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Advances in Adrenocortical Carcinoma: Research and Clinical Treatment
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Advances in Adrenocortical Carcinoma: Research and Clinical Treatment

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Cancer Therapy".

Deadline for manuscript submissions: closed (30 April 2023) | Viewed by 13741

Special Issue Editor

Dr. Antonio Stigliano

E-Mail Website
Guest Editor
Endocrinology, Department of Clinical and Molecular Medicine, Sant’Andrea Hospital, Sapienza University of Rome, 00189 Rome, Italy
Interests: molecular mechanisms in steroidogenic neoplastic diseases, with special reference to adrenocortical tumorigenesis and different therapeutic strategies in adrenocortical carcinoma in vitro and in vivo

Special Issue Information

Dear Colleagues, 

Adrenocortical carcinoma (ACC) is a challenging endocrine disease that deserves to be addressed by a multidisciplinary healthcare team. Currently, surgery is the gold standard of ACC treatment, ensuring a radical possibility of care. Medical therapy is based on the use of mitotane which, even now, represents the main drug in adjuvant therapy, and in advanced ACC. It is associated with chemotherapy entrusted to the combination of etoposide, doxorubicin, and cisplatin (EDP-M) in metastatic disease. Recent accumulating data on radiotherapy also encourage its use in this tumor. On the contrary, data from clinical trials with target therapy and immunotherapy did not yield satisfactory results. Optimization of the current therapies has certainly guaranteed a better management of ACC; however, we still need to gain new knowledge about this disease and try new treatments. Being a rare disease, there are few possibilities to conduct many clinical studies, therefore, the design of future clinical trials needs to be supported by strong evidence from molecular and preclinical studies. Therefore, the purpose of this Special Issue is to bring together new knowledge and therapeutic attempts on ACC in a dedicated issue.

This Special Issue welcomes contributions from molecular, translational, and clinical research within the ACC.

Dr. Antonio Stigliano
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Cancers is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2900 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • adrenocortical carcinoma
  • surgery
  • mitotane
  • chemotherapy
  • radiotherapy
  • genomics
  • metabolomics
  • prognostic fac-tors
  • in vitro studies
  • in vivo studies
  • metastatic disease
  • recurrence disease
  • target therapy
  • immunotherapy
  • molec-ular biology
  • cellular biology
  • steroidogenesis
  • markers disease
  • translational study
  • preclinical study
  • clinical study

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Published Papers (7 papers)

