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La sindrome di Cushing da secrezione ectopica di ACTH

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Riassunto

La sindrome di Cushing da secrezione ectopica di ACTH rappresenta circa il 10% dei casi di sindrome di Cushing ed il 20% delle forme ACTH-dipendenti. I tumori neuroendocrini polmonari ne sono la principale causa. Il quadro sintomatologico della sindrome può essere indistinguibile da quello della malattia di Cushing e la sua differenziazione dalla forma ipofisaria è particolarmente complessa e generalmente richiede l’esecuzione di una serie di test endocrinologici e il cateterismo dei seni petrosi inferiori per la determinazione di ACTH, che rappresenta l’esame gold standard. Stabilita la diagnosi di sindrome di Cushing ectopica è necessario localizzare il tumore secernente ACTH; ciò è talora molto difficile, particolarmente nel caso di produzione di ACTH da parte di un carcinoide bronchiale, che essendo spesso di piccole dimensioni, può rimanere occulto alla diagnostica per immagini per mesi o anni. Il trattamento di scelta in presenza di sindrome da secrezione ectopica di ACTH é la rimozione chirurgica del tumore che ne è la sorgente; nei casi in cui il tumore non sia localizzabile o non resecabile è indispensabile il control lo dell’ipercortisolismo mediante terapia medica o in caso di inefficacia di questa mediante surrenectomia bilaterale.

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Authors and Affiliations

  1. S.C. Endocrinologia, A. O. Ospedale Niguarda Ca’ Granda, Piazza Ospedale Maggiore 3, 20162, Milano, Italia

    Erika Grossrubatscher & Paola Loli

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  1. Erika Grossrubatscher
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  2. Paola Loli
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Correspondence to Erika Grossrubatscher.

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Grossrubatscher, E., Loli, P. La sindrome di Cushing da secrezione ectopica di ACTH. L’Endocrinologo 7, 11–22 (2006). https://doi.org/10.1007/BF03345943

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