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Sarcoame: Osoase & Parti moi

medicina


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

Type

%

Fibrosarcoma

Liposarcoma

Rhabdomyosarcoma




Malignant fibrous histiocytoma

Synovial sarcoma

Neurofibrosarcoma

Leiomyosarcoma

Angiosarcoma

Other

22.6

19.2

12.8

8.5

7.5

5

4.9

1.6

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

Histology

% LN+

Synovial S

RhabdomioS

LipoS

FibroS

NeurofibroS

17.0

12.2

5.7

5.1

0

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)

AJC Stage

% 5 yrs

local control

% 5 DFS

IA

IB

IIA

IIB

IIIA

IIIB

Total

100

93

88

85

93

79

88

100

88

83

52

87

39

66













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