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Research

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12 pages, 4832 KiB  
Article
Effect of Mitotane on Male Gonadal Function
by Federica Innocenti, Sara Di Persio, Marilena Taggi, Roberta Maggio, Pina Lardo, Vincenzo Toscano, Rita Canipari, Elena Vicini and Antonio Stigliano
Cancers 2023, 15(12), 3234; https://doi.org/10.3390/cancers15123234 - 18 Jun 2023
Viewed by 1223
Abstract
Background: Clinical evidence has shown frequent hypogonadism following mitotane (MTT) treatment in male patients with adrenocortical carcinoma. This study aimed to evaluate the impact of MTT on male gonadal function. Methods: Morphological analysis of testes and testosterone assays were performed on adult CD1 [...] Read more.
Background: Clinical evidence has shown frequent hypogonadism following mitotane (MTT) treatment in male patients with adrenocortical carcinoma. This study aimed to evaluate the impact of MTT on male gonadal function. Methods: Morphological analysis of testes and testosterone assays were performed on adult CD1 MTT-treated and untreated mice. The expression of key genes involved in interstitial and tubular compartments was studied by real-time PCR. Moreover, quantitative and qualitative analysis of spermatozoa was performed. Results: Several degrees of damage to the testes and a significant testosterone reduction in MTT-treated mice were observed. A significant decline in 3βHsd1 and Insl3 mRNA expression in the interstitial compartment confirmed an impairment of androgen production. Fsh-R mRNA expression was unaffected by MTT, proving that Sertoli cells are not the drug’s primary target. Sperm concentrations were significantly lower in MTT-treated animals. Moreover, the drug caused a significant increase in the percentage of spermatozoa with abnormal chromatin structures. Conclusion: MTT negatively affects the male reproductive system, including changes in the morphology of testicular tissue and reductions in sperm concentration and quality. Full article
(This article belongs to the Special Issue Advances in Adrenocortical Carcinoma: Research and Clinical Treatment)
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10 pages, 2533 KiB  
Article
Interplay between Immune Cell Infiltration and Tumor Histological Subtype: A Case of Adrenocortical Cancer
by Apollinariya V. Bogolyubova, Nano V. Pachuashvili, Arina V. Tkachuk, Natalia G. Mokrysheva and Liliya S. Urusova
Cancers 2022, 14(21), 5303; https://doi.org/10.3390/cancers14215303 - 28 Oct 2022
Cited by 1 | Viewed by 2274
Abstract
The analysis of the tumor microenvironment, especially tumor-infiltrated immune cells, is essential for predicting tumor prognosis, clinical outcomes, and therapy strategies. Adrenocortical cancer is a rare nonimmunogenic malignancy in which the importance of the presence of immune cells is not well understood. In [...] Read more.
The analysis of the tumor microenvironment, especially tumor-infiltrated immune cells, is essential for predicting tumor prognosis, clinical outcomes, and therapy strategies. Adrenocortical cancer is a rare nonimmunogenic malignancy in which the importance of the presence of immune cells is not well understood. In our study, we made the first attempt to understand the interplay between the histology of adrenocortical cancer and its immune landscape using cases from The Cancer Genome Atlas database and the Endocrinology Research Centre collection (Moscow, Russia). We showed that the oncocytic variant of adrenocortical cancer is characterized by intensive immune infiltration and better survival, and it is crucial to analyze the effect of immune infiltration independently for each histological variant. Full article
(This article belongs to the Special Issue Advances in Adrenocortical Carcinoma: Research and Clinical Treatment)
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19 pages, 4373 KiB  
Article
Estrogen Related Receptor Alpha (ERRα) a Bridge between Metabolism and Adrenocortical Cancer Progression
by Paola Avena, Arianna De Luca, Adele Chimento, Marta Claudia Nocito, Sara Sculco, Davide La Padula, Lucia Zavaglia, Matteo Giulietti, Constanze Hantel, Rosa Sirianni, Ivan Casaburi and Vincenzo Pezzi
Cancers 2022, 14(16), 3885; https://doi.org/10.3390/cancers14163885 - 11 Aug 2022
Cited by 7 | Viewed by 2210
Abstract
The aim of this study was to investigate the metabolic changes that occur in adrenocortical cancer (ACC) cells in response to the modulation of Estrogen Related Receptor (ERR)α expression and the impact on ACC progression. Proteomics analysis and metabolic profiling highlighted an important [...] Read more.
The aim of this study was to investigate the metabolic changes that occur in adrenocortical cancer (ACC) cells in response to the modulation of Estrogen Related Receptor (ERR)α expression and the impact on ACC progression. Proteomics analysis and metabolic profiling highlighted an important role for ERRα in the regulation of ACC metabolism. Stable ERRα overexpression in H295R cells promoted a better mitochondrial fitness and prompted toward a more aggressive phenotype characterized by higher Vimentin expression, enhanced cell migration and spheroids formation. By contrast, a decrease in ERRα protein levels, by molecular (short hairpin RNA) and pharmacological (inverse agonist XCT790) approaches modified the energetic status toward a low energy profile and reduced Vimentin expression and ability to form spheroids. XCT790 produced similar effects on two additional ACC cell lines, SW13 and mitotane-resistant MUC-1 cells. Our findings show that ERRα is able to modulate the metabolic profile of ACC cells, and its inhibition can strongly prevent the growth of mitotane-resistant ACC cells and the progression of ACC cell models to a highly migratory phenotype. Consequently, ERRα can be considered an important target for the design of new therapeutic strategies to fight ACC progression. Full article
(This article belongs to the Special Issue Advances in Adrenocortical Carcinoma: Research and Clinical Treatment)
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13 pages, 1625 KiB  
Article
Screening for Prognostic Biomarkers in Metastatic Adrenocortical Carcinoma by Tissue Micro Arrays Analysis Identifies P53 as an Independent Prognostic Marker of Overall Survival
by Segolene Hescot, Matthieu Faron, Manal Kordahi, Christine Do Cao, Annabelle Naman, Livia Lamartina, Julien Hadoux, Sophie Leboulleux, Francois Pattou, Sébastien Aubert, Jean-Yves Scoazec, Abir Al Ghuzlan and Eric Baudin
Cancers 2022, 14(9), 2225; https://doi.org/10.3390/cancers14092225 - 29 Apr 2022
Cited by 2 | Viewed by 1707
Abstract
Advanced adrenocortical carcinoma (ACC) has poor but heterogeneous prognosis. Apart from Ki67 index, no prognostic or predictive biomarker has been validated in advanced ACC, so far. We aimed at analyzing expression of a large panel of proteins involved in known altered pathways in [...] Read more.
Advanced adrenocortical carcinoma (ACC) has poor but heterogeneous prognosis. Apart from Ki67 index, no prognostic or predictive biomarker has been validated in advanced ACC, so far. We aimed at analyzing expression of a large panel of proteins involved in known altered pathways in ACC (cell cycle, Wnt/ß-catenin, methylation) to identify and prioritize potential prognostic or predictive parameters metastatic ACC population. We conducted a retrospective multicentric study. Overall survival (OS) and partial response according to RECIST 1.1 were primary endpoints. TMA was set up and 16 markers were analyzed. Modified ENSAT and GRAS parameters were characterized for prognostic adjustment. Results: We included 66 patients with a mean age at metastatic diagnosis of 48.7 ± 15.5 years. Median survival was 27.8 months. After adjustment to mENSAT-GRAS parameters, p53 and PDxK were prognostic of OS. No potential biomarker has been identified as predictive factor of response. We identified for the first time P53 as an independent prognostic marker of metastatic adrenocortical carcinoma after mENSAT-GRAS parameter adjustment. Prognostic impact of Wnt/ß-catenin alterations was not confirmed in this cohort of metastatic ACC. Full article
(This article belongs to the Special Issue Advances in Adrenocortical Carcinoma: Research and Clinical Treatment)
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10 pages, 430 KiB  
Article
Health-Related Quality of Life in Adrenocortical Carcinoma: Development of the Disease-Specific Questionnaire ACC-QOL and Results from the PROFILES Registry
by Rebecca V. Steenaard, Thomas M. A. Kerkhofs, Myrte Zijlstra, Floortje Mols, Michiel N. Kerstens, Henry J. L. M. Timmers, Rachel S. van Leeuwaarde, Koen M. A. Dreijerink, Elisabeth M. W. Eekhoff, Els J. M. Nieveen van Dijkum, Eleonora P. M. Corssmit, Ellen Kapiteijn, Marjolein N. T. Kremers, Richard A. Feelders and Harm R. Haak
Cancers 2022, 14(6), 1366; https://doi.org/10.3390/cancers14061366 - 8 Mar 2022
Viewed by 1788
Abstract
We aimed to develop a disease-specific adrenocortical carcinoma (ACC) health-related quality of life (HRQoL) questionnaire (ACC-QOL) and assess HRQoL in a population-based cohort of patients with ACC. Development was in line with European Organization for Research and Treatment of Cancer (EORTC) guidelines, though [...] Read more.
We aimed to develop a disease-specific adrenocortical carcinoma (ACC) health-related quality of life (HRQoL) questionnaire (ACC-QOL) and assess HRQoL in a population-based cohort of patients with ACC. Development was in line with European Organization for Research and Treatment of Cancer (EORTC) guidelines, though not an EORTC product. In phase I and II, we identified 90 potential HRQoL issues using literature and focus groups, which were reduced to 39 by healthcare professionals. Pilot testing resulted in 28 questions, to be used alongside the EORTC QLQ-C30. In Phase III, 100 patients with ACC were asked to complete the questionnaires twice in the PROFILES registry (3-month interval, respondents: first 67, second 51). Confirmatory factor analysis demonstrated the structural validity of 26 questions with their scale structure (mitotane side-effects, hypercortisolism/hydrocortisone effects, emotional effects). Internal consistency and reliability were good (Cronbach’s alpha 0.897, Interclass correlation coefficient 0.860). Responsiveness analysis showed good discriminative ability (AUC 0.788). Patients diagnosed more than 5 years ago reported a good HRQoL compared with the Dutch reference population, but experienced residual fatigue and emotional problems. Patients who underwent recent treatment reported a lower HRQoL and problems in several domains. In conclusion, we developed an ACC-specific HRQoL questionnaire with good psychometric properties. Full article
(This article belongs to the Special Issue Advances in Adrenocortical Carcinoma: Research and Clinical Treatment)
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Review

