Malignant Bone tumors Malignant vs. Benign Tumors - PowerPoint Presentation

Slide1

Malignant Bone tumorsSlide2

Malignant vs. Benign Tumors

“

Rapid growth, warmth, tenderness, and ill defined edges are suggestive of malignancy.

”Slide3

Classification of malignant tumors of bone:

Osteosarcoma (

Osteogenic sarcoma)ChondrosarcomaEwing’s sarcomaMultiple myelomaSlide4

Osteosarcoma

(Osteogenic sarcoma)

It is a malignant mesenchymal tumor in which cancellous cell produce bone matrix.Most common primary malignant tumor of boneOccurs in all age group but has bimodal age distribution75% occur in person younger than 20 years of ageSecond peak occur in elderly who have predisposing condition – Paget disease, bone infarct, prior irradiationMales> femalesSlide5

Usually arise from metaphysis of long bones of extremities, and almost 50% occur about the knee.

Beyond the age of 25 years incidence in flat bones and long bones is almost equal.Slide6

Pathogenesis

Approx. 70% have acquired genetic abnormalities such as

ploidy changes and chromosomal aberrations, none of which is specific for this tumor.Mutation of RB gene (cell cycle regulator) and p53 gene (gene whose product regulate DNA repair and cellular metabolism) frequently associated with osteosarcoma.Germline mutation in RB gene roughly 1000-fold increase the risk of osteosarcomaPatient with Li-Fraumeni syndrome (germline p53 mutation) greatly elevate the incidence of osteosarcoma.Slide7

Several subtypes of osteosarcoma are grouped according to –

Site of origin (intramedullary,

intracortical or surface)Degree of differentiationPrimary (underlying bone is unremarkable)or secondary to preexisting disorders.Histological features (osteoblastic, chondroblastic, fibroblastic, telangiectatic, small cell and giant cell). The most common subtype arises in metaphysis of long bones and is primary, solitary, intramedullary and poorly differentiated.Grossly, Osteosarcoma are big bulky tumors that are gritty, grey-white, and often certain areas of hemorrhage and cystic degeneration.Slide8

OsteosarcomaSlide9

OsteosarcomaSlide10

Clinical features

Localized

pain and swelling Fast growing tumorProgressive weakness and weight lossSkin over the tumor is shiny and stretched with prominent veinsWarm, tender and ill defined margins.Pulsatile tumorMovement of adjacent joint restricted due to mechanical obstruction and effusion.Regional lymph node enlarged only in 25-30% cases.If distal neurovascular deficit present strongly suggest malignancy.Lung metastasis occur in 10-12 months if left untreated.Slide11

Classic X-ray findings:

Codman's triangle (periosteal elevation)

Sunburst pattern/Sunrays appearanceBone destructionIll-defined marginsSlide12

Codman’s triangle

Sunrays appearance

OsteosarcomaSlide13

Codman's triangle

OsteosarcomaSlide14

OsteosarcomaSlide15

Secondary osteosarcoma:

Occurs in old people

Associated with Paget’s disease or chronic osteomyelitisHighly aggressiveSlide16

Differential diagnosis

Among primary and secondary bone tumors

Sub acute and chronic osteomyelitisSlide17

Treatment

Classical

M.C.D WLE M.C.D 70% 5 yrs survival rate M – MethotrexateC – CyclophosphamideD -

Doxorubicin

T10 protocol

High dose

MTX

WLE

Biopsy

> 95% necrosis < 95% necrosis

chemosensitive

chemoresistant

M.C.D

Multiple drugs

(except MCD &

etoposide

) Slide18

Surgery

Disarticulation

AmputationResection with reconstruction/endoprosthesisLimb salvage surgeryResection of metastatic lesion (lobectomy in lung)Slide19

Limb salvage surgery

Principle is to eradicate the bone tumor, retain integrity of skeletal system and preserve the limb with useful function.

