SALUTE

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.

 

Adenocarcinoma

Trisomia 18 o sindrome di Edwards

Trisomia 13-Sindrome di Patau 


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