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21 pages, 376 KiB  
Review
Bridging the Scientific Gaps to Identify Effective Treatments in Adrenocortical Cancer
by Nicholas Michael and Naris Nilubol
Cancers 2022, 14(21), 5245; https://doi.org/10.3390/cancers14215245 - 26 Oct 2022
Cited by 1 | Viewed by 1581
Abstract
Adrenocortical cancer (ACC) typically presents in advanced stages of disease and has a dismal prognosis. One of the foremost reasons for this is the lack of available systemic therapies, with mitotane remaining the backbone of treatment since its discovery in the 1960s, despite [...] Read more.
Adrenocortical cancer (ACC) typically presents in advanced stages of disease and has a dismal prognosis. One of the foremost reasons for this is the lack of available systemic therapies, with mitotane remaining the backbone of treatment since its discovery in the 1960s, despite underwhelming efficacy. Surgery remains the only potentially curative option, but about half of patients will recur post-operatively, often with metastatic disease. Other local treatment options have been attempted but are only used practically on a case-by-case basis. Over the past few decades there have been significant advances in understanding the molecular background of ACC, but this has not yet translated to better treatment options. Attempts at novel treatment strategies have not provided significant clinical benefit. This paper reviews our current treatment options and molecular understanding of ACC and the reasons why a successful treatment has remained elusive. Additionally, we discuss the knowledge gaps that need to be overcome to bring us closer to successful treatment and ways to bridge them. Full article
(This article belongs to the Special Issue Advances in Adrenocortical Carcinoma: Research and Clinical Treatment)

