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Cancers
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Advances in Adrenocortical Carcinoma Research: Diagnosis, Treatment and Prognosis
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Advances in Adrenocortical Carcinoma Research: Diagnosis, Treatment and Prognosis

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

Deadline for manuscript submissions: closed (31 July 2023) | Viewed by 11647

Special Issue Editor

Dr. Letizia Canu

E-Mail Website
Guest Editor
Department of Experimental and Clinical Biomedical Sciences “Mario Serio”, University of Florence, 50139 Florence, Italy
Interests: adrenocortical carcinoma; pheochromocytoma; paraganglioma; adrenal disease

Special Issue Information

Dear Colleagues,

Adrenocortical carcinoma (ACC) is a rare aggressive tumor. Surgery represents the treatment of choice. Mitotane is used in adjuvant settings in high-risk patients (stage III and Ki67>10%) and in palliative case in association with EDP (etoposide-doxorubicin-cisplatin).

Target therapies and immunotherapies do not demonstrate a real efficacy in ACC patients. Considering the poor prognosis, new strategies are necessary.

This Special Issue will focus on future curative options in ACC patients at clinical and preclinical levels, considering possible diagnostic, therapeutic, and prognostic innovations.

Dr. Letizia Canu
Guest Editor

Manuscript Submission Information

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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
  • mitotane
  • adrenal tumor
  • surgery
  • EDP
  • target therapy
  • immunotherapy

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Published Papers (6 papers)

