Bone Tumors by

Dr M.Rajani Cartor MPT (ortho)

Incidence of bone tumors is very low (1 to 1.5%) of the total malignancies of the body.

Bone being mesenchymal origin, the abnormality of cell growth can produce a tumor containing not only bone but also cartilage and fibrous tissue in varying

degrees.

Non inflammatory lesions in bone may be broadly grouped into tumor like lesions and true bone tumors.

Tumor like lesions of bone(simulating tumor)

• 1. Reactive bone lesions a. Osteoid osteoma

b. Benign osteoblastoma c. Non osteogenic fibroma

• 2. Hamartomas (abnormal proliferation, soon mature and stop proliferation)

a. Osteoma b. Osteochondroma c. Enchondroma

• 3. Cystic lesions a. Solitary bone cyst b. Aneurysmal bone cyst

Musculoskeletal Tumour Society staging of malignant bone lesions

• Stage: Definition:• III Any grade, metastatic• IIB High grade, extracompartmental• IIA High grade, intracompartmental• IB Low grade, extracompartmental• IA Low grade, intracompartmental

BONE TUMORS

1. Bone forming tumors (benign, intermediate, malignant)

2. Cartilage forming tumors (benign, malignant)

3. Giant cell tumor4. Marrow tumors (round cell tumors)

5. Vascular tumors (benign, intermediate, malignant)

6. Other connective tissue tumors (benign, intermediate, malignant)

7. Other tumors (benign, malignant)

Bone Tumors: by Age

Bone forming tumorsBenign : Osteoma, Osteoid osteoma & OsteoblastomaIntermediate: Aggressive Osteoblastoma Malignant: Osteosarcoma - central(medullary) - surface(peripheral)

a. parosteal b. periosteal c. high grade surface

Cartilage forming tumors• Benign - chondroma (enchondroma, periosteal)

- osteochondroma(solitary, multiple hereditary)

- chondroblastoma(epiphyseal chondroblastoma)

- chondromyxoid fibroma• Malignant - Chondrosarcoma(primary/secondary)

- Differentiated chondrosarcoma - Juxtacortical chondrosarcoma - Mesenchymal chondrosarcoma - Clear cell chondrosarcoma

GIANT CELL TUMOR• Osteoclastoma

Marrow tumors

• Ewing’s sarcoma

• Neuroectodermal tumor

• Malignant lymphoma (primary/secondary)

• Myeloma

Vascular tumors• Benign - Hemangioma - Lymphangioma - Glomus tumor(glomangioma)

• Intermediate/Indeterminate - hemangioendothelioma - hemangiopericytoma

• Malignant - angiosarcoma - malignant hemangiopericytoma

Other Connective Tissue Tumors• Benign - benign fibrous histiocytoma - lipoma

• Intermediate - desmoplastic fibroma

• Malignant - fibrosarcoma - malignant fibrous histiocytoma - liposarcoma - malignant mesenchymoma - leiomyosarcoma - undifferentiated sarcoma

Other Tumors

• Benign - Neurilemmoma - Neurofibroma

• Malignant - Chordoma - Adamantinoma

DIAGNOSIS

• HISTORY

• PHYSICAL SIGNS

• RADIOGRAPHY (X-ray, CT scan, MRI)

• LAB INVESTIGATIONS (RBC, Hb, ESR, VDRL, calcium, phosphates, urine)

• BIOPSY (open/closed/CT assisted needle biopsy)

Role of Radiography• Anatomical location diaphyseal (ewing’s sarcoma) diaphysiometaphyseal (osteosarcoma) metaphyseal (osteosarcoma) metaphysioepiphyseal (giant cell tumor, aneurysmal bone cyst) epiphyseal (chondroblastoma)

• Borders of tumor benign(well defined, narrow transitional area, reactive sclerosis)

malignant(poorly defined)

• Destruction pattern slow growth (geographical pattern) moderate growth (moth eaten pattern) rapid growth (permeative pattern)

• Matrix formation (new bone formation- woolly to dense sclerosis)

• Periosteal reaction non continuous, often laminated (sunray appearance) – osteosarcoma (Onion peel appearance) – ewing’s sarcoma

OSTEOSARCOMA • Malignant primary tumor characterised by direct

bone formation (proliferating tumor cells)• Occurs in age 10-20 years• Site: distal end of femur, proximal end of tibia, proximal end of humerus

• c/f: pain dominating initial symptom

swelling appears and increases in size(fusiform)

• Observation: skin stretched, shiny, vascular

swelling warm and show pulsation if very vascular firm to hard consistency(softening areas at soft tissue) late stages: tumor fungates• General health- anaemia, weight loss, cachexia

Radiological features:

Tumor arises at metaphyseal region(centrally/cortically).

