Osteosarcoma (1)

1.Osteo = bone/osteoid tissue Sarcoma = malignant tumour of connective tissue 02/04/12 Dr. Pruthviraj Nistane Deptt. Of Orthopaedics Govt. Medical College and Rajindra Hospital, Patiala

Definition

Epidemiology

Pathogenesis

Skeletal distribution

Clinical presentation

Evaluation

Classification

Investigations

Treatment

Prognosis

Highly malignant tumor of mesenchymal origin.

Spindle shaped cells that produce osteoid.

2 ndmost common primary malignant bone tumor after MM.

Incidence 1 to 3 per million per year

Any age

But 75%in 12-25yrs of age

Almost equal in both sexes, slightly more in males .

Associatedsyndromes

Hereditary form of retinoblastoma

Li-Fraumeni syndrome (p53)

Rothmund-Thomson syndrome (8q24)

Unknown

Modal incidence correlates with rapid bone growth

Radiation >2000 rads

Chemicals chlorantherene, AAF, Be compounds

Arise from multipotent mesenchymal cells

Mixture of osteoid, fibrous, cartilaginous, necrotic,

hemorrhagic, cystic areas

Destruction of cortex

Metaphyseal, Central.

Extension into medullary cavity and subperiosteal extension.

Restricted bu periosteun and epiphyseal plate, but eventually crosses it

Reactive periosteal

new bone formation

Metastasis lungs

Stroma - Malignant connective tissue with anaplastic spindle cells

Matrix of osteoid/fibrous/cartilagenous tissue

PRIMARY or SECONDARY

PRIMARY OSTEOSARCOMAS are

Conventional /classicosteosarcoma (highgrade, intra medullar y)

Low-grade intramedullary osteosarcoma

Parosteal osteosarcoma

Periosteal osteosarcoma

High-grade surface osteosarcoma

Telangiectatic osteosarcoma, and

Small cell osteosarcoma.

SECONDARY OSTEOSARCOMAS

Osteosarcomas occurring at the site of another diseaseprocess.

more common in >50 years of age

The most common causes are

Paget disease

Previous radiation treatment

Other associated conditions are

Fibrous dysplasia

Bone infarcts

Osteochondromas

Chronic osteomyelitis

Dedifferentiated chondrosarcomas

Osteogenesis imperfecta

These aggressive, high-grade tumors begin in an intramedullary location, but may break through the cortex and form a soft-tissue mass.

The histologic hallmark -malignant osteoblastic spindle cells producing osteoid,presence of woven bone with malignant appearing stromal cells

subtypes-

osteoblastic,

chondroblastic and

fibroblastic

Distal femur

Proximal tibia

Proximal humerus

(sites of rapid bone growth)

others

Metaphyseal(89%)>diaphyseal(10%)>epiphyseal(1%)

Pain progresssive pain

due to microinfarction

night pain in 25 %

Swelling-Palpable mass is noted in up to 1/3

of patients at the first visit

Fever, malaise or other constitutional symptoms

are not typical of osteosarcoma

Suspected diagnosis by history and physical examination

Supported by investigations

sclerotic

Lesions are usually permeative

Associated with destruction of the cancellous and cortical elements of the bone

Ossification within the soft tissue component, if tumour has broken through cortex

Intra medullary

Borders are ill defined

Periosteal reactionmay appear as the characteristic Codman triangle.

Extension of the tumor through the periosteum may result in a so-called sunburst or hair on end appearance.

Angiogram

Bone scan

Laboratory studies

Biopsy

best to detect extent into bone and soft tissues

Not of much use

CT chest to detect lung metastasis

Determine vascularity of the tumour

Detect vascular displacement

Relationship of vessels to the tumour

A bone scan should be obtained

to look for skeletal metastases

or multi focal disease

Thallium scan - Monitor effects of chemotherapy

Detect local recurrence of tumor

Full blood count, ESR, CRP.

LDH (elevated level is associated with poorprognosis)

ALP (highly osteogenic)

Platelet count

Electrolyte levels

Liver function tests

Renal function tests

Urinalysis

to conform the diagnosis.

Fine needle aspiration

Core needle biopsy

Openincisional biopsy

The staging system is typically depicted as follows

Stage I: Low grade tumors

I-A intra compartmental

I-B extra compartmental

Stage II: High grade tumors

II-A intra compartmental

II-B extra compartmental

Stage III: Any tumors with evidence ofmetastasis

Giant Cell Tumor

Chondrosarcoma

Fibrosarcoma

Aneursymal Bone Cyst

Ewings sarcoma

Osteoblastoma

Metastasis

Lymphoma

Osteomyelitis

Chondroblastoma

Post traumatic callus

Other variants

Parosteal

Periosteal

High grade surface osteosarcoma

5% of osteosarcomas

Posterior metaphysis of

distal femur

Arises from surface,invade

medullary cavity in late stages

Tends to encircle bone

Low grade,Slow growing

Large ossified mass in centre

Arises from surface of diaphysis

Most commonly femur and tibia

Characterized by bony spicule formation

perpendicular to shaft

Strands of osteoid producing spindle cells

radiating between lobules of cartilage

Sunburst

Low grade

Very rare

Age group 20-30s

Appearance as parosteal but histology high grade and medullary involvement more common.