Other

Jump to: Research, Review

11 pages, 1853 KiB  
Systematic Review
Impact of Lymphadenectomy on the Oncologic Outcome of Patients with Adrenocortical Carcinoma—A Systematic Review and Meta-Analysis
by Anne Hendricks, Sophie Müller, Martin Fassnacht, Christoph-Thomas Germer, Verena A. Wiegering, Armin Wiegering and Joachim Reibetanz
Cancers 2022, 14(2), 291; https://doi.org/10.3390/cancers14020291 - 7 Jan 2022
Cited by 15 | Viewed by 2153
Abstract
(1) Background: Locoregional lymphadenectomy (LND) in adrenocortical carcinoma (ACC) may impact oncological outcome, but the findings from individual studies are conflicting. The aim of this systematic review and meta-analysis was to determine the oncological value of LND in ACC by summarizing the available [...] Read more.
(1) Background: Locoregional lymphadenectomy (LND) in adrenocortical carcinoma (ACC) may impact oncological outcome, but the findings from individual studies are conflicting. The aim of this systematic review and meta-analysis was to determine the oncological value of LND in ACC by summarizing the available literature. (2) Methods: A systematic search on studies published until December 2020 was performed according to the PRISMA statement. The primary outcome was the impact of lymphadenectomy on overall survival (OS). Two separate meta-analyses were performed for studies including patients with localized ACC (stage I–III) and those including all tumor stages (I–IV). Secondary endpoints included postoperative mortality and length of hospital stay (LOS). (3) Results: 11 publications were identified for inclusion. All studies were retrospective studies, published between 2001–2020, and 5 were included in the meta-analysis. Three studies (N = 807 patients) reported the impact of LND on disease-specific survival in patients with stage I–III ACC and revealed a survival benefit of LND (hazard ratio (HR) = 0.42, 95% confidence interval (95% CI): 0.26–0.68). Based on results of studies including patients with ACC stage I–IV (2 studies, N = 3934 patients), LND was not associated with a survival benefit (HR = 1.00, 95% CI: 0.70–1.42). None of the included studies showed an association between LND and postoperative mortality or LOS. (4) Conclusion: Locoregional lymphadenectomy seems to offer an oncologic benefit in patients undergoing curative-intended surgery for localized ACC (stage I–III). Full article
(This article belongs to the Special Issue Advances in Adrenocortical Carcinoma: Research and Clinical Treatment)
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