Malignant bone tumours

MALIGNANT BONE

TUMOURS

PROF. AJAY PANTProf. & Head, Orthopaedics, TMMC&RC

TUMOUR

• A swelling of a part of body, generally without

inflammation, possessing no physiological

function, cause by abnormal growth of tissue

whether benign or malignant.

BENIGN

• Benign tumour is non – progressive

• Not harmful to human health

• Does not metastasize

MALIGNANT

• Progressive and tend to become worse and can

potentially result in death.

• Grow fast and seek new territory.

• Spread to distant sites (metastasis).

METASTASIS

• Malignant tumours invade nearby cells and

spread.

• Some cells break off from the tumor and

spread to various parts of body through blood

stream or lymphatic system.

• Establish elsewhere and form new malignant

tumours.

• metastasized cells are same as original.

CARCINOMA

• Tumours derived from skin or tissues that line

body organs (EPITHELIAL CELLS).

• eg. Stomach, Pancreas, Prostate, Lung, Liver,

Breast, Colon.

SARCOMA

• Start off in connective tissue such as -CARTILAGE, BONE, FAT, NERVES.

• Originate in the MESENCHYMAL CELLS

• Called after the cell/tissue/ structures they arise from e.g. –

– Liposarcoma

– Fibrosarcoma

– Osteosarcoma

– Chondrosarcoma

– Angiosarcoma

LYMPHOMA / LEUKEMIA

• Cancer arising from blood forming or

HEMATOPOIETIC CELLS

• Originate in the marrow and mature and

generally mature in the blood or lymph nodes.

GERM CELL TUMOUR

• Arise from –

– GERM CELLS

– PLURIPOTENT CELLS – cells that can turn

into any kind of cells.

• Present in the OVARY or TESTICLE

• Less commonly appear in brain, abdomen or

chest.

BLASTOMA

• Derived from EMBRYONIC TISSUE or

IMMATURE “PRECURSOR” CELLS

• More common in children.

– Glioblastoma

– Medulloblastoma

– Retinoblastoma

– Osteoblastoma

– Neuroblastoma

• Common primary malignant bone tumours-

– Multiple myeloma

– Osteosarcoma

– Ewing’s sarcoma

– Chondrosarcoma

OSTEOSARCOMA

• Osteosarcoma is an aggressive malignant

neoplasm arising from primitive transformed

cells of mesenchymal origin (and thus a

sarcoma) that exhibit osteoblastic

differentiation and produce malignant osteoid.

• It is the most common histological form of

primary bone cancer in age 12-25yrs.

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EPIDEMIOLOGY

• Male more than female

• At any age but

• 75% of cases appear at age 12-25yrs

RISK FACTORS:

• Radiation exposure

• Patients who survive after taking therapy for another

Cancer.

• Retinoblastoma(malignant tumor arising from retina

of eye).

SITES OF PREDILECTION:

• Osteosarcoma :It originates more frequently

(90%) in the metaphyseal region of tubular

long Bones, with 42% occurring in the femur.

• Commonest sites are distal femur, proximal

tibia and proximal humerus.

HISTOPATHOLOGY

This tumor arises from primitive

mesenchymal cells and is diagnosed by the

presence of malignant stromal cells laying

down tumor osteoid along with areas of

hemorrhage and necrosis and spicules of

destroyed bone .Depending on the

predominant tissue-- can be chondroblastic

,fibroblastic, and telangiectatic.

DIAGNOSIS OF

OSTEOSARCOMA

• Clinical examiation

• Radiologic examinatiom

• Biopsy for histopathology

CLINICAL PRESENTATION

SIGNS AND SYMPTOMS

• Persistent pain, may increase at night.

• Deep, firm, fixed mass may be palpable.

• Swelling usually develops near the end of a

long bone.

• Overlying skin may be stretched, thin, glossy

with distended veins.

• Adjacent joint may be impaired.

PLAIN XRAY

• The area of tumor mass

may be Lytic, sclerotic

or mixed

IN X – RAY WE CAN SEE CODMAN’S

TRIANGLE

• Codman’s triangle is a term used to describe

the triangular area of new subperiosteal bone

that is created when a lesion, often a tumour,

raises the periosteum away from the bone.

• sun-burst" appearance on X-ray examination

due to the tumor spicules of calcified bone

radiating in right angles

“Codman’s triangle” of bone appears as tumor

elevates periosteum from underlying bone.

Cortical soft tissue extension may produce

radiating spicules of bone called “sunray”

appearance.

