Kuliah Tumor Ortho

8/10/2019 Kuliah Tumor Ortho

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TUMORIGENESIS :

SELF-SUFFICIENCY IN GROWTH SIGNALS

INSENSITIVITY TO GROWTH-INHIBITORY

SIGNALS

EVASION OF PROGRAMMED CELL DEATH LIMITLESS REPLICATIVE POTENTIAL

SUSTAINED ANGIOGENESIS

TISSUE INVASION AND METASTASIS


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Rare - 0.5% of cancer deaths.

40% Malignant.

Primary & Secondary/metastatic.

Primary in Young. (Osteosarcoma)

Secondary in the old. (Breast, Kidney, thyroid,lung, prostate)

Marrow neoplasms(hemopoietic) myeloma,leukemia, lymphoma etc.


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Anatomic extent of the lesion

Degree of malignancy

Potential for development of metastatic disease


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Prognostic factors

Surgical margin planning Guiding for adjunctive treatment


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The Musculoskeletal Tumor Society (MSTS)

The American Joint Committee on Cancer (AJC)Memorial Sloan-Kettering Cancer Center


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Grade (G)

Site (T)

Metastasis (M)


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G0 = Benign

G1 = Low-grade Malignant

G2 = High-grade Malignant


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T0

= Intracompartment (true capsule)

T1 = Intracompartment (no true capsule)

T2 = Extracompartment


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M0 = Absence

M1 = Presence

Organ, Lymph node or Skip lesion


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Stage 1Latent Benign (G0, T0, M0)


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Stage 2Active Benign (G0,T0, M0)


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Stage 3

Aggressive Benign (G0, T1-2, M0-1)


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Stage IA(G1, T1, M0)


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Stage IB(G1, T2, M0)


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Stage IIA(G2, T1, M0)


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Stage IIB(G2, T2, M0)


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Stage III(G1-2, T1-2, M1)


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Primary bone tumors, unlike most other tumors, maybe difficu

to diagnose based on histology The radiology and clinical features are essential componentsof diagnosis.

A good history is important and very helpful

Important factors:

Age Presentation (mass, pain, paraesthesia, trauma)

Any known malignancy

Sex.


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When needed biopsy, should be the last step in the diagnosticworkup

Biopsy often done by open (surgical) or by FNAB (with/without

CT guidance)


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First decide: lytic or blastic Lytic: Hole in the bone

Blastic: Area that too dense or white

Some lesions are a combination (mixed)


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For most bone tumors plain x-rays offer the mostimportant information about the diagnosis.

X-ray shows how the bone is reacting to the tumor,

and how the tumor is reacting the bone

CT, MRI and bone scans mostly useful in staging


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Location within the bone is also as a factor,

since certain tumors prefer the diaphysis,

metaphysis, or epiphysis

The metaphyseal location is the least helpful, sinceit has a rich blood supply, all etiologies have a

predilection for metaphysis.

Central, eccentric, cortical or bone surface.


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Most primary malignant bone tumors in youngpeople arise in areas of rapid growth such as thedistal femur, proximal tibia, prox. humerus.

Some primary tumors have a predilection for

certain locations. Most metastatic lesions occur in regions that

contain haematopoetic marrow: axial skeleton,proximal extremities


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Primary bone tumors are much less common than metastatictumor.

A hole in the bone is most often due to metastatic disease.


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How the bone reacts to the tumor providesimportant clues to the behavior of the tumor such

as the rate of growth.

- Pattern of bone destruction and pattern of

bone

response to the lesion gives a sense of the rate

of

growth.

- Extension through the cortex/ associated soft


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The tumor margin (margin of the hole in the bone) canbe sharp or fuzzy / illdefined.

Sharp is least aggressive (especially if scleroticrim).Fuzzy is more aggressive,and no clear borderbetween tumor and normal bone is the most

aggressive. Terminology: geographic = focal and well circumscribed(the least aggressive,especially if sharp and scleroticmargin).

Motheaten / permeative = multiple holes ( the most

aggressive).


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Diagnosis should include a thorough radiologicwork up in conjunction with a multidisciplinaryteam that will be providing the definitive care.

Diagnosis for many bone tumors is made by

radiographic features alone.- Pathologic findings may be misleading !

When reguired,a biopsy should be the last,notthe first step in the diagnostic process.


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Next a whole body bone scan should be obtained.This is oftenthe best way to look for other bone lesions.Skeletal survey is

sometimes best for purely lytic / lucent lesions ( e.g...myeloma

sometimes).