After resection, skeletal reconstruction done by bone grafting(auto or allograft) or by endoprosthesis (modular or custom made).Prosthetic reconstruction is more effectiveAs compared to the radical amputation and external prosthetic fitting or limb sparing surgery with bone grafting this treatment is more effective in early mobilization.Slide20
Slide21
Slide22

Chondrosarcoma

Definition:

Malignant tumor of chondroblasts cellsSecond most common malignant matrix producing tumorEtiology:The tumor may arise de novo (primary) or secondary to preexisting enchondroma, exostosis (osteochondromas) or Paget’s disease

Primary

chodrosarcoma

is very uncommon, arises centrally in the bone and found in childrenSlide23

Chondrosarcoma

sub classified according to –

Site – Central (intramedullary) Peripheral (juxtacortical and surface)Histologically - Conventional (hyaline/myxoid) Clear cell Dedifferentiated MesenchymalConventional central tumors constitute about 90% of chondrosarcomaSlide24

Occur more commonly after 40 years of age

Clear cell and especially

mesenchymal variant occur in younger patients in their 20s.Male > femaleIt is most common in the femur, humerus, ribs and on the surface of the pelvisPatients with Ollier's disease (multiple enchondromatosis) or Maffucci's syndrome (multiple enchondromas and hemangiomas) are at much higher risk of chondrosarcoma than the normal population.Slide25

Clinical features

P

resentation of chondrosarcoma depends on the grade of the tumor.A high-grade, fast growing tumor can present with excruciating pain.A low grade, more indolent tumor is more likely to present as an older patient complaining of hip pain and swelling. Pelvic tumors present with urinary frequency or obstruction or may masquerade as "groin muscle pulls".Slide26

Investigations

X – ray –

Chondrosarcoma is a fusiform, lucent defect with scalloping of the inner cortex and periosteal reaction. Extension into the soft tissue may be present as well as punctate or stippled calcification of the cartilage matrix.Slide27

ChondrosarcomaSlide28

C.T. scan –

H

elpful in defining the integrity of the cortex and distribution of calcification.MRI – Surgical planning as it demonstrates the intraosseus and soft tissue involvement of the tumor. MRI is also helpful in evaluating possible malignant degeneration of osteochondromas by allowing accurate measurements of the cartilage capSlide29

Biopsy - Incisional biopsy doneGross examination

–

Chondrosarcoma is a grayish-white, lobulated mass. It may have focal calcification, mucoid degeneration, or necrosisSlide30

ChondrosarcomaSlide31

Treatment

Treatment of

chondrosarcoma is wide surgical excision. There is a very limited role for chemotherapy or radiation.Low grade tm. – Limb salvage surgery(WLE)High grade tm. - AmputationSlide32

Ewing’s sarcoma

Malignant neoplasm of undifferentiated cells arising within the bone marrow

cavityEwing's sarcoma is a highly malignant tumor that is a type of peripheral primitive neuroectodermal tumorFound in the lower extremity more than the upper extremity, but any long tubular bone may be affected.Most common sites are the diaphysis and metaphysis of the femur followed by the tibia and humerus.Most common in the first and second decadeR

atio

of male to female is 3:2.Slide33

Clinical features

Presented

with pain, swelling and tendernessErythema and warmth of the local area are sometimes seenOsteomyelitis is often the initial diagnosis based on intermittent fevers, leukocytosis, anemia and an increased ESR.Contain glycogen granule so can cause hyperglycemiaSlide34

Investigation

X-ray:

Concentric, onion-skin layering of new periosteal boneThis appearance is caused by and splitting and thickening of the cortex by tumor cells. The lesion is usually lytic and central.CT is helpful in defining bone destruction. MRI is essential to elucidate the soft tissue involvementSlide35

Onion peel appearanceSlide36

Differential Diagnosis

Infection

Neuroblastoma metastasisLymphomaLeukemiaSlide37

Open biopsy for bone lesions

Grossly, the tumor is gray to white in color and poorly demarcated

. The consistency is soft and gray and sometimes semi-liquid especially after breaking through the cortex. Areas of hemorrhage and necrosis are common.Ewing sarcomaSlide38

Treatment

Chemotherapy – A –

Actinomycin D B – Bleomycin C – Cylophosphamide D – DoxirubicinSurgery followed by adjuvant chemotherapy.RadiotherapySlide39

Poor Prognostic Factor

High grade

tumourAge > 12 yrsMaleH/O fever and increase TLCProximal lesionLarger lesionMetastasisChemoresistantSlide40

MULTIPLE MYELOMA/ PLASMACYTOMA

Multiple myeloma is a malignant tumor of plasma cells that causes widespread

osteolytic bone damage. Multiple myeloma is the most common primary tumor of bone. Found in the spine, skull, ribs, sternum and pelvis but may affect any bone with hematopoietic red marrow. There are chromosomal abnormalities that are associated with MM, such as 14q32 and deletion of chromosome 13, and these findings are more likely to be found in cases with poor outcome. Other diseases, such as solitary plasmacytoma and monoclonal gammopathy are associate with MM.Slide41

A

verage

age of the patients at diagnosis is 65 years. Men are slightly more likely to get multiple myeloma than women.Slide42

Causes

Multiple

myeloma may occur spontaneouslyOn exposure to ionizing radiation and the pesticide dioxin Infection with some viruses (HIV and human herpes 8) has also been associated with multiple myeloma. No known risk factors are inherited. Slide43

SYMPTOMS

Usually

bone pain is main complain. Other symptoms include: Fatigue Feeling ill Fever Night sweats Weight loss is not common in the early stages.Patients are pale with diffuse bone tenderness, especially around the sternum (breastbone) and pelvis (hips). Slide44

Spine

is the most common location for a pathological fracture. It can also happen in the ribs and pelvis.

