-
Endocrine Reviews Apr 2014Adrenocortical carcinoma (ACC) is a rare endocrine malignancy, often with an unfavorable prognosis. Here we summarize the knowledge about diagnosis, epidemiology,... (Review)
Review
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy, often with an unfavorable prognosis. Here we summarize the knowledge about diagnosis, epidemiology, pathophysiology, and therapy of ACC. Over recent years, multidisciplinary clinics have formed and the first international treatment trials have been conducted. This review focuses on evidence gained from recent basic science and clinical research and provides perspectives from the experience of a large multidisciplinary clinic dedicated to the care of patients with ACC.
Topics: Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Genetic Predisposition to Disease; Humans; Prevalence
PubMed: 24423978
DOI: 10.1210/er.2013-1029 -
Frontiers in Endocrinology 2022Adrenocortical carcinoma (ACC) is a rare endocrine malignancy of the adrenal gland with an unfavorable prognosis. It is rare in the pediatric population, with an... (Review)
Review
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy of the adrenal gland with an unfavorable prognosis. It is rare in the pediatric population, with an incidence of 0.2-0.3 patients per million in patients under 20 years old. It is primarily associated with Li-Fraumeni and Beckwith-Wiedemann tumor predisposition syndromes in children. The incidence of pediatric ACC is 10-15fold higher in southern Brazil due to a higher prevalence of mutation associated with Li-Fraumeni syndrome in that population. Current treatment protocols are derived from adult ACC and consist of surgery and/or chemotherapy with etoposide, doxorubicin, and cisplatin (EDP) with mitotane. Limited research has been reported on other treatment modalities for pediatric ACC, including mitotane, pembrolizumab, cabozantinib, and chimeric antigen receptor autologous cell (CAR-T) therapy.
Topics: Adult; Humans; Child; Young Adult; Adrenocortical Carcinoma; Mitotane; Adrenal Cortex Neoplasms; Li-Fraumeni Syndrome
PubMed: 36387865
DOI: 10.3389/fendo.2022.961650 -
Endocrinology and Metabolism Clinics of... Jun 2015Recent developments in the treatment of adrenocortical carcinoma (ACC) include diagnostic and prognostic risk stratification algorithms, increasing evidence of the... (Review)
Review
Recent developments in the treatment of adrenocortical carcinoma (ACC) include diagnostic and prognostic risk stratification algorithms, increasing evidence of the impact of historical therapies on overall survival, and emerging targets from integrated epigenomic and genomic analyses. Advances include proper clinical and molecular characterization of all patients with ACC, standardization of proliferative index analyses, referral of these patients to large cancer referral centers at the time of first surgery, and development of new trials in patients with well-characterized ACC. Networking and progress in the molecular characterization of ACC constitute the basis for significant future therapeutic breakthroughs.
Topics: Adrenal Cortex Neoplasms; Adrenalectomy; Adrenocortical Carcinoma; Antineoplastic Agents; Antineoplastic Agents, Hormonal; Chemotherapy, Adjuvant; Humans; Mitotane; Neoadjuvant Therapy; Neoplasm Staging; Prognosis; Radiotherapy, Adjuvant
PubMed: 26038209
DOI: 10.1016/j.ecl.2015.03.001 -
QJM : Monthly Journal of the... Jan 2022
Topics: Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Humans
PubMed: 34726762
DOI: 10.1093/qjmed/hcab276 -
Best Practice & Research. Clinical... May 2020Adrenocortical carcinoma (ACC) is an aggressive cancer characterized by poor survival. Apart from radical surgery, there is a limited range of therapeutic options and... (Review)
Review
Adrenocortical carcinoma (ACC) is an aggressive cancer characterized by poor survival. Apart from radical surgery, there is a limited range of therapeutic options and mitotane remains the cornerstone of medical treatment of ACC in either adjuvant or palliative settings. The aim of adjuvant mitotane therapy is to reduce the risk of ACC recurrence following surgical removal of the tumor. Use of mitotane in an adjuvant setting is off-label, but the recent guidelines endorsed by the European Society of Endocrinology (ESE) and the European Network for the Study of Adrenal Tumors (ENSAT) recommend it in ACC patients at high risk of recurrence. The palliative use of mitotane for treatment of advanced ACC aims at controlling tumor progression and, when present, hormone secretion. In this clinical setting, mitotane is used in association with chemotherapy to treat the more aggressive forms, while mitotane monotherapy is reserved for less progressive ACC. Many years after its introduction in clinical practice, there are still uncertainties surrounding the use of this old drug and the derived benefits. Moreover, physicians who use mitotane should recognize and manage the systemic effects of the drug that need a complex supporting therapy.
Topics: Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Antineoplastic Agents, Hormonal; Antineoplastic Combined Chemotherapy Protocols; Endocrinology; Humans; Mitotane
PubMed: 32179008
DOI: 10.1016/j.beem.2020.101415 -
Endocrinology and Metabolism Clinics of... Jun 2018Adrenocortical carcinoma (ACC) is a rare and aggressive tumor. ACC may be associated with different syndromes of hormone excess, most frequently Cushing's syndrome with... (Review)
Review
Adrenocortical carcinoma (ACC) is a rare and aggressive tumor. ACC may be associated with different syndromes of hormone excess, most frequently Cushing's syndrome with or without hypersecretion of androgens. Recent data suggest that cortisol excess is a negative prognostic factor in advanced and localized ACC. Surgery with radical intent, when feasible, is the most effective treatment for ACC with hypercortisolism. Mitotane is the medical treatment of choice, both postoperatively and in inoperable or metastatic cases. Because of its slow onset of action, combination with other antisecretory agents (ie, metyrapone) is helpful to achieve more rapid and effective control of hypercortisolism.