If there are multiple lesions the diagnosis will almost always be

metastatic disease.


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Bone-Forming Tumors

Cartilage-Forming Tumors

Giant Cell Tumor

Benign Vascular Tumors

Tumor-Like Conditions


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Bone-Forming Tumors

A. Osteoma

B. Osteoid Osteoma

C. Osteoblastoma


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Cartilage-Forming Tumors

A. ChondromaB. Osteochondroma

C. Chondroblastoma

D. Chondromyxoid Fibroma


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Benign, Any age Single or multiple sites

Often involves small bones of hands and feet.

Well demarcated, mature cartilage.


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Hereditarymultiple enchondromatosis. Usually over oneside of the body. (Olliersdisease).

Maffucci'ssyndrome - multiple bone chondromas and

hemangiomas of soft tissue

Increased risk for chondrosarcoma


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Osteochondroma


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Benign Vascular Tumors

HemangiomaLymphangioma

Glomus Tumor


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Other Benign Connective TissueTumors

Desmoplastic FibromaFibrous Histiocytoma

Lipoma

NeulilemomaNeurofibroma


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Solitary Bone Cyst

Aneurysmal Bone Cyst

Metaphyseal Fibrous Defect

Eosinophilic Granuloma

Fibrous Dysplasia


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Osteofibrous Dysplasia

Myositis Ossificans

Brown Tumor of Hyperparathyroidism

Intraosseous Epidermoid Cyst

Giant Cell (Reparative) Granuloma


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Osteosarcoma

Chondrosarcoma

Malignant Fibrous Histiocytoma

Adamantinoma

Chordoma


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Common primary cancer of bone

Young adults - 10 and 25 years

Rare in later ageSecondary to previous irradiation

or Pagets disease genetic (retinoblastoma gene)

Metaphysis of a long bone (Knee)

Tenderness / pain / Mass.


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Malignant mesenchymal cells that produce

Irregular lace like osteoid matrix.

May or may not be calcified.

pre-operative chemotherapy with surgicalresection.

The five-year survival ~ 60%


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Next common to Osteosarcoma.

Older adults 30 to 60 years.

Location - axial skeleton (pelvis & pectoral girdles, ribs & spine)

Aggressive, erodes & invades soft tissue,

Metastases to lungs, liver, kidney & brain.


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Malignant cartilage with anaplastic chondrocytes inspaces with focal enchondral ossification andcalcification

Resistant to chemo Surgical resection

Grade I tumors have 5-year survival rates of 90%, whilehigh grade tumors have poor prognosis.

Clear cell chondrosarcoma is a histologic variant that isassociated with a better prognosis.


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10-25 years of age

affects long bones

sensitive tochemotherapy

>40 years of age

affects axial skeleton

not sensitive tochemotherapy


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Adults

Lymphoma

MMPlasmacytoma

Children

HistiocytomaEwings Sarcoma


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Breast

Prostate

Lung

Kidney

Thyroid


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Psychologic Selecting forms of Tx. consider life expectancy (Px)

Benignsurgical

Malignantsurgical ablation/eradication with or

without Rx & adjuvant chemotx. Consider limb salvageprocedure

Radiotx , (Ewing, Retic. Cell Sa.)

Adjuvant syst. Chemotx. (OsteoSa)

ImmunoTx ?


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Stage 1

Observation or Simple Curettage

Stage 2

Extended Curettage


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Stage 3

Extended Curettage, Excision-

Curettage or Marginal or Wide

Excision


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The Principle Treatment of MostBenign Bone Tumors

Curettes

Modest-sized Bone Windows

Bone Graft / Bone Cement

+ Adjuvant Agent


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Stage 1

Large Window

Curette and High-Speed

Blur


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Stage 2 and 3

Large Window

Curette and High-Speed Blur

Adjuvant Agents: Phenol,

Liquid Nitrogen


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Stage 3

En Bloc Resection Curettage of the Inner

Portion

Need Reconstruction


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Curettage

Benign Bone Tumors; Stage 1and 2

Extended Curettage

Benign Bone Tumors; Stage 3


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Noninfiltrating Benign Soft Tissue

Tumors

Benign Bone Tumors Stage 3

and Adjuvant Agent


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Recurrent Stage 3 Benign BoneTumors


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Intra-articularExtra-articular


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Cancellous Autograft

Cancellous Allograft Bone Substitutes

Polymethylmethacrylate


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