Tumori del cuore
Table of Contents
DEFINIZIONE
I tumori cardiaci primitivi sono rari con una prevalenza, nella popolazione adulta, variabile tra 0,001% e lo 0,5 % in quella infantile tra 0,01% lo 0,28 % . Anche il cuore, seppur raramente, può essere colpito da tumori, ma la loro malignità è legata più a fattori emodinamici che biologici. Il cuore può venire metastatizzato da qualsiasi neoplasia maligna capace di diffondere a distanza ed i tassi di frequenza sono molto più elevati di quanto si possa credere, tanto che, quando un paziente affetto da tumore maligno sviluppa una disfunzione cardiaca senza motivo apparente si dovrebbe sospettare una diffusione metastatica al cuore. I tumori maligni che più frequentemente metastatizzano al cuore sono:
• cancro del polmone
• cancro renale
• della faringe
• della mammella
• del fegato
• linfomi-leucemie.
Per quanto concerne i tumori primitivi del cuore, le forme benigne sono di gran lunga più frequenti (90% ) rispetto a quelle maligne (10%).
MIXOMA
Il mixoma è la più comune formazione cardiaca primitiva benigna, rappresenta più della metà di tutti i tumori cardiaci primari. La maggior parte delle neoplasie cardiache primitive ha una localizzazione intracavitaria. La sede più frequentemente interessata è l’ atrio sinistro (85% dei casi), mentre è raro il riscontro di un un mixoma cardiaco nell’ atrio destro (6%). Colpisce le donne in una una fascia d’età fra i 40 e i 70 anni. Rari sono i mixomi in età pediatrica. La presentazione clinica è varia.
SINTOMI
La produzione da parte del tumore di interleuchine rende ragione dei cosiddetti sintomi
- Dispnea
- Sintomi sistemici
- Embolie cerebrali
- Aritmie
- Scompenso cardiaco
- Sincope
- Febbricola
- Astenia
- Dolori osteoarticolari
- Malessere.
- = febbre, calo ponderale, anemia,astenia, mialgia.
Infine, esistono i mixomi cosiddetti “silenziosi” che non danno segni e rappresentano un reperto occasionale autoptico o, oggi molto più frequentemente, ecocardiografico incidentale. L’evoluzione naturale di questi mixomi silenziosi può essere con gli anni la formazione calcifica (litomixoma).
DIAGNOSI
La diagnosi di mixoma è facilmente e rapidamente eseguibile con l’ indagine ecocardiografica per valutare la presenza o meno di una massa tumorale . Si può ricorrere alla TAC e alla risonanza magnetica nucleare.
Analisi del sangue . Le analisi del sangue in presenza di un tumore benigno presentano un aumento delle immunoglobuline sieriche, un aumento dei globuli bianchi nel sangue, un numero di piastrine inferiore alla media e un numero di globuli rossi superiori alla media.
TERAPIA
La terapia è costituita dalla resezione chirurgica in circolazione extracorporea. L’asportazione della base di impianto del setto interatriale previene la possibilità di recidive.
FIBROELASTOMA
Il papilloma endocardico, detto anche fibroelastoma papillare, rappresenta la seconda causa più frequente di neoplasia cardiaca benigna.
Si presenta come un tumore di piccole dimensioni (1-2 cm) e a differenza del mixoma non è friabile.
A volte si presenta in maniera asintomatica, in alcuni casi con sincope e dolore toracico, presenta una potenzialità emboligena. Se localizzato nelle cuspidi sigmoidi aortiche , può incunearsi negli osti coronarici e provocare morte improvvisa.
DIAGNOSI
Ecocardiografia con la quale si può diagnosticare la neoplasia
Radiografia del torace, se la neoplasia è di piccole dimensioni non si riscontra nulla
Esame istologico che mostra ramificazioni non vascolarizzate con accumulo di fibre collagene ricoperte da endotelio.
TERAPIA
L’asportazione chirurgia è d’obbligo per la potenzialità emboligena.
RABDOMIOMA
Il rabdomioma è un tumore cardiaco primitivo benigno, tipico dell’infanzia. Frequentemente il periodo di crescita è tra il secondo e il terzo mese di gravidanza, poi rallenta.
SINTOMI
I sintomi e i segni dipendono dalla dimensione e dalla sede di localizzazione :
• tachicardia sopraventricolare parossistica e ventricolare
• morte cardiaca improvvisa
Diagnostica è la cosiddetta ” spider cell”, ovvero ‘aspetto a ragno del cardiomiocita con accumulo di glicogeno e dispersione a ragnatela dei miofilamenti. Frequente è l’associazione del rabdomioma con la sclerosi tuberosa.
La diagnosi prenatale di rabdomioma è effettuata quando una o più masse sono presenti all’interno del cuore del feto dopo aver eseguito un’ecografia prenatale. Tra gli altri esami volti alla diagnostica del rabdomioma vi sono:
- ecocardiogramma fetale
- angiografia, utilizzato in passato, adesso sostituito da un esame più preciso l’ecocardiografia
- biopsia, l’esame diagnostico più efficace
- esame approfondito dell’anatomia fetale
- analisi del sangue
TERAPIA
In quasi la metà dei casi regredisce spontaneamente e quindi non è necessario alcun trattamento, per gli altri si interviene chirurgicamente.
FIBROMA
Il fibroma è una forma tipica di tumore cardiaco benigno che colpisce soprattutto il ventricolo sinistro. Non sono rari i casi in cui questo tipo di tumori si riscontra già in età infantile.
La sintomatologia può essere anche ostruttiva quando le grosse dimensioni obliterano la cavità. Si interviene chirurgicamente per evitare che la massa si ingrandisca dando possibili complicazioni con aritmie, problemi di flusso sanguigno all’interno del quale; tuttavia ci sono casi clinici in cui si preferisce non intervenire a causa della posizione della massa tumorale.
SARCOMA
I tumori cardiaci maligni sono patologie rare, questo tipo di neoplasia colpisce prevalentemente gli adulti ed è stremamente raro nei bambini. La maggior parte dei pazienti con sarcoma cardiaco si presenta con sintomi cardiopolmonari:
- la dispnea è il sintomi di accompagnamento più frequente
- tamponamento cardiaco
- fenomeni embolici
- dolore toracico
- polmonite
- febbre
- aritmie
- edema periferico
- morte improvvisa
I tumori primitivi cardiaci sono lesioni altamente aggressivi e sempre letali. La sopravvivenza media è di circa 11 mesi sebbene siano descritti casi di sopravvivenza a 4 anni.
TERAPIA
La terapia chirurgica è palliativa sui sintomi e migliora la sopravvivenza. Tuttavia è stata riportata un’alta mortalità nei paziento sottoposti ad intervento chirurgico (17,5% nei primi 10 giorni dall’intervento) per tutte le diverse variabili istologiche. Inoltre nell’asportazione completa del tumore, è frequente un recidiva locale e la comparsa di metastasi è precoce, spesso entro un anno. Il trapianto di cuore è stato tentato in alcuni giovani pazienti con tumore cardiaco non resecabile ottenendo risultati soddisfacenti con molte variabili. La chemioterapia e la radioterapia non si sono dimostrate di alcuna l’utilità nel trattamento di questi pazienti.
ARTICOLI CORRISPONDENTI
- Malattia reumatica
- Insufficienza aortica
- Polmonite da mycoplasma pneumoniae
- Morte cardiaca improvvisa
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