Mottled areas of rarefaction with areas of osteosclerosis.

Extending beyond cortex, periosteum is raised and new bone forms at right angles. (“sunray” appearance)

Reactive new bone subperiosteally is codman’s triangle

Chest radiograph may show shadows by secondary deposits (canon ball appearance)

X ray Sun ray

Codman’s triangle

GIANT CELL TUMOR Tumor arises from supporting connective tissues of

bone. As it arises from osteoclasts – OSTEOCLASTOMA (benign but locally aggressive neoplasm with local recurrence).

Common in Indians Occurs at age of 30-40 years.site: metaphysioepiphyseal region of long bone distal femur/radius, proximal humerus/ tibia

c/f: bony swelling at knee/wrist. gradual growth and slight pain joint not involved but movement restricted due to mechanical

block eccentric swelling (if cortex thin- “egg shell crackling” on palpation) Some cases with pathological fracture.

• Radiological features: - osteolytic lesion occupying epiphyseal end. - trabeculations in the wall of cystic lesion show a “soap bubble”

appearance.

• - GCT into 3 grades : latent, active, aggressive types based on osteolysis, cortical destruction and expansion.

• Macroscopically: fleshy grayish mass with hemorrhage

• Microscopically: stroma with spindle cells(plump and ovoid)

• Treatment: surgical resection/en bloc exicision, curettage, bone graft after adjuvant therapy like phenol/ liquid nitrogen.

GCT- soap bubble appearance

CHONDROSARCOMA• Malignant tumor of cartilagenous origin.• 3rd most common

• Arises from diaphysio metaphyseal region of long bones• Central type occurs in proximal femur,ilium,humerusand scapula

• Common in males aged 30-50 years• c/f: swelling(hard and lobulated), dull aching pain.

grows slowly and restricts joint movement.

• Radiological features: expansion of medulla(central type) and thickening cortex with spotty calcification. Appears as lobulated swelling with patchy and fluffy calcification.

• Macroscopically: white or bluish mass of firm consistency. Appears to have

capsule and areas of myxomatous degeneration and softening.

• Microscopically: cartilage masses are hyperchromatic

• Based on cellularity, pleomorphism and mitosis chondrosacoma classified into 3 grades

• Treatment: surgical/limb saving(en bloc resection)

EWING’S SARCOMA• Highly malignant type (uncommon) occuring in

children.• 10% of all malignant tumors• Age 10-20 years (males)• Arises from primitive mesenchymal cells of

medullary cavity• Site: diaphyseal of long bones like femur, tibia,

humerus• Occurs in flat bones like pelvic bones.

• c/f: presents with pain gradually increasing, followed by swelling(firm to soft),indefinite margins, fever, anaemia, leucocytosis (simulates osteomyelitis), with soft tissues involvement ,swelling rapidly increases and

condition deteriorates, * Peculiar feature- metastasis occur in skull, vertebrae and ribs in

addition to lungs (blood stream).

• Radiological features: mottled rarefaction in bone affected, destruction of cortex, involvement of soft tissues, reactive new bone formation in layers “onion peel” appearance

• Macroscopically: tumor is pale soft mass (minimal bone) areas of degeneration and hemorrhage milky pus like fluid (simulating osteomyelitis)

• Microscopically: cellular with minimal stromal tissue, characteristic cell- small polyhedral with large nucleus, Appearance is monotonously uniform with cells in compact sheets with loose and vacuolated stroma some areas- pseudo rosette formation.

• Treatment: (MDT) vincristin, adriamycin, cyclophosphamide Surgical resection, grafting, Tumor radiosensitive and regression following therapy is remarkable.

Ewing sarcoma of the proximal humerus,presenting as tightly elastic, tense, ulceratedlesion with shining skin, on a grey-white background.Note the marked veins and skin striation

Musculoskeletal Tumour Society staging. Surgical margins

• Type Plane of Dissection

• Intralesional Within lesion

• Marginal Within reactive zone- extra capsular • Wide Beyond reactive zone through normal tissue

within compartment

• Radical Normal tissue extra compartmental

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