Malignant Bone Tumors

Radiography of osseous neoplasms

Standard radiography : X-rays Magnetic resonance imaging (MRI) Computed tomography (CT scan) radionuclide scanning

Radiographic findings in bone tumors

Bone destruction Tumor mineralized matrix Cortical invasion and penetration Periosteal reaction

Bone destruction
Geographic bone destruction : > 1 cm.
sclerotic margin sharp margin ill-defined margin

Moth-eaten destruction : > .5 cm. Permeative destruction

Tumor mineralized matrix

Osteiod : solid, cloud-like, ivory masses
Chondroid : stippled, flocculent, rings and arcs, popcorn, punctate

Cortical invasion and penetration

Cortical expansion Cortical thinning
Complete cortical penetration - soft tissue invasion

Periosteal reaction
Solid periosteal reaction (cortical thickening) Single lamellar reaction Lamellated reaction (onion skinning) Parallel spiculated - hair on end and sunburst Butress Codmans triangle Interrupted lamellated and spiculated

Histologic Classification of Bone Tumors

Bone-forming tumors Cartilage-forming tumors Giant cell tumor Marrow tumors (round cell tumors) Vascular tumors Other connective tissue tumors Other tumors : Chordoma, Adamantinoma Unclassified tumors Tumor-like lesions

Osteosarcoma
Malignant neoplasm of bone with osteoid production by neoplastic cells Primary and secondary osteosarcoma

Primary osteosarcoma
Conventional central osteosarcoma Definition : no underlying bone diseases or under carcinogenic influences (de novo) age : 10-20 M>F distribution and location : metaphyses of long bone
lower end of femur upper end of tibia
70 %

Primary osteosarcoma
Upper end of humerus pelvic girdle, jaw

radiology :
geographic bone destruction of medulla lytic and sclerotic tumor matrix cortical bone destruction and penetration to soft tissue

periosteal reaction : Codmans triangle, sunburnt

Primary osteosarcoma
Gross :
osteoblastic type : gray-white to yellow, firm, finely gritty, areas of hemorrhage and necrosis chondroblastic type : translucent white lobules fibroblastic type : tan, soft to firm mass Codmans triangle ( reactive bone or tumor) spreading along marrow cavity rarely penetrate active epiphyseal plate uncommon joint space involvement

Primary osteosarcoma
Histology : anaplastic sarcomatous cells with osteoid production directly from malignant cells osteosarcoma variants
osteoblastic fibroblastic chondroblastic giant cell-rich

Primary osteosarcoma
Malignant fibrous histiocytoma-like round cell (small cell) poor prognosis Telangiectatic poor prognosis intraosseous well-differentiated good prog. Intracortical rarely found

prognosis
5-yr survival rate 10-20% hematogenous spreading to lungs, brain

Secondary osteosarcoma
Pagets disease of bone (osteitis deformans) Radiation exposure (10-15 yrs) Chemotherapy

Pre-existing benign bone lesions : fibrous dysplasia, osteochondromatosis, chondromatosis Chronic osteomyelitis and bone infarct

Secondary osteosarcoma
Foreign body : total hip replacement
Clinical
Age : > 40 yrs more aggressive

Parosteal osteosarcoma
(juxtacortical osteosarcoma)
Juxtacortical position at metaphyses of long bone common - distal femur age : >20 yrs slow growing, circumscribed cortex late stage - invade medullary cavity

Parosteal osteosarcoma
(juxtacortical osteosarcoma)
X-ray :
extracortical mass with radiodensity radiolucent line (early lesion) must be differentiated from myositis ossificans

Gross : large ossified, exophytic mass attached to the involved bone by broad base, chondroid areas (simulate osteochondroma)

Parosteal osteosarcoma
(juxtacortical osteosarcoma)
Histology :
long, narrow trabeculae or ill-difined islands of osteoid and woven bone sepatated by fibrous stroma minimal cytologic atypia of fibroblastic cells with scattered normal appearing mitosis islands of cartilage

Good prognosis : 5- yr survival 80-90 %

High grade surface osteosarcoma

Juxtacortical position at metaphyses of long bone Histology and prognosis : same as conventional central osteosarcoma

Chondrosarcoma
Central chondrosarcoma - 75% Peripheral chondrosarcoma Juxtacortical chondrosarcoma

Central chondrosarcoma
Age : 30-60 yrs M:F=3:1 pelvis, ribs, knee, shoulder girdle
X-ray : destructive osteolytic lesion with mottling radiodensity -- snowstorm

Gross : varying sizes, lobulated gray-white translucent mass with spotty calcification

Central chondrosarcoma
Histology : anaplasia of chondrocytes in lacuna
hyperchromatic nuclei two or more cells in one lacuna multinucleated giant cells bizarre giant cells grade 1, 2, 3 or low, intermediate , high

Central chondrosarcoma
Clinical :
slow growing late metastasis : lungs, liver

Variants
dedifferentiated chondrosarcoma mesenchymal chondrosarcoma

clear cell chondrosarcoma Myxoid chondrosarcoma

Central chondrosarcoma
Overall 5-yr survival rate
Grade 1 90% Grade 2 80% Grade 3 43%

Peripheral chondrosarcoma
Malignant transformation of
cartilaginous cap of osteochondroma multiple enchondromatosis

Ewings sarcoma
Primitive neuroectodermal cells Ewing/PNET Small round cell tumor Reciprocal translocation : t(11; 22) Age : 5-20 yrs M=F Sites : diaphyses or metaphyses of long tubular bones -- femur, tibia, humerus, fibula, pelvis, ribs

Ewings sarcoma
X-ray :
early : small lytic lesion in cancellous bone late : cortical mottling, permeative destruction, cortical thickening, periosteal reaction -- onion skin, sunray

Gross : soft gray-white with subperiosteal reaction

Ewings sarcoma
Histology :
densely packed small cells with round nuclei, indistinct cytoplasmic border, no nucleoli pseudorosette cytoplasmic glycogen (PAS stain), CD99+ DDx : lymphoma, neuroblastoma, embryomal rhabdomyosarcoma

Ewings sarcoma
Clinical :
osteomyelitis-like : pain, swelling, fever, increase wbc count, increase ESR hematogenous spread : lungs, liver, bone

Prognosis : 5-yr survival rate

5-10% by surgery or radiation alone 40-50% by adjuvant therapy

Metastatic tumors
Most frequent of all bone malignancy Breast, prostate, thyroid, kidneys, GI tract 70% - axial skeletons (skull, ribs, spine, sacrum) metaphyses of long bones usually osteolytic osteoblastic in CA prostate, CA breast