Adrenocortical Carcinoma

Cancer research · Adrenocortical carcinoma · TP53

Adrenocortical carcinoma is a rare endocrine malignancy with limited therapeutic options and a poor prognosis, necessitating early identification of high‑risk patients and the development of targeted therapies.

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy arising from the adrenal cortex often with unexpected biological behavior. It can occur at any age, with two peaks of incidence: in the first and between the fifth and seventh decades of life. Although ACC are mostly hormonally active, precursors and metabolites may also be produced by dedifferentiated and immature malignant cells. Distinguishing the etiology of an adrenal mass, between benign adenomas, which are quite frequent in the general population, and malignant carcinomas with dismal prognosis is challenging. However, recent advances in genomic pathology and staging allow the development of standardization of pathology reporting and refinement of prognostic grouping for planning the treatment of patients with ACC. No single histopathological, as well as no single imaging method, hormonal work‑up, or immunohistochemical labelling, can definitively prove the diagnosis of ACC. Over several decades great efforts have been made to find novel reliable and available diagnostic and prognostic factors including steroid metabolome profiling or target gene identification. Preliminary data show that for localized ACC, molecular markers (gene expression, methylation, and chromosome alterations) could predict cancer recurrence. Nevertheless, many of these markers need further validation and some are difficult to be widely applied in clinical settings. The development of new prognostic tools highlights the need for early identification of high‑risk ACC patients who could benefit from individualized management. ACC is frequently diagnosed in advanced stages and therapeutic options are unfortunately limited. The management of patients with ACC requires a multidisciplinary approach. Surgery remains the “gold standard” treatment whereas a number of systemic therapies including chemotherapy is administered in patients with extensive not amenable to surgical resection disease. Recently, immunotherapy in advanced ACC has also been investigated in different studies. However, the reported rates of overall response rate and progression‑free survival (PFS) were generally poor. Thus, new biological markers that could predict patient prognosis and provide individualized therapeutic options, especially targeted treatments, are required.

Early identification of high‑risk patients and development of targeted therapies are essential to improve outcomes for this rare but aggressive cancer.

Evidence level: Korai humán adat. Kis vagy feltáró emberi adat.

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