ReviewFrontiers in cell and developmental biology2015
Adrenocortical carcinoma (ACC): diagnosis, prognosis, and treatment.
Review in Frontiers in cell and developmental biology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 112 papers.
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Who cites it
112 citing papers in PubMed, 223 citations in OpenAlex.
- A Retrospective Study of Ultrasonographic Features of Hepatic Metastases Following Adrenal Cortical Carcinoma Resection.Journal of clinical medicine · 2026Article
- Glucocorticoids induce a phagocytic C1Q+ macrophage phenotype primed for IFNγ-dependent CXCL9 secretion.Scientific reports · 2026Article
- Association of Age with Outcomes in Adrenocortical Carcinoma: A Combined Cancer Registry and Multi-Omic Analysis.Cancers · 2026Article
- Clinicopathologic Determinants of Overall Survival in Adrenocortical Carcinoma: A SEER-Based Population Study.Cancers · 2026Article
- Delayed Clinical Manifestation of Malignant Potential in a Cortisol-Secreting Adrenal Tumor Initially Diagnosed as Adenoma.IJU case reports · 2026Article
- Article
- Steroid Phenotype Stratification Reveals Distinct HLA Expression Signatures in Adrenocortical Carcinoma.Cancers · 2026Article
- An Excellent Clinical and Radiological Response Pattern to Pembrolizumab in a Patient With Metastatic Adrenocortical Carcinoma and Lynch Syndrome.IJU case reports · 2025Article
- Single-Cell Atlas Reveals Tumorigenic Profiles and Immune Dynamics of Adrenal Incidentalomas.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2025Article
- Apolipoprotein C1 and apoprotein E as potential therapeutic and prognostic targets for adrenocortical carcinoma.Cancer biomarkers : section A of Disease markers · 2025Article
- Review
- Adrenocortical neoplasm in a 2-year-old child: Clinical approach and diagnostic imaging.Radiology case reports · 2024Article
- Cost-effectiveness of Genetic Testing of Endocrine Tumor Patients Using a Comprehensive Hereditary Cancer Gene Panel.The Journal of clinical endocrinology and metabolism · 2024Article
- Neuroendocrine carcinoma of the adrenal gland: A rare case report and literature review.Radiology case reports · 2024Article
- Mild autonomous cortisol secretion: pathophysiology, comorbidities and management approaches.Nature reviews. Endocrinology · 2024Review
- TBiophysical reports · 2024Article
- A Rare Case of Adrenal Carcinoma With Isolated Hypercortisolism Mimicking Hyperaldosteronism.Cureus · 2024Article
- Article
- Survival nomogram and risk classification system for patients with adrenocortical carcinoma: a study based on the SEER database and external validation in China.Translational andrology and urology · 2024Article
- Adrenal cortical carcinoma: Paediatric aspects - literature review.Pediatric endocrinology, diabetes, and metabolism · 2024Review
52 more citing papers are in PubMed but not listed here.
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Authors and funding
1 author at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Adrenocortical carticnoma (ACC) is a rare malignancy with an incidence of 0.7-2.0 cases/million habitants/year. The diagnosis of malignancy relies on careful investigations of clinical, biological, and imaging features before surgery and pathological examination after tumor removal. Most patients present with steroid hormone excess or abdominal mass effects, but 15% of patients with ACC is initially diagnosed incidentally. After the diagnosis, in order to assess the ACC prognosis and establish an adequate basis for treatment decisions different tools are proposed. The stage classification proposed by the European Network for the Study of Adrenal Tumors (ENSAT) is recommended. Pathology reports define the Weiss score, the resection status and the proliferative index, including the mitotic count and the Ki67 index. As far as the treatment is concerned, in case of tumor limited to the adrenal gland, the complete resection of the tumor is the first option. Most patients benefit from adjuvant mitotane treatment. In metastatic disease, mitotane is the cornerstone of initial treatment, and cytotoxic drugs should be added in case of progression. Recently, the First International Randomized (FIRM-ACT) Trial in metastatic ACC reported the association between mitotane and etoposide/doxorubicin/cisplatin (EDP) as the new standard in first line treatment of ACC. In last years, new targeted therapies, including the IGF-1 receptor inhibitors, have been investigated, but their efficacy remains limited. Thus, new treatment concepts are urgently needed. The ongoing "omic approaches" and next-generation sequencing will improve our understanding of the pathogenesis and hopefully will lead to better therapies.
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