large tumor, the cartilage cap was very thin)



X-ray: Left Proximal Femur Osteochondroma


MRI: Left proximal Femur Osteochondroma
Cortical Medullary Continuity and Thin Cartilage Cap



Area between arrows: cartilage cap (approximately 1 cm thickness)


CT Scan: Left Proximal Femur Osteochondroma






Gross Specimen: Left Proximal Femur Osteochondroma




OSTEOCHONDROMA vs. SECONDARY CHONDROSARCOMA

The cartilaginous cap deserves the most attention when differentiating a benign osteochondroma from a secondary chondrosarcoma that arose from a pre-existing osteochondroma

In adults, the cartilaginous cap regresses and becomes thin due to enchondral ossificastion of the majority of the cap.

Malignant transformation is suggested by:
       Cartilaginous cap thickness greater than 2cm 
       Cortical destruction
       Backgrowth of the cartilaginous cap into the stalk or medullary canal
       Lysis of calcifications in cap


Osteochondroma: Cartilage Cap


Radiographs
      Chondroid Calcification in cap
      Increasing destruction or change in appearance is worrisome for malignancy
Ultrasound - Good for cap and bursae
Bone Scan - Increased uptake in the cap
MRI:  Best test for evaluating thickness of cap and surrounding bursa
      Intermediate T1W Images
      High Intensity T2W Images because of fluid content
CT
     The cap will appear as soft tissue with calcification
     Can be difficult to distinguish from muscle
Cap thickness
     Benign < 1.5cm (0.1 - 3.0cm; Avg. 0.6 - 0.9 cm)
     Malignant > 1.5 cm (1.5 - 12cm;  Ave. 6cm)


Plain X-ray: Secondary Chondrosarcoma of Proximal Femur

MRI: Secondary Chondrosarcoma of Proximal Femur: Thick Cartilage Cap (>2cm)



PATHOLOGY

Pathology:
     Medullary and cortical continuity w/ underlying bone
     Hyaline Cartilage Cap with lobular growth
     Cartilage cap involutes after growth


GROSS PATHOLOGY

The osteochondroma is completely covered in periosteum
Cut surface shows hyaline composition of cartilage cap
      Cap
            Younger patients – thicker cap because of growth hormone
            Smooth or knobby
            2 mm to 1 cm thick
Beneath the cap, calcified cartilage which appear as white deposits are present


Intraoperative Photograph: Lobular Growth
(Top arrow)  Osteochondroma
(Bottom arrow)  Fibula

 

Specimen



Specimen Radiograph:  Osteochondroma

 

 


MICROSCOPIC PATHOLOGY

Hyaline cartilage cap overlying medullary bone
Hyaline cartilage cap has normally appearing chondrocytes
Nuclei magnified up to 5x normal diameter
Mild/Moderate nuclear atypia
Junction of cap and bone resembles epiphyseal plate
Enchondral ossification
Medullary bone contains fatty marrow

Microscopic Pathology:  Osteochondroma


(Top arrow)  Cells in Lacunae; Ground Glass Hyaline Cartilage Matrix





(Middle arrow)  Zone of Hypertrophy

 


(Bottom arrow)  Zone of Provisional Calcification; Enchondral Ossification


Microscopic Pathology:  Osteochondroma
Junction of Cap with Medullary Bone:  Similar to Growth Plate


TREATMENT

Simple excision:
           Cosmetic reasons
           Impingement on tendons, nerves or blood vessels
           Pain and limitation of motion
For multiple exostoses, corrective surgery may be necessary due to secondary deformities


PROGNOSIS

Recurrence after excision is rare
Rarely, osteochondromas may give rise to malignant chondrosarcoma
         Solitary osteochondromas 1%-2%
         Multiple osteochondromas 5%-25%
Most common sites to undergo malignant change
         Scapula, pelvis, ribs, proximal femur


Multiple Heredity Exostoses (MHE)

Clinical Data:
      Male predominance (3:1)
      AD inheritance
      Variability in size and number
      Any portion of the skeleton preformed in cartilage may be involved
      Present in childhood
      May be bilaterally symmetric
      One side may predominate
      Increased incidence of malignant transformation (10-20%)
      Radiographically characterized by multiple osteochondromas and undertubulation of bones  
      (Erlenmeyer Flask Deformity)


X-ray: Multiple Hereditary Exostoses of Proximal Femur


                                   

X-ray: Multiple Hereditary Osteochondromas of Distal Femur and Proximal Tibia/Fibula

 



X-Ray: Multiple Hereditary Osteochondromas of Distal Fibula



 

X-Ray: Multiple Hereditary Osteochondromas of Scapula/Proximal Humerus

 


Subungal Exostosis - Dupuytren Exostosis

Osteochondroma Variant
Females > Males (2:1)
Often painful and associated with trauma and infection
Fibrocartilage cap
Located away from physis 


Dysplasia Episphysealis Hemimelica - Trevor Disease


Male predominance (3:1)
Very rare < 100 cases
Swelling, pain and deformity
Usually lower extremity, unilateral
65% multiple done involvement: talus, distal femur, tibia


Dysplasia Episphysealis Hemimelica - Trevor Disease

Ankle and knee most common
Medial joint 2X lateral
Lobular epiphyseal mass
Histologically identical to an osteochondroma
May produce deformity and secondary osteoarthritis


 

 
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