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Research

20 pages, 4992 KiB  
Article
Low PRKAB2 Expression Is Associated with Poor Outcomes in Pediatric Adrenocortical Tumors, and Treatment with Rottlerin Increases the PRKAB2 Level and Inhibits Tumorigenic Aspects in the NCI-H295R Adrenocortical Cancer Cell Line
by Alcides Euzebio Tavares Xavier, Luciana Chain Veronez, Luís Fernando Peinado Nagano, Carolina Alves Pereira Correa, Mirela Baroni, Milena Silva Ramos, Rosane de Gomes de Paula Queiroz, Carlos Augusto Fernandes Molina, José Andres Yunes, Silvia Regina Brandalise, Sonir Antonio Rauber Antonini, Luiz Gonzaga Tone, Elvis Terci Valera and Carlos Alberto Scrideli
Cancers 2024, 16(6), 1094; https://doi.org/10.3390/cancers16061094 - 8 Mar 2024
Cited by 1 | Viewed by 1510
Abstract
Pediatric adrenocortical tumors (ACTs) are rare, highly heterogeneous neoplasms with limited therapeutic options, making the investigation of new targets with potential therapeutic or prognostic purposes urgent. The PRKAB2 gene produces one of the subunits of the AMP-activated protein kinase (AMPK) complex and has [...] Read more.
Pediatric adrenocortical tumors (ACTs) are rare, highly heterogeneous neoplasms with limited therapeutic options, making the investigation of new targets with potential therapeutic or prognostic purposes urgent. The PRKAB2 gene produces one of the subunits of the AMP-activated protein kinase (AMPK) complex and has been associated with cancer. However, little is known about the role AMPK plays in ACTs. We have evaluated how PRKAB2 is associated with clinical and biological characteristics in 63 pediatric patients with ACTs and conducted in vitro studies on the human NCI-H295R ACC cell line. An analysis of our cohort and the public ACC pediatric dataset GSE76019 showed that lower PRKAB2 expression was associated with relapse, death, metastasis, and lower event-free and overall survival rates. Multivariate analysis showed that PRKAB2 expression was an independent prognostic factor when associated with age, tumor weight and volume, and metastasis. In vitro tests on NCI-H295R cells demonstrated that Rottlerin, a drug that can activate AMPK, modulated several pathways in NCI-H295R cells, including AMPK/mTOR, Wnt/β-catenin, SKP2, HH, MAPK, NFKB, and TNF. Treatment with Rottlerin decreased cell proliferation and migration, clonogenic capacity, and steroid production. Together, these results suggest that PRKAB2 is a potential prognostic marker in pediatric ACTs, and that Rottlerin is promising for investigating drugs that can act against ACTs. Full article
(This article belongs to the Special Issue Advances in Adrenocortical Carcinoma Research: Diagnosis, Treatment and Prognosis)
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19 pages, 855 KiB  
Article
The Value of Local Therapies in Advanced Adrenocortical Carcinoma
by Otilia Kimpel, Barbara Altieri, Marta Laganà, Thomas J. Vogl, Hamzah Adwan, Tina Dusek, Vittoria Basile, James Pittaway, Ulrich Dischinger, Marcus Quinkler, Matthias Kroiss, Soraya Puglisi, Deborah Cosentini, Ralph Kickuth, Darko Kastelan and Martin Fassnacht
Cancers 2024, 16(4), 706; https://doi.org/10.3390/cancers16040706 - 7 Feb 2024
Cited by 2 | Viewed by 1868
Abstract
International guidelines recommend local therapies (LTs) such as local thermal ablation (LTA; radiofrequency, microwave, cryoablation), transarterial (chemo)embolisation (TA(C)E), and transarterial radioembolisation (TARE) as therapeutic options for advanced adrenocortical carcinoma (ACC). However, the evidence for these recommendations is scarce. We retrospectively analysed patients receiving [...] Read more.
International guidelines recommend local therapies (LTs) such as local thermal ablation (LTA; radiofrequency, microwave, cryoablation), transarterial (chemo)embolisation (TA(C)E), and transarterial radioembolisation (TARE) as therapeutic options for advanced adrenocortical carcinoma (ACC). However, the evidence for these recommendations is scarce. We retrospectively analysed patients receiving LTs for advanced ACC. Time to progression of the treated lesion (tTTP) was the primary endpoint. The secondary endpoints were best objective response, overall progression-free survival, overall survival, adverse events, and the establishment of predictive factors by multivariate Cox analyses. A total of 132 tumoural lesions in 66 patients were treated with LTA (n = 84), TA(C)E (n = 40), and TARE (n = 8). Complete response was achieved in 27 lesions (20.5%; all of them achieved by LTA), partial response in 27 (20.5%), and stable disease in 38 (28.8%). For the LTA group, the median tTTP was not reached, whereas it was reached 8.3 months after TA(C)E and 8.2 months after TARE (p < 0.001). The median time interval from primary diagnosis to LT was >47 months. Fewer than four prior therapies and mitotane plasma levels of >14 mg/L positively influenced the tTTP. In summary, this is one of the largest studies on LTs in advanced ACC, and it demonstrates a very high local disease control rate. Thus, it clearly supports the guideline recommendations for LTs in these patients. Full article
(This article belongs to the Special Issue Advances in Adrenocortical Carcinoma Research: Diagnosis, Treatment and Prognosis)
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11 pages, 721 KiB  
Article
Feasibility and Activity of Megestrol Acetate in Addition to Etoposide, Doxorubicin, Cisplatin, and Mitotane as First-Line Therapy in Patients with Metastatic/Unresectable Adrenocortical Carcinoma with Low Performance Status
by Antonella Turla, Marta Laganà, Andrea Abate, Valentina Cremaschi, Manuel Zamparini, Matteo Chittò, Francesca Consoli, Andrea Alberti, Roberta Ambrosini, Mariangela Tamburello, Salvatore Grisanti, Guido Alberto Massimo Tiberio, Sandra Sigala, Deborah Cosentini and Alfredo Berruti
Cancers 2023, 15(18), 4491; https://doi.org/10.3390/cancers15184491 - 9 Sep 2023
Cited by 2 | Viewed by 1500
Abstract
(1) Background: The standard first-line therapy for advanced adrenocortical carcinoma (ACC) is represented by EDP-M (etoposide, doxorubicin, cisplatin + mitotane). Progestins have shown cytotoxic activity both in vitro and in vivo on ACC; better EDP-M tolerability and efficacy have been hypnotized due to [...] Read more.
