TumorsKhalid Al Hamadani

OUTLINE Introduction Classification of Bone tumors Clinical Presentation Staging of Bone tumors Primary Bone tumors Case Presentation

Introduction A bone tumor is an abnormal growth of

cells within a bone. The cause of bone tumors is unknown. They often occur in areas of rapid bone

growth. Possible causes include:1.Genetic defects passed down through families2.Radiation3.InjuryMedline plus: Service of U.S national Library of Medicine http://www.nlm.nih.gov/medlineplus/ency/article/001230.htm

Introduction Cancers that start in the bones are

referred to as primary bone tumors. Cancers that start in another part of the

body (such as the breast, lungs, or colon) are called secondary or metastatic bone tumors.

Medline plus: Service of U.S national Library of Medicine http://www.nlm.nih.gov/medlineplus/ency/article/001230.htm

Classification of Bone tumors

Most classifications of bone tumors are based on the dominant tissue in the various lesions.

Classification of Bone tumors

Clinical presentation History Examination Imaging Biopsy Important differential diagnosis

History History is often prolonged, results in delay

of treatment. Patients most of the time will be completely

asymptomatic until the abnormality is discovered on X-ray.

This is more of benign lesions, common in children and rare after 30

Malignant tumors can be silent if they are slow growing and there is room for expansion like cavity of the pelvis.

History AGE: Useful clue. Many benign tumors present during

childhood and adolescence Chondrosarcoma and fibrosarcoma typically

occur in 4th and 6th decades adults. Myeloma rarely seen before 6th decade. Patients over 70 years metastatic bone

lesions are more common than primary tumors.

History PAIN: common complaint and gives little

indication of the nature of lesion. Progressive and unremitting pain is a

very important symptom though. It may be caused by:1. Rapid expansion2. Central hemorrhage or degeneration of

the tumor3. Pathological fracture.

History Swelling: Appearance of a lump, may

be alarming. Neurological symptoms: Parasthesia

and numbness may be caused by pressure or stretching of a peripheral nerve.

Progressive dysfunction is alarming and suggests invasion by an aggressive tumor.

History Pathological fracture: may be the

first and only clinical sign. In elderly people whose bones usually

fracture at the cortico-cancellous junctions, if they get mid shaft fracture it is regarded as pathological until proven otherwise.

Examination If there is a lump: Where does it arise? Is it well defined or not? Soft, hard or pulsatile? Tender? Swelling can be diffuse and the

overlying skin warm and inflamed which makes it hard to distinguish from infection and hematoma.

Examination If it is near a joint: is there any

movement limitation? Spinal lesions cause muscle spasm,

back stiffness or painful scoliosis. Examination will focus on symptomatic

part but it should include lymph nodes, pelvis, abdomen, chest and spine.

Imaging X-ray: Most useful of all imaging

techniques. There might be obvious abnormality of

the bone:1. Cotrical thickening2. Discrete lump3. Cyst4. Ill-defined destruction

Imaging Is it in the metaphysis or diaphysis? Is it solitary or multiple lesions? Margins are well or ill defined? Note: cystic lesions are not necessarily

hollow cavities: any radiolucent material may look like a cyst (e.g fibroma and chondroma)

Imaging If the boundaries of the cyst is well

defined is mostly benign. If it is hazy and diffuse it is mostly

invasive tumor. Bone surfaces: periosteal new bone

formation and extension of the tumor to the soft tissues are suggestive of a malignant tumor.

Imaging Soft tissues: are the muscle planes

distorted by the swelling? Is there any calcification?

X-ray is not a definitive diagnosis and further investigation must be done to confirm.

X-RAY

Imaging Other techniques of imaging used are

Radinuclide scanning, CT and MRI. They all help in viewing the lesions

better, view soft tissue and detect skip lesions too.

Patient must not go for biopsy if MRI or CT is planned for him as it will distort the image and appearances.

Biopsy There are three ways:1.Needle biopsy: Must be performed by experienced personal.2. Open biopsy: most reliable way of obtaining a representative sample.3. Excisional biopsy: for benign tumors.

Deferential Diagnosis

Deferential Diagnosis

Deferential Diagnosis

Deferential Diagnosis

Staging of bone tumors Staging is the process of finding out how

far the cancer may have spread. This is very important because the type

of treatment and the outlook for recovery (prognosis) depend on the stage of the cancer.

Staging of bone tumors In treating tumors we are facing two

conflicting principles:1. Lesion must be removed widely to

ensure it doesn’t recur.2. Damage must be kept minimal.

Staging of bone tumors The balance between the 2 conflicting

objectives depends on knowing:1. How the tumor behaves

(Aggressiveness)2. How far it has spread.

The answers to these two questions are embodied in the staging system of Enneking.

Tumor

Benign

Latent

Active

Aggressive

Malignant

Low Grade

High Grade

Aggressiveness

Enneking Staging system of bone tumorsBenign Tumors

Latent Well defined margin. Grows slowly and then stops.Remains static/heals spontaneously E.g Osteoid osteoma

Active Progressive growth limited by natural barriers.Not self limiting. Tendency to recur E.g Aneurysmal Bone cyst

Aggressive Growth not limited by natural Barriers E.g Gaint cell tumor

Enneking Staging system of bone tumors

Malignant Tumors

Low Grade Moderatly aggressive and takes a long time to metastasize

High Grade Very aggressive and metastasize early

Spread Assuming that there is no metastases,

the local extent of the tumor is the most important factor in deciding how much tissue to be removed.

