Südame tuumorite radioloogiline diagnostika. Maksim Zagura II aasta resident
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1 Südame tuumorite radioloogiline diagnostika Maksim Zagura II aasta resident
2 Sissejuhatus Südame primaarsed tuumorid esinevad 0,2%-l elanikkonnast 12% südame primaarsetest tuumoritest avastatakse juhuslikult meditsiinilise läbivaatuse käigus Kliiniline pilt on mittespetsiifiline: väsimus, hingeldus, õhupuudus, valu rinnus, rütmihäired, sünkoop, subfebriilne palavik Dellamore et al. J Chir 2013; 34:
3 Südame tuumorid 2% 98% Primaarsed tuumorid 75% Beniigsed tuumorid Metastaasid 25% Maliigsed tuumorid
4 Radioloogilised uurismeetodid Röntgenograafia Kardiomegaalia Ebatavaline südame kontuur Kopsupais Transtorakaalne ehhokardiograafia Esmane hindamine tuumori kahtluse korral Transösofageaalne ehhokardiograafia Tuumori täpsemaks hindamiseks
5 Radioloogilised uurismeetodid KT Tuumori suurus ja lokalisatsioon Ekstrakardiaalsete struktuuride haaratus MRT Anatoomiline suhe südamekambritesse ja müokardi; perikardi ja külgnevate struktuuride haaratuse hindamine Tuumorite diferentsiaaldiagnostika PET Kaugmetastaaside diagnoosimine
6 Südame healoomulised kasvajad Müksoom Papillaarne fibroelastoom Lipoom Rabdomüoom
7 Müksoom Moodustab ~50% südame primaarsetest tuumoritest Esineb naistel kaks korda sagedamini kui meestel Seotud geneetiliste sündroomidega: Carney sündroom (müksoomid südames ja nahal, pigmentneevused, endokriinhäired, testiste tuumorid) LAMB sündroom (lentigines, atrial myxomas, mucocutaneous myxomas and blue nevi) Shin et al. Acta Radiol 2014; 55:
8 Müksoom Kliiniline pilt: õhupuudus (75%), subfebriilne palavik (35%), kaalulangus (25%), pearinglus (20%), sünkoop (10%) Keskmine läbimõõt: 2 4 cm KT tihedus 9 28 HU, esineb vähene kontrasteerumine Heterogeense struktuuriga: müksoidkude, nekroos, tsüstid ja fibrooskude 32% müksoomidest sisaldavad lubistusi Mlika et al. Pathologica 2013;105: Shin et al. Acta Radiol 2014; 55:
9 62-aastane naine Hospitaliseeritud süvenenud õhupuuduse ja madala koormustaluvuse tõttu Anamneesis kodade virvendusarütmia PERH Radioloogiakeskuse andmed
10 62-aastane naine Vasakus kojas on 2,2 x 1,7 x 2,3 cm suurune lobulaarsete kontuuridega lisamass Histoloogia: müokardi pinnal on hulgaliste kapillaarset tüüpi veresoonte pesadega ja värskete verevalandustega kasvaja, mille põhikomponendiks on müksoidne kude PERH Radioloogiakeskuse andmed
11 Papillaarne fibroelastoom Moodustab 15% südame primaarsetest tuumoritest Koosneb kollageen- ja elastiinkiudude võrgustikust, mis on kaetud endoteelirakkudega Pärineb klappide endokardist Seotud CMV infektsiooniga Keskmine läbimõõt: 1 cm Isointensiivne T1 ja T2 kujutistel, esineb mõõdukas kontrasteerumine Li et al. Am J Med 2013; 126:
12 60-aastane naine FIESTA Lisamass aordiklapi piirkonnas Kim et al. Korean J Radiol 2009; 10:
13 60-aastane naine Triple IR Lisamass aordiklapi piirkonnas Kim et al. Korean J Radiol 2009; 10:
14 Lipoom Aeglaselt kasvav tuumor, mis koosneb küpsetest adipotsüütidest Ümbritsetud kapsliga Sagedasem lokalisatsioon: parem vatsake, parem koda, kodade vahesein Läbimõõt võib ulatuda 15 cm-ni Hüperintensiivne signaal T1 ja T2 sekventsis Hüpointensiivne signaal rasva supressiooniga sekventsides
15 57-aastane mees Õhupuudus füüsilise koormuse puhul, auskultatsioonil on kuuldav süstoolne kahin T1 STIR Rajiah et al. Circulation 2011;124:
16 Rabdomüoom Kõige sagedasem südame primaarne tuumor lastel Pärineb vatsakeste müokardist Tugevalt seotud tuberoosse skleroosiga 47%-l tuberoosse skleroosiga patsientidest esinevad rabdomüoomid1 70% rabdomüoomidest taandub spontaanselt2 Isointensiivne T1 sekventsis, hüperintensiivne T2 sekventsis 1. Benyounes et al. Arch Cardiovasc Dis 2012; 105: Yadava et al. Indian Heart J 2012; 64:
17 Vastsündinu Esimesel elupäeval tekkisid tsüanoos ja bradükardia Tuumor vasakus vatsakeses Padalino et al. Circulation 2011; 124:
18 Vastsündinu SSFP SSFP+Gd Ulatuslik lisamass vasakus vatsakeses Padalino et al. Circulation 2011; 124:
19 Vastsündinu Rabdomüoomi resektsioon Kardiomüotsüüdid on mõõtmetelt suurenenud, tsütoplasma on vakuoliseeritud Padalino et al. Circulation 2011; 124:
20 Südame pahaloomulised kasvajad Angiosarkoom Fibrosarkoom Rabdomüosarkoom Diferentseerumata sarkoom
21 Angiosarkoom Moodustab 10% südame primaarsetest tuumoritest Hästi vaskulariseeritud mesenhümaalne tuumor Esineb sagedamini keskealistel meestel Kliiniline pilt: väsimus, õhupuudus, rindkerevalu, subfebriilne palavik Infiltreerib külgnevaid struktuure Metastaseerub enamasti kopsudesse, maksa ja ajju
22 41-aastane mees Vereköha, valu rinnus, alaseljavalu, kaalulangus Paremas kojas on ebakorrapärase kujuga tuumor, mis infiltreerib paremat vatsakest perikardi ja ülemist õõnesveeni Dong et al. Heart Lung Circ 2014.
23 41-aastane mees T1 T2 Metastaas L5 lülikehas Dong et al. Heart Lung Circ 2014.
24 Päikesekiire tunnus (sun ray sign) T1 Akkaya et al. Cardiol Young 2013; 24: 1 3.
25 Fibrosarkoom Enamasti paikneb vasakus kojas Lobulaarsete kontuuridega homogeenne lisamass, mis infiltreerib ümbritsevaid struktuure Kaugmetastaasid diagnoosimise ajaks 38%-l patsientidest Kopsud, mediastiinum, lülisammas Madalalt diferentseerunud fibrosarkoomidele on iseloomulikud verevalandused ja tsentraalne nekroos Kogon et al. Ann Thorac Surg 2011; 91:
26 7-kuune imik Laps muutus loiuks, ei huvitu ümbritsevast Esineb tahhüpnoe, nahk on tsüanootiline Vasakus vatsakeses on lisamass, mis ulatub perikardiõõnde Kogon et al. Ann Thorac Surg 2011; 91:
27 Rabdomüosarkoom Embrüonaalne vorm (lapseeas) Pleomorfne vorm (70%-l juhtudest meestel vanuses üle 50 eluaasta) Ebakorrapäraselt piirdunud tuumor, mis pärineb enamasti vatsakeste müokardist Haarab sageli südameklappe Isointensiivne signaal T1-s ja T2-s Mõõdukas hiliskontrasteerumine Keskmine elulemus on <12 kuud
28 9-aastane poiss Progresseeruv hingeldus ja õhupuudus füüsilise koormuse korral viimase kolme kuu jooksul Objektiivne leid: Jugulaarveenid on täitunud Vererõhk: 62/44 mmhg Südame löögisagedus: 142 lööki/min Ehho-KG: südame tamponaadi tunnused Ashraf et al. Pediatr Cardiol 2013; 34:
29 9-aastane poiss Parasternaalne lühike telg Enne perikardiotsenteesi Pärast perikardiotsenteesi Ashraf et al. Pediatr Cardiol 2013; 34:
30 9-aastane poiss SSFP + Gd Vasaku vatsakese lateraalset seina infiltreeriv tuumor; metastaas parema kopsu alasagaras; pleura- ja perikardiefusioon SSFP + Gd Metastaasid mediastiinumis Ashraf et al. Pediatr Cardiol 2013; 34:
31 Diferentseerumata sarkoom Moodustab kuni 25% südame sarkoomidest Keskmine vanus haigestumisel on 44 aastat, M=N Pleomorfsed käävjad või polügonaalsed rakud, esineb atüpism ja rohkelt mitoose Lobulaarsete kontuuridega tuumor, kinnitub endokardile jalakese abil Paikneb enamasti vasakus kojas Põhjustab mitraalstenoosi, südamepuudulikkust ja embolisatsiooni Randhawa et al. Curr Probl Diagn Radiol 2011; 40:
32 50-aastane naine Õhupuudus, tahhükardia, subfebriilne palavik Südamevari on laienenud, KTI 0,60 Ramos et al. J Heart Valve Dis 2008; 17:
33 50-aastane naine Vasak koda on laienenud Mitraalklapi ava 1,5 cm2 (norm 4-6 cm2) Lisamass mitraalklapi eesmise hõlma piirkonnas Ramos et al. J Heart Valve Dis 2008; 17:
34 50-aastane naine Hospitaliseerimise ajal tekkis valu vasakus reies, vasak jalg muutus jahedaks Täitumisdefekt aordi bifurkatsiooni piirkonnas Anterolateraalsest papillaarlihasest lähtunud diferentseerumata sarkoom Ramos et al. J Heart Valve Dis 2008; 17:
35 Metastaasid Tuumor Südame metastaaside osakaal Melanoom 53,9% Leukemia 34% Kopsuvähk 10,2% Rinnavähk 8% Söögitoruvähk 7,7% Munasarjavähk 5,7% Neeruvähk 5,3% Perikard > epikard > müokard > endokard Maraj et al. Int J Cardiol 2009; 133:
36 Metastaasid Metastaseerumine perikardi võib põhjustada perikardiiti ja perikardi efusiooni Enamik metastaasidest on hüpointensiivsed T1 kujutistel ja hüperintensiivsed T2 kujutistel Melanoom on T1-s hüperintensiivne melaniini paramagneetiliste omaduste tõttu Randhawa et al. Curr Probl Diagn Radiol 2011; 40:
37 57-aastane mees Vasakult küünarvarrelt eemaldatud maliigne melanoom, teostatud aksillaarne lümfadenektoomia vasakult metastaaside tõttu Edasi olnud regulaarselt jälgimisel Kaks aastat hiljem S-100 taseme suurenemine, PET-uuringul esineb haiguse progresseerumine metastaasid kopsudes, maksas ja mediastiinumi lümfisõlmedes PERH Radioloogiakeskuse andmed
38 57-aastane mees PET-KT-uuring FDG-ga PERH Radioloogiakeskuse andmed
39 39-aastane mees Neli kuud tagasi märkas tihket vohandit parema õlavarre distaalses osas, mis on mõõtmetelt suurenenud 17 aastat tagasi sattus autoavariisse, diagnoositi parema õlavarreluu distaalse osa ja küünarluu proksimaalse osa fraktuurid PERH Radioloogiakeskuse andmed
40 39-aastane mees PERH Radioloogiakeskuse andmed
41 39-aastane mees Vasaku vatsakese kõrval on lisamass mõõtmetega 15 x 35 mm PERH Radioloogiakeskuse andmed
42 39-aastane mees FIESTA+Gd Triple-IR FSE Vasaku vatsakese tipu ja keskmise segmendi piirkonnas on ühtlase struktuuriga lisamass, mis infiltreerib müokardi selles piirkonnas kogu paksuses, piirneb perikardiga, parietaalsest lestmest läbikasvu ei ole. PERH Radioloogiakeskuse andmed
43 Kokkuvõte Südame tuumorid võivad põhjustada kopsupaisu, südamepuudulikkust, rütmihäireid, klapirikkeid, embolisatsiooni ja perikardiefusiooni ¾ südame primaarsetest tuumoritest on beniigsed, ¼ on maliigsed Metastaasid esinevad 50 korda sagedamini kui primaarsed südame tuumorid
44 Kasutatud kirjandus Sparrow PJ, Kurian JB, Jones TR, Sivananthan MU. MR imaging of cardiac tumors. Radiographics 2005; 25: Dellamore A, Albertini A, Lamarra M. Twenty years experience in oncologic surgery for primary cardiac tumors. G Chir 2013; 34: Khan MS, Sanki PK, Hossain MZ, Charles A, Bhattacharya S, Sarkar UN. Cardiac myxoma: A surgical experience of 38 patients over 9 years, at SSKM hospital Kolkata, India. South Asian J Cancer 2013; 2: Li A, Azarisman SM, Teo KS, Worthley MI, Sidharta S, Glenie T, Samaraie L, Chua SK, Bailie TJ, Stuklis R, Worthley SG. The innocent bystander: papillary fibroelastoma. Am J Med 2013; 126: Mecchia D, Lavezzi AM, Matturri L. Primary cardiac fibroma and cardiac conduction system alterations in a case of sudden death of a 4-monthold infant. Open Cardiovasc Med J 2013; 7: Randhawa K, Ganeshan A, Hoey ET. Magnetic resonance imaging of cardiac tumors: part 2, malignant tumors and tumor-like conditions. Curr Probl Diagn Radiol 2011; 40:
45 Kasutatud kirjandus 7. Hiremath R, Noronha GP, Naik SB, Tejasvi S. Metastatic Cardiac Tumor from Bronchogenic Carcinoma via Pulmonary Vein: A Case Report. J Clin Diagn Res 2013; 7: Shin W, Choe YH, Kim SM, Song IY, Kim SS. Detection of cardiac myxomas with non-contrast chest CT. Acta Radiol 2014; 55: Mlika M, Ben Youssef A, Hamrouni R, Ayadi-Kaddour A, Kilani T, El Mezni F. Diagnosis and clinical course of cardiac myxoma. Pathologica 2013;105: Kim EY, Choe YH, Sung K, Park SW, Kim JH, Ko YH. Multidetector CT and MR imaging of cardiac tumors. Korean J Radiol 2009; 10: Rajiah P, To AC, Tan CD, Schoenhagen P. Multimodality imaging of an unusual case of right ventricular lipoma. Circulation 2011;124: Yadava OP. Cardiac tumours in infancy. Indian Heart J 2012; 64:
46 Kasutatud kirjandus 13. Teis A, Sheppard MN, Alpendurada F. Unusual location for a large cardiac fibroma. Circulation 2011; 124: Ainsworth CD, Salehian O, Nair V, Whitlock RP. A bloody mass: rare cardiac tumor as a cause of symptomatic ventricular arrhythmias. Circulation 2012; 126: Dong Y, Zhu D, Dong L, Ren L. Primary cardiac angiosarcoma with pulmonary and lumbar metastases presenting as unexplained pericardial effusion in a 41 year-old man. Heart Lung Circ 2014 (in press). 16. Kogon B, Shehata B, Katzenstein H, Samai C, Mahle W, Maher K, Olson T. Primary congenital infantile fibrosarcoma of the heart: the first confirmed case. Ann Thorac Surg 2011; 91: Sandoval E, Quintana E, Ninot S, Castella M. Myxoid liposarcoma: an unusual primary cardiac tumour. Eur J Cardiothorac Surg 2012; 42: Ashraf T, Day TG, Marek J, Hughes M, Giardini A. A triad: cardiac rhabdomyosarcoma, stroke and tamponade. Pediatr Cardiol 2013; 34:
47 Kasutatud kirjandus 19. Jurcut R, Savu O, Popescu BA, Florian A, Herlea V, Moldovan H, Ginghina C. Images in cardiovascular medicine: Primary cardiac leiomyosarcoma: when valvular disease becomes a vascular surgical emergency. Circulation 2010; 121: Padalino MA, Vida VL, Bhattarai A, Reffo E, Milanesi O, Thiene G, Stellin G, Basso C. Giant intramural left ventricular rhabdomyoma in a newborn. Circulation 2011; 124: Neragi-Miandoab S, Kim J, Vlahakes GJ. Malignant tumours of the heart: a review of tumour type, diagnosis and therapy. Clin Oncol 2007; 19: Maraj S, Pressman GS, Figueredo VM. Primary cardiac tumors. Int J Cardiol 2009; 133: Butany J, Nair V, Naseemuddin A, Nair GM, Catton C, Yau T. Cardiac tumours: diagnosis and management. Lancet Oncol 2005; 6: Aksu G. Clinical findings and therapeutic options in cardiac tumours. Rep Pract Oncol Radiother 2006; 11:
48 Kasutatud kirjandus 25. Bendel EC, Maleszewski JJ, Araoz PA. Imaging sarcomas of the great vessels and heart. Semin Ultrasound CT M. 2011; 32: Araoz PA, Mulvagh SL, Tazelaar HD, Julsrud PR, Breen JF. CT and MR imaging of benign primary cardiac neoplasms with echocardi ographic correlation. Radiographics 2000; 20: Dutta N, Agarwal S, Upretti L, Satsangi DK. Extensive local spread of rhabdomyosarcoma. J Card Surg 2011; 26: Grebenc ML, Rosado-de-Christenson ML, Green CE, Burke AP, Galvin JR. Cardiac myxoma: imaging features in 83 patients. Radiographics 2002; 22:
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