Soft Tissue Sarcomas

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    Presented by: dr. Peri handayani

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    Adult soft tissue sarcomas rare

    Incidence 5/100.000/year in Europe

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    Soft Tissue Sarcomas (STS) ubiquitous in the siteof origin and often treated with multimodality

    treatment.

    Multidisciplinary approach pathologist, radiologist,

    surgeons, radiation therapists, medical oncologists,paediatric oncologists.

    All patients who suspected with sarcoma or with an

    unexplained deep mass of soft tissues, or with asuperficial lesion of soft tissues having a diameter of

    > 5 cm, or arising in paediatric age.

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    MR main imaging modality

    CT

    Multiple core needle biopsies

    Excisional biopsy lesions of < 5cm

    Open biopsy

    Histological diagnosis based on the latest WHO

    classification

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    The Federation Nationale des Centres de Lutte Contrele Camcer (FNCLCC) grading system > generally

    used based on differentiation, necrosis and mitotic

    rate.

    Tumour size and tumour depth recorded as a

    prognostic value

    Pathological report

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    The american joint committee on cancer(AJCC)/International Union against cancer (UICC)

    stage classification system important in

    malignancy grade in sarcoma

    In addition tumour size, depth and resectability

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    Surgical report contains of:

    The preoperative and intraoperative diagnosis

    The surgical conduct

    Surgical actual completeness vis--vis planned quality

    of margins

    A chest spiral CT scan

    Histological type and other clinical features

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    Limited disease

    Surgery wide excision with negative margins (R0)

    A marginal excision selected cases

    Wide excision + radiation th/ high grade, deep

    lesions, > 5 cm

    Radiation Th/ addition in

    The case of low grade, superficial, > 5cm

    Low grade, deep, < 5cm soft tissue sarcoma.

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    Radiation th/

    Dose 50-60 Gy

    Fractions of 1,8

    2 Gy

    Possibly with boosts up to 66 Gy

    Preoperatively 50 Gy

    Intraoperative radiation theraphy (IORT) and

    Brachytheraphyselected cases

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    R1 resections considered Re-operation

    R2 surgery re-operation is mandatory

    Adjuvant chemotheraphy

    not standard treatment in adult-type soft tissue sarcomas

    Proposed as an option to the high risk individual patient (

    >G1, deep, > 5cm tumour)

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    Extensive disease

    Metachronous resectable lung metastases without extra

    pulmonary diseases complete excision

    Chemotheraphy

    may be added as an option

    Extrapulmonary diseases standard treatment

    Anthracyclines first line treatment

    Anthracyclines + Ifosfamide trearment of choice

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    Angiosarcome taxanes are aan alternative option

    Patients who have already received chemotheraphy without

    ifosfamide option for giving high dose ifosfamide (-

    14g/m2)

    Trabectedin 2ndline option effective in

    leiomyosarcoma and liposarcome

    Gemcitabine + docetaxel 2ndline theraphy

    Dacarzabine 2ndline th/

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    Core needle biopsies the standard procedure

    Biopsies pathway should becarefull avoid

    contamination and complication

    Radiological imaging lipomatous tumours

    Postoperative radiation th/ Not feasible

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    Includes leiomyosarcomas, endometrial stromalsarcomas, undifferentiated endometrial sarcomas,

    pure heterologous sarcomas

    Total abdominal hysterectomystandard treatment

    Radiation th/ not recommended inleiomyosarcoma

    Adjuvant in selected cases

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    Treatment consist of:

    Surgery

    Radiation th/

    Observation

    Isolated limb perfussion

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    Systemic th/

    Hormonal th/ (tamoxifen, toremifene, GnRH analogues)

    NSAID

    Low dose chemotheraphy MTX + Vinblastine / MTX +

    vinorelbine

    Low dose interferon

    Imatinib

    Full dose chemotheraphy

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    Difficult anatomical loccation

    Radiation th/ widely resorted to

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    Breast conserving surgerydepending on thequality of margins versus the size of the tumour and

    the breast

    Angiosarcoma of the mammary gland tendency to

    recur that mastectomy is generally preferred

    Adjuvant chemotheraphy is concerned

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