angiosarcoamele pulmonare

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Angiosarcoamele Angiosarcoamele pulmonare pulmonare Adina Adina Boc Boc ănete ănete

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Angiosarcoamele pulmonareAdina Bocnete

Neoplasme rare, reprezinta 1% din totalul sarcoamelor de pri moi(incidena anual n SUA = 2-3cazuri / 1.000.000 loc.); Se pot localiza n esutul conjuntiv - 50%=cap, gt - Apoi - membrele inferioare - hepatic - sn - 2% = plmn

MorbiditateToate tind a fi agresive, deseori multicentrice. Cu rat mare de recuren, metastazare. Supravieuire la 5 ani < 20%. Nu rspund la tratament.

Factori de riscExpunere la diferite toxice(thorotrast, clorura de vinil, hormoni androgeni anabolizani, stilbestrol), radioterapie + ali carcinogeni: DACRON, proteze metalice, corpi strini metalici, plastic Status hormonal(sarcin, menopauz) = sn n limfedemul cronic = ulceraii, noduli / papule, violacee, echimoze aparent post traumatice.

ClinicMase(esuturi moi) ce cresc rapid, adenopatii rapid instalate. Compresiune pe ramurile nervoase(semne neurologice). 30% dintre pacieni prezint hemoragie(coagulopatie recent: anemie, hematoame persistente, hemotorax, ascit hemoragic, sngerri gastro-intestinale.

Pulmonarn general sunt tumori metastatice, exist i forme primitiv pulmonare. Punctul de plecare(ME) este cordul, cnd pacienii rmn mult timp asimptomatici sau prezint semne ce imit pericardita acut, emboliile pulmonare sau stenoza tricuspidian; pe locul 2 angiosarcoame primitive de sn.

ImagisticRgr. Pulmonar = 75% - noduli bilaterali de 0,5 3 cm; opaciti alveolare extinse, care n contextul hemoptizie + anemie pot evoca dg. de hemoragie intraalveolar; formele primitive au aspect identic; embolii cronice(sarcoame membre inf.). CT = leziuni nodulare solide cu chisturi cu perete subire.

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.

RMN = permite identificarea leziunii tumorale intracardiace primitive, sau n artera pulmonar(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 examinriLBA = permite identificarea hemoragiei intraalveolare(nr. siderofage) n absena hemoptiziei. Examinri de evaluare oncologic(cord, ficat, sn, piele, os) + bilanul extensiei(ficat, ganglioni limfatici) Ecografia transtoracic = sensibilitate sczut transesofagian

DiagnosticBiopsie transbronica toracoscopie deschisa pulmonar Anatomo-patologic = focare tumorale constituite de o poliferare de celule endoteliale maligne ntr-o reea anastomotica(imunohistochimie pentru factorul VIII, antigene CD31 i CD34).

Diagnosticul diferenial1) Imagistica pulmonar: -tumori pulmonare secundare(sn, digestive, rinichi) -hemoragii intraalveolare de alta etiologie: maladii sistemice infecii insuficien cardiac congestiv anomalii ale hemostazei

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

Tratamentul1) Chirurgical plmn, pleur, trahee(localizat). 2) -Chimioterapie = supravieuire 6-9 luni prin detres respiratorie(hemoragie intraalveolar). -Doxorubicin liposomal(PLD).