AngiosarcoameleAngiosarcoamelepulmonarepulmonare

AdinaAdina BocBocăneteănete

Neoplasme rare, reprezinta 1% din totalulsarcoamelor de părţi moi(incidenţa anuală înSUA = 2-3cazuri / 1.000.000 loc.);Se pot localiza în ţesutul conjuntiv

- 50%=cap, gât- Apoi - membrele inferioare

- hepatic- sân

- 2% = plămân

MorbiditateMorbiditate

Toate tind a fi agresive, deseorimulticentrice.Cu rată mare de recurenţă, metastazare.Supravieţuire la 5 ani < 20%.Nu răspund la tratament.

FactoriFactori de de riscrisc

Expunere la diferite toxice(thorotrast, clorura de vinil, hormoni androgeni anabolizanţi, stilbestrol), radioterapie + alţi carcinogeni: DACRON, proteze metalice, corpi străinimetalici, plasticStatus hormonal(sarcină, menopauză) = sânÎn limfedemul cronic = ulceraţii, noduli / papule, violacee, echimoze aparent post traumatice.

ClinicClinicMase(ţesuturi moi) ce cresc rapid, adenopatii rapid instalate.Compresiune pe ramurile nervoase(semneneurologice).30% dintre pacienţi prezintăhemoragie(coagulopatie recentă: anemie, hematoame persistente, hemotorax, ascităhemoragică, sângerări gastro-intestinale.

PulmonarPulmonar

În general sunt tumori metastatice, existăşi forme primitiv pulmonare.Punctul de plecare(ME) este cordul, cândpacienţii rămân mult timp asimptomaticisau prezintă semne ce imită pericarditaacută, emboliile pulmonare sau stenozatricuspidiană; pe locul 2 angiosarcoameprimitive de sân.

ImagisticImagisticăă

Rgr. Pulmonară = 75% - noduli bilateralide 0,5 – 3 cm; opacităţi alveolare extinse, care în contextul hemoptizie + anemie pot evoca dg. de hemoragie intraalveolară; formele primitive au aspect identic; emboliicronice(sarcoame membre inf.). CT = leziuni nodulare solide cu chisturi cu perete subţire.

• Fig. 1B. —Metastaticangiosarcoma of lung in 32-year-old woman. Photomicrograph of histopathologicspecimen taken at autopsy shows atypical endothelial cells involving lung parenchyma mixed with marked hemorrhage. (H and E, x200)

• Fig. 2A. —Metastaticangiosarcoma of lung in 61-year-old woman. Thin-section CT scan (lung window setting) shows solid nodular lesion (arrow) with irregular contour in right lower lobe.

• Fig. 2B. —Metastaticangiosarcoma of lung in 61-year-old woman. Contrast-enhanced CT scan (mediastinal window setting) shows slightly inhomogeneous enhancement. Note punctate calcification (arrow) in periphery of lesion.

• Fig. 3. —Metastaticangiosarcoma of lung in 48-year-old woman. Chest CT scan shows multiple solid nodular lesions and ground-glass attenuation. Note septal thickening (arrows) throughout lung, suggesting lymphangiticspread of tumor cells. Poorly demarcated solid nodular lesions (arrowheads) accompanied by ground-glass attenuation are seen in periphery of both lungs.

• Fig. 4A. —Metastaticangiosarcoma of lung in 78-year-old man. Chest CT scan (lung window setting) shows multiple thin-walled cysts (arrows) with ground-glass attenuation (arrowheads). Air–fluid levels are seen in several cysts.

• Fig. 4B. —Metastaticangiosarcoma of lung in 78-year-old man. Photograph taken at autopsy of gross histopathologicspecimen of right lung shows multiple cystic tumors with marked hemorrhage.

• Fig. 5A. —Metastaticangiosarcoma of lung in 60-year-old woman with history of mastectomy of left breast for cancer approximately 10 years previously. Chest CT scan (lung window setting) shows thin-walled cyst (arrow) in left upper lobe. Irregular nodule (arrowhead) is also seen in right lower lobe.

• Fig. 5B. —Metastaticangiosarcoma of lung in 60-year-old woman with history of mastectomy of left breast for cancer approximately 10 years previously. Chest CT scan 10 months later exhibits right hemothorax and enlargement of cyst. Areas of ground-glass attenuation have enlarged diffusely in both lungs. Thickened cyst wall and surrounding hemorrhage (arrows) is noted. Irregular nodule (arrowhead) in right lower lobe has enlarged.

RMN = permite identificarea leziuniitumorale intracardiace primitive, sau înartera pulmonară(trunchi / ramuri).

• Figure 1a. Cardiac angiosarcoma. (a) Axial contrast-enhanced CT scan shows a low-attenuation, lobulated right atrial mass (arrow). (b) Axial T1-weighted MR image obtained with gadolinium shows nodular enhancement of the right atrialmass (arrow). (c) Coronal T1-weighted MR image obtained without gadolinium shows the mass (arrow) arising from the free wall of the right atrium. The mass is slightly hyperintense relative to myocardium. (d) Right coronary angiogram shows tumor blush (arrow).

• Figure 1b. Cardiac angiosarcoma. (a) Axial contrast-enhanced CT scan shows a low-attenuation, lobulated right atrial mass (arrow). (b) Axial T1-weighted MR image obtained with gadolinium shows nodular enhancement of the right atrialmass (arrow). (c) Coronal T1-weighted MR image obtained without gadolinium shows the mass (arrow) arising from the free wall of the right atrium. The mass is slightly hyperintenserelative to myocardium. (d)Right coronary angiogram shows tumor blush (arrow).

• Figure 1c. Cardiac angiosarcoma. (a) Axial contrast-enhanced CT scan shows a low-attenuation, lobulated right atrial mass (arrow). (b) Axial T1-weighted MR image obtained with gadolinium shows nodular enhancement of the right atrialmass (arrow). (c) Coronal T1-weighted MR image obtained without gadolinium shows the mass (arrow) arising from the free wall of the right atrium. The mass is slightly hyperintenserelative to myocardium. (d)Right coronary angiogram shows tumor blush (arrow).

AlteAlte examinexaminăriări

LBA = permite identificarea hemoragieiintraalveolare(nr. siderofage) în absenţahemoptiziei.Examinări de evaluare oncologică(cord, ficat, sân, piele, os) + bilanţulextensiei(ficat, ganglioni limfatici)Ecografia transtoracică = sensibilitate

scăzutătransesofagiană

DiagnosticDiagnostic

Biopsie transbronşicatoracoscopiedeschisa pulmonară

Anatomo-patologic = focare tumoraleconstituite de o poliferare de celuleendoteliale maligne într-o reţeaanastomotica(imunohistochimie pentrufactorul VIII, antigene CD31 şi CD34).

DiagnosticulDiagnosticul diferendiferenţţialial

1) Imagistica pulmonară:-tumori pulmonare secundare(sân, digestive,

rinichi)-hemoragii intraalveolare de alta etiologie:

maladii sistemiceinfecţiiinsuficienţă cardiacă congestivăanomalii ale hemostazei

2) Sarcomul de Arteră Pulmonară = opacitate hilară + semne de HTP + transembolism cronic. pulmonar(leiomiosarcoame, fibrosarcoame); lacuna pe angiografie, angioscanner, RMN.3) Hemangioteliomul difuz.4) Alte tumori primitive ale inimii drepte.

secundare

TratamentulTratamentul

1) Chirurgical – plămân, pleură, trahee(localizat).2) -Chimioterapie = supravieţuire 6-9 luniprin detresă respiratorie(hemoragieintraalveolară).

-Doxorubicină liposomală(PLD).