Neuroblastoma
Generalità
Il neuroblastoma è una neoplasia maligna di insorgenza dalle cellule della cresta neurale e poiché tali cellule sono dislocate ubiquitariamente (gangli paravertebrali, surreni, cute ecc..) la neoplasia può svilupparsi in qualsiasi organo o apparato. Nel 70% dei casi si sviluppa nell’addome-in prossimità dei surreni– ma può localizzarsi anche a livello toracico, sul collo o sulla colonna vertebrale. Il neuroblastoma ha un incidenza di 6.5-10.1 nuovi casi/ anno 1.000.000 di soggetti di età <15 anni, con un tasso d’ incidente tra 0-2 anni e una lieve prevalenza nel sesso maschile.
Eziologia
L’eziologia è sconosciuta.
Sintomi
I sintomi variano a seconda della localizzazione del tumore:
- localizzazione addominale: è la più frequente e spesso si manifesta con una massa palpabile nei quadranti laterali dell’addome o in regioni altocostale, abitualmente oltrepassante la linea mediana
- linfoadenoma
- dolori addominali diffusi
- anoressia
- vomito.
Localizzazione intratoracica:
- tosse
- dispnea
- disfagia
- segni complessivi a carico del midollo spinale.
Segni sistemici:
- dolori osteo-articolari
- astenia
- perdita di peso
- pallore
- febbre ricorrente.
- Può dare metastasi in vari organi (fegato, ossa, cute..)
Diagnosi
La diagnosi avviene a seguito di un’approfondita visita attraverso la quale lo specialista raccoglie tutte le informazioni necessarie sulla storia familiare e i segni del bambino. In caso di sospetto si precede con una serie completa di analisi del sangue e delle urine alla ricerca di sostanze chiamate catecolamine, molecole prodotte dalle cellule nervose simpatiche – che in presenza di neuroblastoma vengono rilasciate a livello sanguigno in quantità sufficiente per poter essere individuate.
La diagnosi si avvale anche di altri esami diagnostici strumentali quali:
- ecografia
- TAC
- RMN
- scintigrafia ossea (per lo studio morfologico della lesione e delle eventuali metastasi)
Il prelievo istologico della lesione (tumore primario e metastasi) è l’esame che permette una diagnosi certa.
La stadiazione è determinante per la scelta terapeutica e la prognosi.
Stadio I
Asportazione chirurgica.
90-100% di sopravvivenza
Stadio II
Asportazione chirurgica
Il tumore è più esteso ma ancora circoscritto.
L’asportazione chirurgica può lasciare residui che richiedono brevi periodi di chemioterapia per ridurre il rischio di proliferazione delle cellule tumorali. (90% di sopravvivenza)
Stadio III
Chirurgia e chemioterapia
Il tumore è già esteso e bisogna ricorrere alla chemioterapia per ridurre la massa tumorale e poter intervenire chirurgicamente. (30-40% di sopravvivenza).
Stadio IV
Il tumore ha disseminato cellule malate in altri organi.
Richiede un intenso trattamento di chemioterapia data l’aggressività della malattia. (10-15% sopravvivenza)
Stadio 4 S
Nessun trattamento (supporto minimo)
Si tratta di una forma particolare di neuroblastoma e in alcuni casi può guarire da solo, senza alcun trattamento. Solo in alcuni casi richiede un breve ciclo di chemioterapia.
La disseminazione coinvolge cute e fegato ma non lo scheletro. .
Trattamento
Negli stadi iniziali del tumore la chirurgia rappresenta il trattamento di prima scelta. La chemioterapia è indicata negli stadi più avanzati per ridurre le dimensioni del tumore o se la malattia presenta metastasi. Il trattamento chirurgico, radioterapico e chemioterapico sono le scelte a disposizione per contrastare il tumore. L’immunoterapia è il fronte terapeutico più promettente che ha garantito un evidente aumento della sopravvivenza Il nuovo farmaco è un anticorpo monoclonale che ha come bersaglio l’antigene GD2, una molecola particolarmente presente (sovraespressa) sulla superficie delle cellule di neuroblastoma.
Trisomia 18 o sindrome di Edwards
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