(1) Background: The standard first-line therapy for advanced adrenocortical carcinoma (ACC) is represented by EDP-M (etoposide, doxorubicin, cisplatin + mitotane). Progestins have shown cytotoxic activity both in vitro and in vivo on ACC; better EDP-M tolerability and efficacy have been hypnotized due to the association with progestins. (2) Methods: The feasibility and tolerability of EDP-M combined with oral megestrol acetate (EDP-MM) were tested in 24 patients (pts) affected by metastatic ACC with a low performance status (PS); the case group was compared with a 48 pts control group according to the propensity score. The secondary objectives were clinical benefit rate (CBR), progression-free survival (PFS), and overall survival (OS). (3) Results: Thirteen pts (54.2%) in the EDP-MM population experienced progestin-related toxicities; in particular, five pts experienced vaginal bleeding (20.8%); four pts experienced weight gain (16.7%); and thromboembolic events, worsening of hypertension, skin rashes, and hyperglycemia were registered in one patient each (4.2%). This led to the discontinuation of megestrol acetate in four pts (16.7%). EDP-M-related toxicities were similar in both groups. No differences in PFS and OS curves were observed; the CBR was 75.0% and 60.4%, respectively. (4) Conclusions: The association of EDP-M + megestrol acetate in ACC pts with a low PS is feasible and well tolerated; its efficacy appeared to be non-inferior to EDP-M administered to pts with a good PS. Full article
(This article belongs to the Special Issue Advances in Adrenocortical Carcinoma Research: Diagnosis, Treatment and Prognosis)
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11 pages, 812 KiB  
Article
Locally Advanced Adrenocortical Carcinoma in Children and Adolescents—Enigmatic and Challenging Cases
by Michaela Kuhlen, Pascal Mier, Marina Kunstreich, Lienhard Lessel, Christoph Slavetinsky, Jörg Fuchs, Guido Seitz, Paul-Martin Holterhus, Stefan A. Wudy, Christian Vokuhl, Michael C. Frühwald, Peter Vorwerk and Antje Redlich
Cancers 2023, 15(17), 4296; https://doi.org/10.3390/cancers15174296 - 28 Aug 2023
Cited by 2 | Viewed by 1118
Abstract
Background: Locally advanced tumors account for approximately 50% of children and adolescents with adrenocortical carcinoma (ACC), and of these, up to 50% relapse. We explored the five-item microscopic score and the pS-GRAS score for guiding management. Methods: Data from children and adolescents with [...] Read more.
Background: Locally advanced tumors account for approximately 50% of children and adolescents with adrenocortical carcinoma (ACC), and of these, up to 50% relapse. We explored the five-item microscopic score and the pS-GRAS score for guiding management. Methods: Data from children and adolescents with COG stage II and III ACC registered in the MET studies were included. The five-item and pS-GRAS score were retrospectively calculated. Results: By December 2021, 55 patients with stage II and III (stage II n = 18, stage III n = 37) had been reported. Median age was 4.3 years [0.1–17.8], median duration of follow-up 6.0 years [0–16.7]. 3-year event-free survival (EFS) rate was 76.5% and 49.8% (p = 0.088), respectively. In stage II tumors, neither the five-item score (p = 0.872) nor pS-GRAS grouping (p = 0.218) had any effect as prognostic factors. In stage III patients, EFS was impaired in tumors with unfavorable histology according to the five-item score (100% vs. 30.8%, p = 0.018). No difference was observed for pS-GRAS groups (p = 0.798). Conclusions: In patients with COG stage III, but not stage II, the five-item score affected EFS. Further studies are needed to identify patients at risk in COG stage II. Full article
(This article belongs to the Special Issue Advances in Adrenocortical Carcinoma Research: Diagnosis, Treatment and Prognosis)
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10 pages, 917 KiB  
Article
The Overall Survival and Progression-Free Survival in Patients with Advanced Adrenocortical Cancer Is Increased after the Multidisciplinary Team Evaluation
by Irene Tizianel, Mario Caccese, Francesca Torresan, Giuseppe Lombardi, Laura Evangelista, Filippo Crimì, Matteo Sepulcri, Maurizio Iacobone, Marta Padovan, Francesca Galuppini, Vittorina Zagonel, Carla Scaroni and Filippo Ceccato
Cancers 2022, 14(16), 3904; https://doi.org/10.3390/cancers14163904 - 12 Aug 2022
Cited by 13 | Viewed by 2537
Abstract
We aimed to evaluate the role of adrenal multidisciplinary team evaluation (MTE) in affecting the overall survival (OS) and progression-free survival (PFS) in patients with adrenocortical carcinoma (ACC). We included in a retrospective monocentric study 47 patients with ACC. We divided our cohort [...] Read more.
We aimed to evaluate the role of adrenal multidisciplinary team evaluation (MTE) in affecting the overall survival (OS) and progression-free survival (PFS) in patients with adrenocortical carcinoma (ACC). We included in a retrospective monocentric study 47 patients with ACC. We divided our cohort into group 1 (without adrenal-MTE discussion, ACC diagnosis from 2004 to 2012, n = 14) and group 2 (diagnosis and beginning of treatments after 2013, all discussed in the adrenal MTE, n = 33). OS was defined by the survival between the first and the last visit, while PFS as the time from the first visit to the progression of the disease. Kaplan–Meier curves were used to compare OS and PFS between Group 1 and Group 2. Group 1stages III–IV (n = 10) presented a shorter median OS than Group 2stages III–IV (25 patients, 4 vs. 31 months, p = 0.023). Likewise, the median PFS was lower in Group 1 as compared to Group 2 (2.9 vs. 17.2 months, p < 0.001). The gain in PFS (6 months) was also confirmed in stage III-IV patients (2.9 vs. 8.7 months, respectively, for Group 1 and Group 2, p = 0.02). Group 1 presented a median PFS of 4 months, while the median PFS of Group 2 was 14.7 months (p = 0.128). In conclusion, we found a significant gain in terms of survival in patients after the MTE discussion in 2013. Therefore, ACC patients should be referred to a tertiary center, ideally from the time of diagnosis, to promptly apply all available treatments, according to the single patient’s clinical history and based on multidisciplinary management. Full article
(This article belongs to the Special Issue Advances in Adrenocortical Carcinoma Research: Diagnosis, Treatment and Prognosis)
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Graphical abstract