Spread

Intracompartmental

Extracompartmental

Spread Lesions that are confined to an enclosed

space (e.g Bone cavity, joint cavity or muscle group within its fascial envelope) are called Intracompartmental.

Lesions that extend into interfascial or extrafascial with no natural barrier to proximal or distal spread are called Extracompartmental. (E.g pelvis, axilla)

Surgical stage Staging the tumor is an important step

towards selecting the best operation suited to the patient.

Bone sarcomas are divided as follows:1. Stage 1: All low grade sarcomas2. Stage 2: Histologically high grade

lesions3. Stage 3: Sarcomas which have

metastasized.

Surgical stages described by EnnekingStage Grade Site Metastases

IA Low Intracompartmental

No

IB Low Extracompartmental

No

IIA High Intracompartmental

No

IIB High Extracompartmental

No

IIIA Low Intra/extracompartmental

Yes

IIIA High Intra/extracompartmental

Yes

Management

Primary bone tumors

Divided into Benign and Malignant.

They are rare to occur and secondary bone tumors are more common.

Primary Bone tumors Benign Tumors: Osteoid Osteoma Giant cell Tumor Enchondroma

Primary Bone tumors Malignant tumors: Multiple myeloma Osteosarcoma Ewing’s sarcoma Chondrosarcoma

Osteoid Osteoma peak incidence in 2nd and 3rd decades,

M:F = 3:1 small, round radiolucent nidus (<1 cm)

surrounded by dense bone tibia and femur most common

produces severe intermittent pain, mostly at night

characteristically relieved by NSAIDs

Osteoid Osteoma

Osteochondroma 2nd and 3rd decades, M:F = 1.8:1 metaphysis of long bone

cartilage-capped bony spur on surface of bone may be multiple

higher risk of malignant change

generally asymptomatic unless impinging on neurovascular structure

malignant degeneration occurs in 1-2%

Enchondroma 2nd and 3rd decades 50% occur in the small tubular bones of the

hand and foot; others in femur, humerus, ribs benign cartilagenous growth, develops in

medullary cavity single/multiple enlarged rarefied areas in

tubular bones lytic lesion with sharp margination and central

calcification malignant degeneration occurs in 1-2%

Enchondroma

Cystic lesions includes unicameral/solitary bone cyst (most

common), fibrous cortical defect children and young adults local pain, pathological fracture or incidental

detection lytic translucent area on metaphyseal side of

growth plate cortex thinned/expanded; well defined lesion

treatment of unicameral bone cyst with steroid injections ± bone graft

Cystic lesions Treatment treatment only necessary if

symptomatic osteochondroma: resection cystic lesions: currettage and bone graft

Cystic lesions

Giant cell Tumor affects patients of skeletal maturity, peak 3rd decade distal femur, proximal tibia, distal radius, sacrum, tarsal

bones, spinal (osteoblastoma) cortex appears thinned, expanded; well-demarcated

sclerotic margin local tenderness and swelling aggressively destroy bone 15% recur within 2 years of surgery giant cell tumour occasionally metastasizes (1-2%) Treatment intralesional curettage + bone graft or cement wide local excision of expendable bones

Giant cell tumor

Osteosarcoma mostly frequently diagnosed in 2nd decade of life (60%) Mostly affects distal femur (45%), proximal tibia (20%)

and proximal humerus (15%) invasive, variable histology; frequent metastases without

treatment painful, poorly defined swelling x-ray shows

characteristic periosteal elevation and spicule formation representing tumour extension into periosteum

treatment: complete resection (limb salvage, rarely amputation), chemotherapy

survival “ 70%

Osteosarcoma

Chondrosarcoma primary

previous normal bone, patient over 40; expands into cortex to give pain, pathological fracture, flecks of calcification

secondary malignant degeneration of pre-existing cartilage

tumour such as enchondroma or osteochondroma most commonly occurs in pelvis, femur, ribs,

scapula, humerus unresponsive to chemotherapy, treat with

aggressive surgical resection + reconstruction

Chondrosarcoma

Ewing’s sarcoma most occur between 5-20 years old florid periosteal reaction in diaphysis of long

bone moth-eaten appearance with periosteal

lamellated pattern (onion-skinning) present with mild fever, anemia, leukocytosis

and increased ESR metastases frequent without treatment treatment “ resection, chemotherapy, radiation survival “ 70%

Multiple myeloma most common primary malignant tumour of bone in

adults 90% occur in people >40 years old present with anemia, anorexia, renal failure,

nephritis, increased ESR, bone pain, compression fractures, hypercalcemia

diagnosis punched-out lytic lesions on x-ray at multiple bony

sites serum/urine protein electrophoresis

treatment: chemotherapy, radiation, surgery for symptomatic lesions or impending fractures

References Medline plus: Service of U.S national Library of

Medicine http://www.nlm.nih.gov/medlineplus/ency/article/001230.htm

Apley’s system of orthopedics and fractures (Ninth Edition)

Apley’s Concise system of orthopedics and fractures (Third Edition)

American Cancer society http://www.cancer.org/Cancer/BoneCancer/OverviewGuide/bone-cancer-overview-staging

First Aid for the USMLE Step 1 2011 Toronto notes 2009

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