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
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(arrow) The Cartilage Cap
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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
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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 |
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Microscopic Pathology: Osteochondroma
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(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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