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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    Tumor-Like Conditions

    Solitary Bone Cyst

    Aneurysmal Bone Cyst

    Metaphyseal Fibrous Defect

    Eosinophilic Granuloma

    Fibrous Dysplasia

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    Tumor-Like Conditions

    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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