Compression of the spinal cord in 10%-15%This causes pain in the back and legs and numbness and weakness in the legs. Patients who have high levels of calcium in the blood may experience nausea, fatigue, confusion, constipation, and frequent urination. Patients with anemia may experience fatigue, weakness, and shortness of breath with exercise. In advanced cases, patients typically have recurrent infections and can have kidney failure. Slide45

Investigations

Blood

and urine tests – Monoclonal immunoglobulin (Ig G)is found on serum electrophoresis and on urinalysis. Immunoglobulin (Ig) is the protein that is produced by the tumor cells. Light chain subunits of immunoglobulin are called Bence Jones proteins and are present in urine.Bone marrow aspiration and/or biopsy – A procedure that involves taking a small amount of bone marrow fluid (aspiration) and/or solid bone marrow tissue (called a core biopsy), usually from the hip bones, to be examined for the number, size, and maturity of blood cells and/or abnormal cells. Slide46

DIAGNOSIS

X-

ray - a diagnostic test Punched out lesion in skullWhen only one lesion is found it is called a plasmacytomaMagnetic resonance imaging (MRI) - a diagnostic procedure. Slide47

Differential Diagnosis

E

wing sarcomaLymphomaLeukemia Acute infectionSlide48

CHEMOTHERAPY

The standard treatment medications are

melphalan and prednisone. The median survival rate is three years with this treatment alone. For patients in whom this therapy is ineffective, alternatives include: VBMCP (vincristine, carmustine, melphalan, cyclophosphamide and prednisone) VAD (vincristine, adriamycin and dexamethasone) Slide49

A recent advancement in the treatment of multiple myeloma has increased, response rates and survival.

This treatment consists of high-dose chemotherapy, followed by autologous stem cell transplantation. With this treatment, patients have a 20 percent chance of living longer than 10 years.

This stem cell transplantation involves: Harvesting a patient's own blood cells Conditioning them with very high doses of melphalan Re-infusing the blood cells back into the patient Slide50

RADIATION THERAPY

Radiation therapy is reserved for decreasing the size of symptomatic bone lesions. Slide51

SUPPORTIVE CARE

Supportive care is critical. Supportive care includes managing the bone disease, anemia, infections, kidney failure, and pain associated with multiple myeloma.

Bisphosphonates (medication) can prevent destructive bone lesions and spine fractures. Erythropoetin or occasional blood transfusions can manage anemia. Antibody infusions and vaccinations can help patients with recurrent infections. Corticosteroids and hydration can be used to treat high blood calcium concentrations (from bone loss) and dehydration. Narcotics can decrease the pain associated with bone lesions. Operative intervention may be required to stabilize and control the pain associated with bone fractures. Slide52

SURGICAL TREATMENT

Surgery will not cure multiple myeloma. Surgery is used to treat fractures and impending fractures in the spine, pelvis, hip, and shoulder. The goal of these surgeries is to decrease pain and maintain function.

Internal fixation augmented with cement is frequently recommended, as are joint replacements and vertebroplasties (for spine fractures). Operative intervention does not alter the survival rate, but it does increase the quality of life. Slide53

Bone Metastasis

It is catastrophic complication for most patient of cancer.

Usually occur in elderly age groupClinical features – PainMetastatic destruction of bone reduces load bearing capacity.Usually manifests as complication like pathological fracture, paraplegia (medullary compression), pressure symptoms. Slide54

M/C cause – Lung Carcinoma

Male – Prostate Carcinoma

Female – Breast CarcinomaM/C Area – D-L SpineIn prostate carcinoma – Pelvis > Lumber spine > Dorsal spineSlide55

M/C lesion – Lytic lesion

Purely

osteoblastic sec. – Prostate - CarcinoidMetastasis do not cross elbow and kneeSlide56

Bone tm. Which have bony metastasis –

Osteosarcoma

NeuroblastomaEwing sarcomaSlide57

THANK YOU