Adrenocortical Carcinoma with Hypercortisolism

• Autores: Soraya Puglisi, Paola Perotti, Anna Pia, Giuseppe Reimondo, Massimo Terzolo
• Localización: Endocrinology and metabolism clinics of North America, ISSN 0889-8529, Vol. 47, Nº. 2, 2018 (Ejemplar dedicado a: Cushing's Syndrome), págs. 395-407
• Idioma: inglés
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• Resumen

  • Adrenocortical carcinoma (ACC) is a rare and aggressive tumor with an annual inci- dence between 0.7 and 2 cases per million population. ACC is more frequently detected in women (55%–60%) and certain age groups (fourth and fifth decades);

    however, ACC can occur at any age.

    ACC can affect children, with an exceedingly high incidence reported in southern Brazil because of the high prevalence of a TP53 germline mutation.

    ACCs most frequently present as sporadic tumors, but can be encountered in the setting of hereditary tumor syndromes, such as Li Fraumeni (TP53 germline and somatic mutations), familial adenomatous polyposis coli (b-cate- nin somatic mutations), and Beckwith–Wiedeman (IGF-2 overexpression).

    Patients with ACC have an extremely poor prognosis, with an overall 5-year survival rate between 16% and 47%.

    Prognosis is mainly influenced by completeness of sur- gical removal and tumor stage at diagnosis, with a 5-year stage-dependent survival of