SOFT TISSUE SARCOMAS

• AETIOLOGY : UNKNOWN

• High-risk factor :

- Neurofibromatosis ( Von Recklinghausen`s disease)

- Previous radiation therapy

- Long-lasting lymphoedema

Pathological appearance

• Originate from all mesenchymal tissues ( fat, muscular, connective tissue, vessels, peripheral nerves, tendon and synovial

• Benign tumor(- omas), malignant tumor (- sarcomas) - Connective tissue : fibroma- fibrosarcoma - Fat tissue : lipoma – liposarcoma - Striated muscle : rhabdomyoma- rhabdomyosarcoma - Smooth muscle : leiomyoma- leiomyosarcoma - Blood vessel : haemangioma-haemangiosarcoma - Lymph vessel : lymphangioma-lymphangIosarcoma

Grade of malignancy : grade I,II,III,IV ( well-moderate, poorly and undifferentiated)

Biological behaviour

• Benign soft tissue tumour grow non-invasive but expansive, do not metastases

• Malignant soft tissue tumour grow expansive and invasive, frequently metastases

• Expansive growth lead to compression surrounding tissue and make pseudocapsules

• Sarcoma nearly always spread hematogeneously,mainly to the lung, only occasionally to lymphnode (synoviosarcoma, rhabdomyosarcoma, fibrosarcoma, angiosarcoma)

Diagnostic approach

Physical examination :

• Inspection : compare the two side symmetry?, atrophic / contracture muscle, colour of the skin, functional disorder

• Palpation : skin temperature, swelling sharply demarcated, ill-defined, size, fixation to surround tissue, painful, neurological disorder

Microscopic diagnostics

• Incisional biopsy : standard procedure to accurate diagnostic

• FNAB : not establish and not standard, indication for tumour recurrent, tumour metastases

• Immuno-histochemistry : golden standard to mention accurate diagnostic

Staging tumour

According to TNM-G system

T 1 : tumour < 5 cm, T1a : superficial tum or T1b : deep tumor

T 2 : tumour > 5 cm, T2a and T2b ( superficial and deep)

No : no regional metastasesN1 : positive regional lymphnode metastases

M0 : No distant metastasesM1 : Positive distant metastases

G1,2,3,4 (well,moderate,poorly,undiffer)

Stage 1A : G1-2, T1a or T1b ,N0,M0 ( low grade, small, superficial ,deep)Stage IB : G 1-2, T2a , No,MO (low grade, large,superficial, Stage IIA : G!-2, T2b, No,MO ( low grade,large,deepStage IIB : G3-4, T1-b,NO,MO ( high grade,small superf, or deep)Stage IIC : G3-4, T2a, No,MO ( high grade, large, superficial tumorStage III : G3-4, T2b, No,Mo ( high grade, large, deep tumor)Stage IV : any G,Any T,No,Ni,Mo,Mi (any meta lymphnode,distant metastases)

Treatment of STS

• Local treatment : standart therapy

- Wide excision ( compartment procedure,

amputation, safety margin 2 cm )

-Radiotherapy• Systemic treatment : adjuvant therapy

Chemotherapy adjuvant (cyclophosphamide, ifosphamid, adriamycin, dacarbacin, vincristin, taxane, Growth factor /VEGF

Prognosis of STS

• Prognostic factors : - Type of tumor - Grade of malignancy - Size of tumor - Location - Metastases - Age of patient - Recurrence