14 pages, 1772 KiB  
Article
Targeted Therapy for Adrenocortical Carcinoma: A Genomic-Based Search for Available and Emerging Options
by Daniel Alexander Hescheler, Milan Janis Michael Hartmann, Burkhard Riemann, Maximilian Michel, Christiane Josephine Bruns, Hakan Alakus and Costanza Chiapponi
Cancers 2022, 14(11), 2721; https://doi.org/10.3390/cancers14112721 - 31 May 2022
Cited by 2 | Viewed by 2452
Abstract
In rare diseases such as adrenocortical carcinoma (ACC), in silico analysis can help select promising therapy options. We screened all drugs approved by the FDA and those in current clinical studies to identify drugs that target genomic alterations, also known to be present [...] Read more.
In rare diseases such as adrenocortical carcinoma (ACC), in silico analysis can help select promising therapy options. We screened all drugs approved by the FDA and those in current clinical studies to identify drugs that target genomic alterations, also known to be present in patients with ACC. We identified FDA-approved drugs in the My Cancer Genome and National Cancer Institute databases and identified genetic alterations that could predict drug response. In total, 155 FDA-approved drugs and 905 drugs in clinical trials were identified and linked to 375 genes of 89 TCGA patients. The most frequent potentially targetable genetic alterations included TP53 (20%), BRD9 (13%), TERT (13%), CTNNB1 (13%), CDK4 (7%), FLT4 (7%), and MDM2 (7%). We identified TP53-modulating drugs to be possibly effective in 20–26% of patients, followed by the Wnt signaling pathway inhibitors (15%), Telomelysin and INO5401 (13%), FHD-609 (13%), etc. According to our data, 67% of ACC patients exhibited genomic alterations that might be targeted by FDA-approved drugs or drugs being tested in current clinical trials. Although there are not many current therapy options directly targeting reported ACC alterations, this study identifies emerging options that could be tested in clinical trials. Full article
(This article belongs to the Special Issue Advances in Adrenocortical Carcinoma Research: Diagnosis, Treatment and Prognosis)
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