CODMAN’S TRIANGLE

OSTEOSARCOMA

Elevated periosteum described as “Codman’s Triangle”

OTHER LOCAL

INVESTIGATIONS

• CT

• MRI

• Angiogram

SYSTEMIC INVESTIGATIONS

• Bone scan

• CT Chest

DIFFERENTIAL DX

• Giant Cell Tumor

• Aneursymal Bone Cyst

• Ewings tumor

• Osteoblastoma

• Metastasis

• Lymphoma

TREATMENT

Preoperative and/or postoperative chemotherapy Drugs

used are methotrexate,adriamycin and cisplatin (MAP

regimen) in a cyclical manner)

Resection - A procedure performed for the specific

purpose of removal

Allograft replacement - the process of transplanting

tissues and organs-limb salavging procedures

Amputation is to be reserved only for late presentation

EWING’S SARCOMA

• Identified in 1921 by James Ewing

• Third most common primary tumour of bones

• Second most common malignant bone tumour

of late childhood and early adulthood.

• Precise cell of origin unclear.

• Thought to arise from primitive mesenchymal

cells.

• ETIOLOGY - More common in white

population compared to Africans & Asians.

• AGE – first two decades of life.

• SEX – M:F ratio 1.4 to 1

• SITE – occurs throughout the skeleton but

more frequently in pelvis, long bone, ribs and

vertebral column.

CLINICAL PICTURE

• Commonest – pain and swelling

• About 10% patients present with pathological

fracture.

• Occasionally signs and constitutional

symptoms of systemic infection (often

confused with infection).

• Diagnosis often delayed in the pelvis and other

axial sites.

RADIOGRAPHIC FINDINGS

• X-rays usually show an area of bone destruction

predominantly in the diaphysis.

• New bone formation may extend along the shaft

and appear as fusiform layers of bone around the

lesion – ONION PEEL APPEARANCE

• Sometimes tumour extend into surrounding soft

tissues with radiating streaks of ossifications and

reactive periosteal bone presenting with

Codman’s triangle and Sun Ray appearance.

• MRI is excellent for describing lesions

especially in the marrow for extent of disease.

• PET – CT scans are used for metastasis and

staging of tumours.

• Lab findings may include anaemia,

Leukocytosis, elevated ESR and Lactate

Dehydrogenase (LDH levels).

• Biopsy

TREATMENT

• Multidisciplinary approach –

– Systemic multiagent chemotherapy

– Surgery

– Radiation therapy

OR

Combination of the two

• Multimodality treatment gives 60-70%

survival in localized disease.

MULTIPLE MYELOMA

• Most common primary malignancy of bone

• Malignant B cell lymphoproliferative disorder of the bone marrow.

• Characterized by presence of neoplastic plasma cells which replace bone and marrow spaces.

• Formation of abnormal protein leads to immune deficiency.

• Marrow cell proliferation and increased osetoclastic activity – osteoporosis and appearance of discrete lytic lesions in the skeleton.

• Large colony of plasma cells may form a solitary tumour – plasmacytoma

• Bone resorption Hypercalcemia

• Late features -1) Renal dysfunction

2) Vertebral collapse -Nerve root compression or cord

3) Pathological fractures

Age:

• Older persons – 40-70 Years

• Sex – Male > Female

ETIOLOGY

• Genetic Cases

• Environmental / occupational (wood, leather,

Paint workers).

• Chronic Inflammation

• Radiation

CLINICAL PICTURE

• 30 % cases asymptomatic and detected incidentally.

• Bone pain

• Anemia

• Infection

• Weakness

• Hypercalcemia

• Pathological fracture

• Spinal cord compression

• Signs & symptoms of renal failure in late cases –tubular blockade by myeloma proteins.

RADIOGRAPHIC FINDINGS

• Generated osteopenia

• Bi concave vertebral bodies

• Lytic “Punched out” bone lesions in skull,

pelvis, ribs, vertebral column, less commonly

in long bones.

LABORATORY FINDINGS

• Anemia

• Thrombocytopenia

• Increase blood urea

• Increase Serum Calcium

• Increase ESR

• Presence of Para proteins in –Serum – B2 Micro globulin

Urine – Bence Jones Proteins

• ‘M’ Band is serum protein electrophoresis

• Malignant plasma cells in filtrate the bone marrow –Histological examination of aspirate.

TREATMENT

• Supportive Medical therapy:

– Control of pain, Rest, Diet, Hydration

• Chemotherapy:

– Alkylating cytotoxic agents , E.g Melphelan,

Cyclophosphamide

– Corticosteroids

• Radiotherapy:

– Solitary Plasmacytoma more amenable.

– Control of pain in vertebral lesions.

• Surgical care:

– Internal fixation of pathological fracture s

– Decompression of Spinal cord