Angiosarcoamele Pulmonare

Angiosarcoamele pulmonare Adina Bocănete

¾ Neoplasme rare, reprezinta 1% din totalul sarcoamelor de părţi moi(incidenţa anuală în SUA = 2-3cazuri / 1.000.000 loc.); ¾ Se pot localiza în ţesutul conjuntiv - 50%=cap, gât - Apoi - membrele inferioare - hepatic - sân - 2% = plămân

Morbiditate ¾Toate tind a fi agresive, deseori multicentrice. ¾Cu rată mare de recurenţă, metastazare. ¾Supravieţuire la 5 ani < 20%. ¾Nu răspund la tratament.

Factori de risc ¾ Expunere la diferite toxice(thorotrast, clorura de vinil, hormoni androgeni anabolizanţi, stilbestrol), radioterapie + alţi carcinogeni: DACRON, proteze metalice, corpi străini metalici, plastic ¾ Status hormonal(sarcină, menopauză) = sân ¾ În limfedemul cronic = ulceraţii, noduli / papule, violacee, echimoze aparent post traumatice.

Clinic ¾Mase(ţesuturi moi) ce cresc rapid, adenopatii rapid instalate. ¾Compresiune pe ramurile nervoase(semne neurologice). ¾30% dintre pacienţi prezintă hemoragie(coagulopatie recentă: anemie, hematoame persistente, hemotorax, ascită hemoragică, sângerări gastro-intestinale.

Pulmonar ¾În general sunt tumori metastatice, există şi forme primitiv pulmonare. ¾Punctul de plecare(ME) este cordul, când pacienţii rămân mult timp asimptomatici sau prezintă semne ce imită pericardita acută, emboliile pulmonare sau stenoza tricuspidiană; pe locul 2 angiosarcoame primitive de sân.

Imagistică ¾Rgr. Pulmonară = 75% - noduli bilaterali de 0,5 – 3 cm; opacităţi alveolare extinse, care în contextul hemoptizie + anemie pot evoca dg. de hemoragie intraalveolară; formele primitive au aspect identic; embolii cronice(sarcoame membre inf.). ¾CT = leziuni nodulare solide cu chisturi cu perete subţire.

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

• Fig. 2A. —Metastatic angiosarcoma 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. —Metastatic angiosarcoma of lung in 61-year-old woman. Contrastenhanced CT scan (mediastinal window setting) shows slightly inhomogeneous enhancement. Note punctate calcification (arrow) in periphery of lesion.

• Fig. 3. —Metastatic angiosarcoma 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 lymphangitic spread of tumor cells. Poorly demarcated solid nodular lesions (arrowheads) accompanied by groundglass attenuation are seen in periphery of both lungs.

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

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

• Fig. 5A. —Metastatic angiosarcoma 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 thinwalled cyst (arrow) in left upper lobe. Irregular nodule (arrowhead) is also seen in right lower lobe.

• Fig. 5B. —Metastatic angiosarcoma 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.

¾RMN = permite identificarea leziunii tumorale intracardiace primitive, sau în artera 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 atrial mass (arrow). (c) Coronal T1weighted 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 atrial mass (arrow). (c) Coronal T1weighted 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 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 atrial mass (arrow). (c) Coronal T1weighted 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).

Alte examinări ¾LBA = permite identificarea hemoragiei intraalveolare(nr. siderofage) în absenţa hemoptiziei. ¾Examinări de evaluare oncologică(cord, ficat, sân, piele, os) + bilanţul extensiei(ficat, ganglioni limfatici) ¾Ecografia transtoracică = sensibilitate scăzută transesofagiană

Diagnostic ¾Biopsie transbronşica toracoscopie deschisa pulmonară ¾Anatomo-patologic = focare tumorale constituite de o poliferare de celule endoteliale maligne într-o reţea anastomotica(imunohistochimie pentru factorul VIII, antigene CD31 şi CD34).

Diagnosticul diferenţial ¾ 1) Imagistica pulmonară: -tumori pulmonare secundare(sân, digestive, rinichi) -hemoragii intraalveolare de alta etiologie: )maladii sistemice )infecţii )insuficienţă cardiacă congestivă )anomalii ale hemostazei

¾2) Sarcomul de Arteră Pulmonară = opacitate hilară + semne de HTP + transembolism cronic. pulmonar(leiomiosarcoame, fibrosarcoame); lacuna pe angiografie, angioscanner, RMN. ¾3) Hemangioteliomul difuz. ¾4) Alte tumori primitive ale inimii drepte. secundare

Tratamentul ¾1) Chirurgical – plămân, pleură, trahee(localizat). ¾2) -Chimioterapie = supravieţuire 6-9 luni prin detresă respiratorie(hemoragie intraalveolară). -Doxorubicină liposomală(PLD).