Booooooone Tumors REFERAT

8/13/2019 Booooooone Tumors REFERAT

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NURLAILA ISHAQ

BONE TUMORS


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EFINISI

Tumor tulang merupakan kelainan pada sistem

muskuloskeletal yang bersifat neoplastik, dapat bersifat jinakatau ganas.

Tumor benjolan,

Neoplasma setiap pertumbuhan baru dan abnormal

Tumor ganas tulang dapat bersifat primer yang berasal dariunsur unsur tulang sendiri atau sekunder dari metastasis (infiltrasi ) tumor tumor ganas organ lain ke dalam tulang.


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3. True Neoplasms of Bone

A. Osteogenic1. Osteosarcoma2. Parosteal sarcoma

B. Chondrogenic1. Benign

chondroblastoma2. Chondromyxoid

fibroma3. Chondrosarcoma

C. Collagenic1. Fibrosarcoma2. Angiosarcoma

D. Myelogenic1. Plasma cell myeloma2. Ewings tumor 3. Reticulum cell

sarcoma4. Hodgkins disease

E. Osteoclastoma (Giantcell tumor of bone)


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5

EPI EMIOLOGI

Dari seluruh tumor tulang primer; 65,8 % bersifat jinak dan34,2 % bersifat ganas.

Tumor jinak primer osteoma ( 39,3 % ), osteokondroma( 32,5 % ), kondroma ( 9,8 % ) dan sisanya oleh tumor

tulang jinak yang lain.

Osteogenik sarcoma ( 48,8 % ), giant cell tumor ( 17,5 % ),

kondrosarkoma ( 10 % ) merupakan tumor ganas

primer yang paling sering ditemukan


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CLINICAL MANIFESTATION

History :may be completely asymptomatic before xray findings more likely in benign lesion

Malignant lesion may remain silent if :

Slow growing


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Pain : common complaint Progressive & unremitting pain

symptom, caused by : Rapid expansion with stretching of

surrounding tissues, central haemorrhage ordegeneration of tumour

An incipient pathological fracture

Tiny lesion very painful if encapsulatedin dense bone


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Swelling / lump may be alarmingwhen mass becomes painful / continuousto grow px seeks advice

History of trauma : whether the injury

initiate pathological changes or merelydraws attention unanswered


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Neurological symptoms (paraesthesia /numbness) pressure upon or stretching ofperipheral nerveProgressive dysfunction invasion by anaggressive tumour

Pathological fracture: First (& only) clinical sign Injury was slight Elderly people with any break (fracture) of mid-shaft


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If the lesion is to close to a joint functiondisturbed and there may also be painfulrestriction of joint motion


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CLINICAL MANIFESTATIONExamination Lump : where, discrete / ill-defined,

soft / hard, pulsatile, tenderness

Swelling : distinguish it from infection orhaematoma

Near joint : effusion & / limitation of

movement Spinal lesions : muscle spasm, back

stiffness, painful scoliosis


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In malignant neoplasm, blood vesselsgrow in radial fashion from cortex accompanied by neoplastic bone &reactive bone Sunburst appearance

Pathological fracture occurs through

abnormal area, due to weakening bylocal destruction (osteoclatic resorption) Rapidly growing neoplasms there

may be little or no reactive bone.Usually due to metastase from otherprimary sites tumor


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DIAGNOSIS

Made by clinical history, physical signs,radiographic features, biochemical findings, grossand microscopic appearance of biopsyBiochemical findings:- Serum Ca ~ Osteolytic metastase

- Alkaline phosphatase ~ Osteoblastic activity- Acid phosphatase ~ Ca prostate- Total protein concentration ~ multiple

myeloma


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PRINCIPLES OFMANAGEMENT

Tumour excisionmethods:

1. Intracapsularexcision

2. Marginal excision3. Wide local excision

4. Radical excision


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PRINCIPLES OF MANAGEMENT

Multi-Agent Chemotherapy For sensitive tumour reduce the size, prevent

metastatic seeding, improve survival Drugs : MTX, Doxorubicin (adriamycin),

cyclophosphamide, vincristine, cisplatin 8 12 weeks pre-op, if not effective change

to different drug for post-op treatment Maintenance chemotherapy Another 6

12 months


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Osteoid osteoma

The patient complains of persistent pain Any bone except skull may affected, but

over half cases occur in the femur andtibia

Px complains : persistent pain localizedor referred over wide area

Patients are usually under 30 years oldand male predominate


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SUBPERIOSTEAL CORTICAL

DEFECT The commonest radiographic lesion

Detected in 10-20% children in skeletal growth Commonly seen in metaphyseal region ofdistal femur

No clinical significance for this lesion Usually without symptoms No treatment required for this lesion


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Non Osteogenic Fibroma

Similar to sub periosteal cortical defect Seen in children and adolescents Commonest sites in long bones Usually asymptomatic


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Osteochondroma The benign tumor lesion consists of an outgrowth of bone

& cartilage In young persons, may persist in adult Commonest sites are distal femur, proximal tibia, proximal

humerus Malignant changes in 1%, higher in multiple form Not a painful lesions, but interfere with function of

surrounding soft tissues

The lesion always appear larger clinically thanradiologically Not all osteochondromas require treatment, only

cosmetically ugly or interfering function of limb should beexcised


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Enchondroma

Although benign, it probably develops localabnormality of growth from cartilage cells ofepiphyseal plate during childhood

Painless lesion until pathological fracture inthin cortex makes local injury

Best treated with curettement and bonegrafting


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Angioma of Bone

Hemangioma relatively common in manytissues

In bone, usually appears in vertebral bodies &skull

Rarely causes bone destruction


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Aneurysmal Bone Cyst

A solitary vascular abnormality within marrowtissues of cancellous bone

Most frequently in adolescents & young adults

Locally destructive, pain usually occure If untreated, may rupture and producing

hematoma Suitable treatment is curettement and bone

grafting Massive bleeding is an important risk Radiotherapy may adjunctive in sclerosing

blood vessels


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OSTEOSARCOMA

Classic (intramedullary) form highlymalignant tumour arising w/i the bone &spread rapidly periosteum &surrounding soft tissue

Predominantly in children & adolescent Most common at long-bone metaphysis

(knee & proximal end humerus


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Major sites of origin of osteosarcomas. The numbersare approximate percentages.


