SALUTE

Sarcoma istiocitico

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Sarcomi
Sarcoma istiocitico

Definizione

Il sarcoma istiocitico è una neoplasia di derivazione istiocitaria. Le sedi colpite sono i linfonodi, la cute e il tratto gastrointestinale. Ne esistono due forme: localizzata e sistemica.


Epidemiologia

 

Il sarcoma istiocitico è una neoplasia rara che interessa individui compresi in un ampio range di età, più frequente nell’adulto con una mediana di 46- 55 anni, e con una lieve predilezione per il sesso maschile. Si riconoscono una forma localizzata e una forma sistemica, indicata anche come istiocitosi maligna. Alcuni casi vengono diagnosticati in associazione a linfomi di derivazione da linfociti B periferici, più spesso indolenti, mentre altri possono occorrere in pazienti con leucemia/linfoma B o T.

Clinica

Il sarcoma istiocitico può avere un’insorgenza acuta, con marcata compromissione dello stato generale. Presenza di sintomi sistemici e sintomi legati alla localizzazione di malattia. Manifestazioni cutanee e talvolta coinvolgimento scheletrico. Nella maggior parte dei casi la malattia si presenta in sedi extranodali, più frequentemente a livello del tratto gastroenterico, cute e tessuti molli. La presentazione clinica è variabile e va da quadri ad insorgenza acuta, con marcata compromissione dello stato generale e con sintomi  sistemici quali:

  • Febbre
  • Astenia
  • Dimagrimento
  • Sudorazioni

a quadri indolenti, con la comparsa di una massa solitaria, nelle forme localizzate. Relativamente frequente è l’epato-splenomegalia e il coinvolgimento osseo in forma di lesioni litiche. Possono osservarsi linfoadenopatie. Le manifestazioni cutanee consistono in un semplice rara, lesione solitaria o lesioni disseminate, in genere indolenti al tronco e all’estremità. I casi a localizzazione gastroenterica possono presentarsi con un’ occlusione intestinale.

Laboratorio

Nel 30% dei casi l’emogramma mette in evidenza una citopenia trilineare legata sia ad una ridotta produzione che ad una aumentata emarginazione  o distruzione: quest’ultima interessa particolarmente gli eritrociti, fagocitati dai macrofagi. Più spesso, nel decorso clinico, compaiono in circolo elementi mononucleati di grandezza variabile, spesso di forma bizzarra, che evocano l’aspetto del monoblasto-monocito. Raramente il quadro leucemico assume importanti proporzioni per un cospicuo aumento delle forme plastiche in circolo.
Citopenia, lisozima sierico aumentato, possibile ipertransaminasemia, fosfatasi  alcalina, iperbilirubinemia.

Decorso e diagnosi

  • Forma sistemica: prognosi severa
  • Forma localizzata: prognosi migliore


 Terapia

Una terapia aggressiva è indispensabile. Questa comporta l’applicazione di protocolli polichemioterapici ad elevata tossicità, generalmente utilizzati in associazione con radioterapia. La chirurgia può avere indicazione nelle forme localizzate. Polichemioterapia.


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Tutte le informazioni presenti nel sito non sostituiscono in alcun modo il giudizio di un medico specialista, l’unico autorizzato ad effettuare una consulenza medica ed esprimere un parere medico.

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