Sarcoma short note by S.

Wichien (SNG KKU)

Sarcoma arise from embryonic mesoderm Incidence Adult (<1% of ca) 1.MFH 28% 2.leiomyosarcoma 12% 3.synovial sarcoma 10% 4.malig n sheath tumor 6% Children (7% of ca) 1.rhabdomyosarcoma Risk factor 1.XRT--50x,10yr 2.occu chemical -phenoxyacetic acid,chlorophenol (soft tissue sarcoma) -vinyl chloride,arsenic,thorium oxide (hepatic angiosarcoma) 3.trauma 4.chronic lymphedema--ALND,filaria Genetic Hereditary synd -rerinoblastoma -Li Fraumeni synd -Nurofibromatosis type 1 -FAP Oncogene -MDM2, M-myc, c-erbB2 Tumor suppressor gene -p53, Rb, NF-1 mutation Clinical -asymp mass--most -obstruc symp--less Imaging 1.local extent 2.stage malignant dz 3.assist percu.bx 4.dx low gr/benign or hi gr/malig CT -retroperitoneal sarcoma -myxoid liposarc of ext--abdo.metas :should CT abdomen MRI -extremity sarcoma Biopsy technic 1.FNA -r/o & confirm metas or local recur (procedure of choice) -not in grading for Tx planning 2.CNB 3.incisional bx -deep tumor , superficial >3cm -non-dx in 1,2 -most reliable methods 4.excisional bx -superficial <3cm Patho Limited metas -desmoid -atyp.lipomatous (well diff liposarc) -dermatofibrosarc.protuberan Metas -myxoid liposarcoma -chrondosarcoma -angiosarcoma -leiomyosarcoma -rhabdomyosarcoma -synovial sarcoma Staging T1a <=5cm superficial tumor T1b <=5cm deep tumor T2a >5cm superficial tumor T2b >5cm deep tumor N1 regional node metas M1 distant metas Histo gr1 well diff gr2 mod diff gr3 poorly diff 1a 1b 2a 2b 3 4 T1 T2a T1 T2a T2b any n0 n0 n0 n0 n0/1 any m0 m0 m0 m0 m0 m1 g1 g1 g2,3 g2,3 g2,3 any

histo gr=most important prog factor M=lung(most com),bone,liver,brain Ki-67,s100=proliferation marker

Sarcoma short note by S.Wichien (SNG KKU)

Retroperitoneal sarcoma 5 yr survival 36-58% sarcomatosis--diffuse in peritoneum 1.liposarcoma 2.MFH,leiomyosarcoma Clinical -large mass, 1/2 > 20cm at dx -compress adjacent structure -Ix--LDH(lympho),b-HCG(germ cell) Rate of death -tumor stage -high grade -unresectable -gross +ve margin Tx 1.complete sx resection -en bloc resection -not offer unless Ix indicate 2.XRT hi-risk pt-->preop XRT 50 Gy -large,hi-grade tumor -recurrent low grade tumor 3.CMT -study show fail survival for adju CMT -may advocate for uncom.histo :dediff liposarc,MFH,PNET GI sarcoma Clinical -non specific GI symptom -GI bleed -abdo.mass Ix -large intra abdo.mass -no evidencof LN metas :not 1lymphatic metas Tx -margin 2-4 cm -small bowel-->segmental resection -not attempt radical mesen LND GIST -interstitial cell of cajal (intes pacemaker cell) c-Kit gene (CD117) -exon 11 -- better prognosis -exon 9 -- wild type Kit PET -pre op staging -detect early metastasis -baseline metabolic activity Tx 1.localized dz -complete resection w neg margin -extent of sx not influence survival -risk stratification--for adjuvant Tx 2.locally advance / metastasis 1.imatinib (Gleevec) -400,800 mg/d -s/e:GI toxic,periorbital edema,cramp 2.sunitinib (Sutent) -unable tolerate imatinib imatinib refractory GIST -50 mg/d*4wk--stop 2wk

Sarcoma short note by S.Wichien (SNG KKU)

Extremity sarcoma Tx stage1--sx resection Stage2--sx resection +/- XRT Stage3--sx resection,XRT +/- CMT Stage4--CMT +/- sx resection Recommendation -T>3cm-->should imaging+tissue dx -T1,low grade-->WLE 2cm margin -T2,hi grade-->should metas w/u -T2-->XRT -recur/distant-->CMT -isolate recur/resect metas-->sx Sx 1.WLE -1-2 cm margin Clinical/imaging suspected LN+ve -should confirm before RLND-->FNAB 2.amputation -in can't gross resect /preserve fxn 3.isolated regional perfusion 1.limb sparing alternative Tx 2.palliative Tx/local control Limb perfusion -thigh--ext illiac vv calf--femoral/popliteal vv upper ext--axillary vv -ligate collateral vv -connect to pump oxygenator -touniquet/Esmarch band -hyperthemia--40c Chemotherapeutic agent -doxorubicin 4.sx resection for metas sarcoma -lung--most common -<4 nodule,no endobronchial invasion Other sarcoma Breast sarcoma -rare, <1% of ca Breast -angiosarc,stromal sarc,fibrosarc,MFH Angiosarcoma -1/2 of breast sarcoma -asso XRT (3-20 yr) -expanding erythema patch -red papular euption -bluish black lesion -bluise like discolor on indurate area -MMG--nonspecific -dx--punch/incision bx Tx -completely excision w -ve margin -not routine ALND -XRT,CMT in large/hi risk Cystosarcoma phyllodes *not be sarcoma (from hormone rxn stromal cell) Uterine sarcoma -<5% uterine ca -local dz--TAH 3 subgroup 1.endometrial sarcoma -expres progesterone receptor -adjunct hormone Tx 2.leiomyosarcoma -unresect=gemcitabine+docetaxel 3.mixed mullerian tumor Desmoid -low grade sarcoma -not metastasis -1/2=ext, 1/2=trunk,retroperitonium -Gardner synd Tx -local excision -XRT reduce recurrence DFSP -dermatofibrosarcoma protuberans -chromosome translocation 17,22 -rare, low grade sarcoma -trunk -cutaneous mass -slow growth Tx -wide local excision -local recurrent <10%