Topics: Adrenocortical Carcinoma; Antineoplastic Agents, Hormonal; Cushing Syndrome; Humans
PubMed: 29754640
DOI: 10.1016/j.ecl.2018.02.003 -
Surgical Pathology Clinics Dec 2019Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis. ACC is capable of secreting excess adrenocortical hormones, which can compound morbidity and... (Review)
Review
Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis. ACC is capable of secreting excess adrenocortical hormones, which can compound morbidity and compromise clinical outcomes. By the time most ACCs are diagnosed, there is usually locoregional or metastatic disease. Surgery is the most important treatment to offer possibility of cure or prolong survival. Several adjuvant therapies are used depending on grade and stage of the tumor and other patient-related factors. This review provides an overview of treatment approaches for ACC, highlighting evidence to support each treatment and acknowledging where more data and research are needed to improve care.
Topics: Adrenal Cortex Neoplasms; Adrenalectomy; Adrenocortical Carcinoma; Antineoplastic Combined Chemotherapy Protocols; Humans; Neoplasm Staging; Prognosis
PubMed: 31672303
DOI: 10.1016/j.path.2019.08.010 -
Surgical Oncology Clinics of North... Apr 2023Adjuvant and neoadjuvant chemotherapy in the treatment of adrenocortical carcinoma (ACC) is limited by few existing trials, most of which are retrospective. The drug... (Review)
Review
Adjuvant and neoadjuvant chemotherapy in the treatment of adrenocortical carcinoma (ACC) is limited by few existing trials, most of which are retrospective. The drug mitotane has been used for the treatment of ACC, although existing guidelines only support its use in high risk of recurrence. The first phase 3 trial involving systemic chemotherapy for ACC supports the use of etoposide, doxorubicin, cisplatin, and mitotane for combination therapy. No significant breakthrough has been discovered thus far in of targeted and immunotherapies. Neoadjuvant chemotherapy is only used to allow for complete surgical resection because complete excision is the definitive treatment of ACC.
Topics: Humans; Adrenocortical Carcinoma; Mitotane; Neoadjuvant Therapy; Adrenal Cortex Neoplasms; Retrospective Studies; Antineoplastic Combined Chemotherapy Protocols; Chemotherapy, Adjuvant
PubMed: 36925185
DOI: 10.1016/j.soc.2022.10.005 -
Current Oncology Reports Feb 2019Adrenocortical carcinoma (ACC) is a rare endocrine malignancy typically with poor prognosis. This review aims to summarize the current knowledge regarding the clinical... (Review)
Review
PURPOSE OF REVIEW
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy typically with poor prognosis. This review aims to summarize the current knowledge regarding the clinical management of ACC.
RECENT FINDINGS
Surgery remains the cornerstone for localized ACC management. In more advanced cases, debulking surgery when feasible can help with hormonal control and may allow the initiation of systemic therapy. Over the last few years, our understanding of ACC molecular pathogenesis has expanded with no significant change in treatment options. Platinum-based chemotherapy is the gold standard in metastatic ACC despite suboptimal efficacy. Tyrosine kinase inhibitor use did not result in meaningful benefit in ACC patients. Multiple clinical trials are currently exploring the role of immunotherapy in ACC. Despite the remarkable improvement in our understanding of the molecular signature and pathways in ACC, this knowledge did not yield a major breakthrough in management of advanced ACC. Multi-institutional and international collaborations are needed to identify promising treatments and new therapeutic targets to improve the care of ACC patients.
Topics: Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Antineoplastic Agents; Disease Management; Humans; Immunotherapy; Prognosis
PubMed: 30798468
DOI: 10.1007/s11912-019-0773-7 -
Cancer Treatment and Research... 2023Adrenocortical carcinoma (ACC) is a rare cancer with an estimated incidence of 0.7 to 2.0 cases per 1 million population per year in the United States. It is an... (Review)
Review
Adrenocortical carcinoma (ACC) is a rare cancer with an estimated incidence of 0.7 to 2.0 cases per 1 million population per year in the United States. It is an aggressive cancer originating in the cortex of the adrenal gland with a poor prognosis. The 5-year survival rate is less than 15% among patients with metastatic disease. In this article, we review the epidemiology and pathogenesis of ACC, the diagnostic procedures, the prognostic classification of ACC, and the treatment options from localized and resectable forms to advanced disease detailing recent therapeutic developments such as immunotherapy and molecularly targeted therapy.
Topics: Humans; Adrenocortical Carcinoma; Prognosis; Adrenal Cortex Neoplasms; Immunotherapy; Antineoplastic Combined Chemotherapy Protocols
PubMed: 37690343
DOI: 10.1016/j.ctarc.2023.100759