Aggressive

Presents with pathological fracture

5% of all osteosarcomas

arises within the diaphysis

Radiology

Often entirely osteolytic

Bone and cortex destruction

Periosteal reaction

Codman's triangles

Pathology

Gross appearance is a multi-cystic similar to an aneurysmal bone cyst.

Microscopically it has large blood filled spaces and thin septation. Within the septa there is scanty osteoid production by the pleomorphic malignant cells

Extent of the disease

Pts with pulmonary, non pulmonry (bone) or skip metastasis have poor prognosis

Grade of the tumor

High grade tumor have poor prognosis

Size of the primary lesion

Large size tumors have worse prognosis then small size tumors

Skeletal location

proximal tumors do worse than distal tumors.

Secondary osteosarcoma:Poor prognosis

Current standard of care

Radiological staging

Biopsy to confirm diagnosis

Preoperative chemotherapy

Repeat radiological staging (access chemo response, finalize surgical treatment plan)

Surgical resection with wide margin

Reconstruction using one of many

techniques

Post op chemo based on preop response

Chemotherapy given preoperatively - Neoadjuvant

Given postoperatively -Adjuvant

Advantages ofneoadjuvantchemotherapy -

regression of the primary tumor, making a successful limbsalvage operation easier.

may decrease the spread of tumor cells at the time of surgery

Effectively treating micrometastases at the earliest time possible.

It avoid tumor progression, which may occurduring any delaybefore surgery.

Given for about 3-4 weeks before definitive procedure

The drugs used most often to treat osteosarcoma are:

Methotrexate with leucovorin (folinic acid)

Doxorubicin (Adriamycin)

Cisplatin or carboplatin

Etoposide

Ifosfamide

Cyclophosphamide

Actinomycin D (dactinomycin)

Bleomycin

Themain goal of surgery is to safely and completely remove the tumor.

Historically amputation.

Over the past few years -limb-sparing procedures have become the standard, mainly due to advances in chemotherapy and sophisticated imaging techniques

Limb salvage procedures now can provide rates of local control and long-term survival equal to amputation.

Surgical procedures fall into three basic categories:

Amputation

Limb salvage

Rotationplasty

If the tumor can be removed safely while retaining a viable extremity, a limb sparing procedure may be appropriate.

If major nerves or blood vessels are involved, or if complete tumor removal results in significant loss of function, amputation may be a better choice.

Patients age, desired level of function, cosmetic preference and long-term prognosis must also be considered.

Amputation involves removal of the limb with a safe margin between the end of

the retained portion and the

It should not be viewed as a

failure of treatment, but rather

as the first step towards patients return to a more comfortableand productive life.

Indication

1. Grossly displaced pathologic fracture

2. Encasement of neurovascular bundle

3. Tumor that enlarges during preop chemo andis adjacent to neurovascular bundle

4. Palliative measure in metastatic disease

5. If the tumor has caused massive necrosis,fungation, infection, or vascular compromise.

Removing the tumor with a normal cuff of tissue surrounding it while preserving vascular and nerve supply to the extremity.

The skeletal defect must be reconstructed by

Endoprosthesis (most common)

replacing the removed bone with

a metal implant

Allograft (cadaveric) bone

Vascularized bone acquired from the patient

Allograft-prosthetic composite constructions

compromise between amputation and limb salvage

most commonly used for osteosarcomas of the distal femur in skeletally immature patients

It is a procedure where the neurovascular structures and distal aspect of the limb (leg) are retained, and re-attached to the proximal portion after the tumor has been removed.

For functional purposes, the distal segment is turned 180 degrees so that the ankle joint functions as a knee joint, thus converting an above-knee to a below-knee amputation in order for prosthetic use to be maximized

Radiation therapy has no major role in osteosarcoma

Radiation therapy may be useful in some cases where the tumor cannot be completely removed by surgery. E.g. in pelvic bones or in the bones of the face.

Megavoltage (upto 6000-8000 rads)

Signs of recurrence, metastasis and treatment related complications

Physical examination,radiographs of the primary site, serial chest imaging,bone scans and laboratory examinations

50 % cases with high grade osteosarcoma have some type of relapse in 5 months

If recurrence is detected, additional surgery(radical amputation)and chemotherapy may be warranted.

5 year survival rate is 5% - 23%

THANK YOU !

Osteosarcoma (1)

Health & Medicine

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