Sarcoame Osoase & Parti moi

Tumori osoase:TO

Epidemiologie & Eti 13313d314n ologie (1) :

• incidenta: crescuta in adolescenta (3/100,000)
• incidenta specifica:
• mielom multiplu: 35-45% din tu os !: varsta medie & adulti in varsta (peak incidentei 5th-7th decada)
• osteoS: cea mai frecventa tu os: adolescent, adulti tineri; rar dupa RT, B Paget.
• chondroS: 13%; primara sau secundar dezvoltat pe lez benigne preexistente (enchondroma, Ollier's dis, osteoch., exostoza ereditara multipla : risk 15-25%)

Epidemiologie & Eti 13313d314n ologie (2):

• incidenta specifica:
• fibrosarcom: < 4%
• "tum cu celule rotunde": sarcom Ewing (7%), neuroblastom metastatic
• tum cu cel gigante: in meta-/epifize os lungi,adulti tineri, 4.5% din tum os;
• metastaze: 60-65%; frecvent : pelvis
• picior & mana: plaman
• san, prostata, rinichi, tiroida

Etiologie :

• B Paget's (GCT, osteoS), hyperparatiroidism (brown T), osteomielita cr (sq cell Ca, osteo S), fractura
• RT (externa/interna; occupational vs medical): osteogenic S, chondroS, fibroS
• agenti infectiosi, traumatisme: ?
• markeri cytogenetici in sarcom Ewing: t(11:22)

Clinic:

• durere locala, in leg cu activ fizica , devine permanenta
• modificarea reg anatomice, fractura patologica
• systemic/constitutional simptome: scadere in G, febra, transpiratii nocturne
• anamnestic: RT

Proceduri diagnostice:

• radiografii osoase: osteoliza, osteoscleroza sau lez mixte, reactie periostala, distructie corticala
• CT & RMN apreciaza extinderea os si extracompartimentala a tu
• Angiografie - relatia cu marile vase
• Rgr pulm : meta pulm
• Scinti os: meta os
• Lab fosf: alc crescuta
• Biopsie os : confirma dg

TO: Benign vs Malign

TO: Clasificarea TO

Chondrosarcom

b)humerus dr proximal, distructie corticala, extensie in parti moi

Tumora cu celule gigante

Ewing's sarcoma:

TO: Stadializare

TO: Principii de tratament

T maligne: tratament multidisciplinar in fct de tipul tumoral

• S grad (G) scazut: chirurgie cu MR -
• osteoS G inalt : CH + chimio adj
• Ewing's S: RTE + chimioterapie

Chimioterapia

• tratament primar pt osteo S, S Ewing metastatic, nerezecabil
• chimio neoadjuvant (preop)
• chimio adjuvanta
• med active & combinatii:
• doxorubicin, HD-MTX, DDP, IFO, CTX
• DOX, HDMTX/LV; DOX, DDP

RTE:

• mielom, limfoame

Rezultate terapeutice:

• 17-23% CH solo vs 35-64% trat multidisciplinar

Follow-up:

• metastaze pulm: 90% risk in primii 2 ani
• monitorizare: X ray, alk Ph

Sarcoame parti moi - SPM:

Epidemiologie & Eti 13313d314n ologie:

• incidenta: aprox 1% TM , copil + adolescent
• mortalit: 50%

Etiologie: radiatii ionizante, predispozitie genetica (retinoblastom, sdr Li-Fraumeni), fact chimici (clorura vinil, arsenic, acizi fenoxiacetici), limfedem cronic

Origine:

Muschi striat = rabdomiosarcom

T adipos = liposarcom

T fibros = fibrosarcom

Pereti vasculari = hemangiosarcom, sarcom Kaposi

Limfatic = limfangisarcom

T sinovial = sarcom sinovial

Fibroblast , histiocit = histiocitom fibros malign

Histologia & Incidenta

Sarcoame parti moi

Clinic : masa tum , dimensiuni crescute, semne de compresie vasculo-nervoasa, semne date de prezenta metastazelor

Proceduri diagnostice:

• imagistica: CT, echo, RMN
• biopsie

SPM: Fibrosarcom

SPM: Histiocitom fibros malign

• pseudocapsula fibroasa

Histologia:

• factor important de prognostic : gradul histologic!
• AJC vs MTS: G1-3 (bine, moderat, rau diferentiat) vs G1: low-grade & G2: high grade

• numarul mitozelor, prezenta necrozei, pleomorfismul nuclear, neovascularizatia, invazia vasculara,aneuploidia nucleara

Extindere:

• locala: de-a lungul planurilor fasciale musculare cu realiz unei pseudocapsule cu prelungiri digitiforme ale procesului tu

• limfatic: rar

• hematogen: 90% in primii 2 ani, in special plaman

SPM: Stadializare

• T : T1 vs T2 = <5 vs >5 cm
• N : N0 vs N1
• N1 M1

SPM: Stadializare

Liposarcom antebrat

Sarcom Kaposi

limphangiosarcom postmastectomie: Stewart-Treevs' syndr.

• interval de timp: 0 - 6 luni - 11 luni

SPM : Principii de tratament

Obiective: control local & distanta

Chirurgia:

• rezectia tu primare cu MR -
• disectia LN
• rezectia M: ±
• chirurgia de salvare

Radioterapia:

• obiective:

reduce morbiditatea functionala & cosmetica care apare postchirurgical

• doza: 65 Gy

• modalitati: pre-, post-, intraoperator, singura, interstitiala

Chimioterapia:

• control la distanta al micro/macro metastazelor efect sinergic local cu RT
• chimioterapice: doxorubicin, dacarbazin, ifosfamida
• regimuri: CYVADIC = OR 50%, CR 17%, MAID

SPM: Rezultate ale tratamentului

Massachusetts General Hosp (Surg&RT)