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OSTEOSARCOMA

Clinical featureEarly symptom pain :constant, worse atnight, severityLumpRarely pathological #

Physical exam : local tenderness(palpable mass, swollen & inflamed)Lab : ESR & Alkali Phosphatase


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OSTEOSARCOMA

X-rays: appearance variable hazy osteolytic area osteoblastic area

endosteal margin poorly defined cortex breached & tumour extend to adjacentsoft tissue

streaks of new bone, radiating outwards from

cortex sunburst effect reactive new bone form at angles of periostealelevation Codmans triangle


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Osteosarcoma

Radiographs of the primarytumor usually show a large,destructive, mixed lytic andblastic mass. The tumorfrequently breaks through

the cortex and lifts theperiosteum, resulting inreactive periosteal boneformation. The triangularshadow between the cortexand raised ends ofperiosteum is knownradiographically as Codman triangle and is characteristic,but not diagnostic of thistumor.

Distal femoral osteosarcoma with prominent boneformation extending into the soft tissues. Theperiosteum, which has been lifted, has laid down aproximal triangular shell of reactive bone known as aCodman triangle (arrow) .


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Central osteosarcoma. A, A destructive lesion is seen in the metaphysis on this anteroposterior

view of the knee in a young teenager with pain. B, A magnetic resonance scan of both legs showsthe soft tissue extent of the tumor (arrows).

Osteosarcoma


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Parosteal Sarcoma

Tends to afflict adolescents and young adults Most frequent site in distal femur Arise from osteoblastic cells Slowly growing compared to osteosarcoma Pathological fracture is rare Prognosis much better than osteosarcoma

Total resection by limb sparing or amputation canbe expected to result in a permanent cure in 80%of patients


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Benign Chondroblastoma

Rare & benign neoplasm Within epiphysis of distal femur, proximal

tibia of older children & adolescents Pain & disturbed function of nearby joint Respond well to local curettement and bone

grafting


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Chondromyxoid Fibroma

Frequently developed in metaphyseal regionof long bones and small bones ofadolescent and young adult

Malignant changes is infrequent Local excision is best treatment rather than

curettement


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MULTIPLE MYELOMA

Malignant B-cell lymphoproliferative disorder ofmarrow w/ plasma cell predominating

marrow cell proliferation & osteoclastic activity osteoporosis & discrete lytic lesions Large colony of plasma cell plasmacytoma Bone resorption hypercalcaemia thirst,

polyuria, abdominal pain Late feature: renaldysfunction, spinal cord / root compression


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MULTIPLE MYELOMA

Usual age: 45 -65 y.o. w/ weakness,backache, bone pain or pathological #

Hypercalcaemia thirst, polyuria &abdominal pain

Labs: mild anaemia, high ESR, creatinine

, hypercalcaemia, Bence-Jones protein(urine), marrow puncture plasmacytosis


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MULTIPLE MYELOMA

Treatment:Supportive corticosteroid (pain control) ,

correction of fluid balance & hypercalcaemia# internal fixation & MMASpecific therapy alkylating cytotoxic agents

Prognosis poor


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MULTIPLE MYELOMA

X-ray: generalizedosteoporosisclassic lesion : multiplepunch out defect w/soft margin (skull,pelvis, prox femur, acrushed vertebrae, orsolitary lytic tumour inlarge-bone metaphysis)


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EWINGS SARCOMA Arise from endothelial cells in bone

marrow Common between the age of 10 20

years Usually in tubular bone (tibia, fibula &

clavicle)

Presents w/ (throbbing) pain & swelling Generalized illness, pyrexia, warm, tenderswelling, ESR suggest osteomyelitis


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EWINGS SARCOMA X-ray: an area of bone

destruction (mid-diaphysis)new bone formation

extend along the shaft fusiform layer of

bone around the lesion onion -peel effect

also common sunrayappearance &Codmans triangle
http://c/Documents%20and%20Settings/aminardian/My%20Documents/EROS/orthooo/Saving_data/Oncology/Reticuloendothelial%20Bone%20Tumours_files/Ewings1.jpg


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EWINGS SARCOMA

Treatment: prognosis poorSurgery little effectRadiotherapy overall survival not enhancedChemotherapy 5-year survival = 50%Best combination pre-op chemotherapy,wide excision / amputation or radiotherapy w/local excision & chemotherapy for 1 year


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Reticulum Cell Sarcoma

Better prognoses than Ewings sarcoma

Mostly in adults Slower growing than Ewings Less pain than Ewings

More locally destructive More radiosensitive thanother neoplasms


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Giant Cell Tumor(Osteoclastoma)

Potentially malignant Arises in cancellous bone of long bones in

young adult

Commonest sites in distal radius, proximaltibia, distal femur 2/3 benign in behavior, 1/6 locally aggressive,

1/6 malignant

Hemorrhage within lesion is common Local pain and function disturbance of

articular is common Tends to recur after local surgical treatment


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Giant Cell Tumor(Osteoclastoma)

Treated wit complete excision andreplacement of resected part byautogenous bone graft, osteocartilaginousallograft or a custom made andoprosthesisincluding prosthetic joint replacement

Amputation is reasonable for aggressive

osteoclastoma


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TERIMA KASIH

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