“…Recently, a discussion on the role of adjuvant radiotherapy has been ongoing and is the scope of reviews and meta-analyses. 46 , 47 Its role is still not exactly clear according to available data, and a recent analysis using the SEER database has shown that 10.5% of 865 patients eligible for analysis were given radiation. 46 According to this analysis patients given radiation for stage III node negative ACC had an OS benefit and, also, another analysis (featuring 75 patients) showed that not only loco regional recurrence-free survival, but also that OS might be positively influenced by adjuvant radiotherapy.…”
Adrenocortical carcinoma (ACC) is a rare malignancy characterized by aggressive biology and potential endocrine activity. Surgery can offer cure for localized disease but more than half of patients relapse and primary unresectable or metastasized disease is frequent. Prognosis of metastatic ACC is still limited, with less than 15% of patients alive at 5 years. Recent advances in understanding the molecular profile of ACC underline the high complexity of this disease, which is characterized by limited drugable molecular targets as well as by a complex interplay between a yet scarcely understood microenvironment and potential endocrine activity. Particularly steroid-excess further complicates therapeutic concepts such as immunotherapy, which have markedly improved outcome in other disease entities. To date, mitotane remains the only approved drug for adjuvant and palliative care in ACC. Standard chemotherapy-based protocols with cisplatin, doxorubicin and etoposide offer only marginal improvement in long-term outcome and the number of clinical trials conducted is low due to the rarity of the disease. In the current review, we summarize principles of oncological management for ACC from localized to advanced disease and discuss novel therapeutic strategies, including targeted therapies such as tyrosine kinase inhibitors and antibodies, immunotherapy with a focus on checkpoint inhibitors, individualized treatment concepts based on molecular characterization by next generation sequencing methods, the role of theranostics and evolvement of adjuvant therapy.
“…Recently, a discussion on the role of adjuvant radiotherapy has been ongoing and is the scope of reviews and meta-analyses. 46 , 47 Its role is still not exactly clear according to available data, and a recent analysis using the SEER database has shown that 10.5% of 865 patients eligible for analysis were given radiation. 46 According to this analysis patients given radiation for stage III node negative ACC had an OS benefit and, also, another analysis (featuring 75 patients) showed that not only loco regional recurrence-free survival, but also that OS might be positively influenced by adjuvant radiotherapy.…”
Adrenocortical carcinoma (ACC) is a rare malignancy characterized by aggressive biology and potential endocrine activity. Surgery can offer cure for localized disease but more than half of patients relapse and primary unresectable or metastasized disease is frequent. Prognosis of metastatic ACC is still limited, with less than 15% of patients alive at 5 years. Recent advances in understanding the molecular profile of ACC underline the high complexity of this disease, which is characterized by limited drugable molecular targets as well as by a complex interplay between a yet scarcely understood microenvironment and potential endocrine activity. Particularly steroid-excess further complicates therapeutic concepts such as immunotherapy, which have markedly improved outcome in other disease entities. To date, mitotane remains the only approved drug for adjuvant and palliative care in ACC. Standard chemotherapy-based protocols with cisplatin, doxorubicin and etoposide offer only marginal improvement in long-term outcome and the number of clinical trials conducted is low due to the rarity of the disease. In the current review, we summarize principles of oncological management for ACC from localized to advanced disease and discuss novel therapeutic strategies, including targeted therapies such as tyrosine kinase inhibitors and antibodies, immunotherapy with a focus on checkpoint inhibitors, individualized treatment concepts based on molecular characterization by next generation sequencing methods, the role of theranostics and evolvement of adjuvant therapy.
“…High-risk ACC patients (R1 or Rx resection or in stage III) was suggested consideration of RT according to the current recommendations ( 24 ). Registry analyses using National Cancer Database (NCD) and the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database also have resulted in conflicting conclusions to the benefit of adjuvant radiation ( 25 , 26 ). A recent population-based publication on the role of adjuvant RT for nonmetastatic ACC used the SEER Database and included 365 patients ( 27 ).…”
ContextAdrenocortical carcinoma (ACC) is rare and have high rates of recurrence and mortality. The role of adjuvant radiation therapy (RT) in localized ACC was controversial.MethodsWe conducted a retrospective study in our center between 2015 and 2021 to evaluate the efficacy and safety of adjuvant RT in localized ACC. Overall survival (OS) and disease-free survival (DFS) were estimated using the Kaplan-Meier method. Cox proportional hazards regression models were used to estimate the independent risk factors. Adverse events associated with RT were documented according to the toxicity criteria of the radiation therapy oncology group (RTOG) and the common terminology criteria for adverse events (CTCAE v5.0).ResultsOf 105 patients with localized ACC, 46 (43.8%) received adjuvant RT after surgery. The median radiation dose was 45.0Gy (range:30.0-50.4) and median follow up time was 36.5 (IQR: 19.7-51.8) months. In comparison to the no adjuvant RT group, patients with adjuvant RT had better 3-year OS (87.9% vs 79.5%, P=0.039), especially for patients with ENSAT I/II stage (P=0.004). Adjuvant RT also improved the median DFS time from 16.5months (95%CI, 12.0-20.9) to 34.6months (95%CI, 16.1-53.0). Toxicity of RT was generally mild and moderate with six grade 3 events.ConclusionsPostoperative adjuvant RT significantly improved OS and DFS compared with the use of surgery alone in resected ACC patients. Although this retrospective study on RT in localized ACC indicates that RT is effective in ACC, its findings need to be prospectively confirmed.
“…Therefore, ENSAT stage I and II were combined. However, this methodology was shared with previous ACC analyses that relied on SEER 5,24 . Fourth, limited detail regarding treatment type was available.…”
Section: Discussionmentioning
confidence: 99%
“…However, this methodology was shared with previous ACC analyses that relied on SEER. 5,24 Fourth, limited detail regarding treatment type was available. Specifically, the SEER database does provide information on adrenalectomy.…”
Background and Objectives: We examined the effect of disease-free interval (DFI) duration on cancer-specific mortality (CSM)-free survival, otherwise known as the effect of conditional survival, in surgically treated adrenocortical carcinoma (ACC) patients.Methods: Within the Surveillance, Epidemiology, and End Results database (2004−2018), 867 ACC patients treated with adrenalectomy were identified.Conditional survival estimates at 5-years were assessed based on DFI duration and according to stage at presentation. Separate Cox regression models were fitted at baseline and according to DFI.Results: Overall, 406 (47%), 285 (33%), and 176 (20%) patients were stage I−II, III and IV, respectively. In conditional survival analysis, providing a DFI of 24 months,
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