Temporal Trends in Outcomes in Patients With Adrenocortical Carcinoma: A Multidisciplinary Referral-center Experience

Daher, Marilyne; Varghese, Jeena; Gruschkus, Stephen K.; Jiménez, Camilo; Waguespack, Steven G.; Bedrose, Sara; Altameemi, Lina; Bazerbashi, Hadil; Naing, Aung; Subaiah, Vivek; Campbell, Matthew T.; Shah, Amishi Yogesh; Zhang, Miao; Sheth, Rahul A.; Karam, Jose A.; Wood, Christopher G.; Perrier, Nancy D.; Graham, Paul H.; Lee, Jeffery E.; Habra, Mouhammed Amir

doi:10.1210/clinem/dgac046

Cited by 12 publications

(8 citation statements)

References 40 publications

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“…The median OS was 20 months (CI 9.5-30.5) and was significantly correlated with mENSAT and R status. This is similar to previous studies utilizing mENSAT and R status as prognostic factors [ 7 , 10 , 11 , 14 ] and a recent larger cohort [ 15 ]. A considerable percentage (27%) of patients in our cohort underwent primary resection followed by upfront radiotherapy or metastasectomy for oligometastatic disease to achieve R0 status, emphasizing the importance of these treatments in locally advanced or oligometastatic disease to achieve a disease-free interval and improve prognosis [ 16 , 17 ].…”

Section: Discussionsupporting

confidence: 91%

Prognostic Factors in Advanced Adrenocortical Carcinoma: Summary of a National Referral Center’s 20 years of Experience

Arnon

Grozinsky‐Glasberg

Oleinikov

et al. 2022

Journal of the Endocrine Society

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Introduction Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis for both locally advanced and metastatic disease. Standard treatment with combination Etoposide-Doxorubicin-Cisplatin-Mitotane (EDP-M) is highly toxic and some patients benefit from mitotane monotherapy. However, identification of these patients remains challenging. We present a summary of the Israeli national referral center’s 20 years of experience in treating advanced ACC, with the aim of identifying prognostic factors and assisting in treatment decision making. Methods We conducted a retrospective multivariate analysis of patients treated for metastatic or locally advanced ACC at Hadassah Medical Center between 2000 and 2020 to determine clinical, pathological and treatment factors correlated with overall survival (OS). Results In our cohort of 37 patients, a combination of modified European Network for the study of Adrenal Tumors (mENSAT) staging with either grade and R-Status, or age and symptoms (GRAS) was validated to stratify prognosis (p= 0.01 and p= 0.03, respectively). Patients who underwent R0-resection followed by radiotherapy or metastasectomy for oligometastatic disease had longer OS as compared with patients with residual disease: median OS of 55 months versus 14 months, respectively, hazard ratio 3.1 (CI 1.4–6.7, p= .005). Patients treated with mitotane monotherapy had a significantly better prognosis, yet this result was attenuated in a multivariate analysis controlling for mENSAT and R-status. Of patients treated with EDP-M, 41.4% experienced grade 3 or higher adverse events. Conclusion Advanced ACC patients achieving R0-status have a better prognosis and might benefit from mitotane monotherapy.

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Section: Discussionsupporting

confidence: 91%

Prognostic Factors in Advanced Adrenocortical Carcinoma: Summary of a National Referral Center’s 20 years of Experience

Arnon

Grozinsky‐Glasberg

Oleinikov

et al. 2022

Journal of the Endocrine Society

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“…Patients with locally advanced ACC are less likely to undergo an operation, in particular when managed outside highvolume surgical centers (44), and may be a candidate for a neo-adjuvant approach whose aim is to downstage the tumor. Patients who have responded to induction therapy and are then submitted to surgery may attain a better control of the disease (43,45). Another potential advantage of neoadjuvant therapy is that it gives the possibility of administering nephrotoxic systemic therapy prior to possible nephrectomy.…”

Section: Neo-adjuvant Therapymentioning

confidence: 99%

ENDOCRINE TUMOURS: Our experience with the management of patients with non-metastatic adrenocortical carcinoma

Terzolo

Fassnacht

2022

European Journal of Endocrinology

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Adrenocortical carcinoma (ACC) accounts for a minority of all malignant tumors in adults. Surgery remains the most important therapeutic option for non-metastatic ACC. Whether a subset of patients with small ACC may benefit from minimally invasive surgery remains a debated issue, but we believe that surgeon’s expertise is more important than surgical technique to determine outcome. However, even a state-of-the-art surgery cannot prevent disease recurrence that is determined mainly by specific tumor characteristics. We consider that the concomitant presence of the following features characterizes a cohort of patients at low risk of recurrence, i) R0 resection (microscopically free margin), ii) localized disease (stage I-II ACC), and iii) low grade tumor (ki-67 <10%). After the ADIUVO study, we do not recommend adjuvant mitotane as a routine measure for such patients, who can be managed with active surveillance thus sparing a toxic treatment. Patients at average risk of recurrence should be treated with adjuvant mitotane. For patients at very high risk of recurrence, defined as the presence of at least one of the following: Ki67 >30%, large venous tumor thrombus, R1 resection or stage IV ACC, we increasingly recommend to combine mitotane with four cycles of platinum-based chemotherapy. However, patients at moderate-to-high risk of recurrence should be ideally enrolled in the ongoing ADIUVO2 trial. We do not use frequently adjuvant radiotherapy of the tumor bed at our institutions, and we select for this treatment patients with microscopically, or macroscopically, incomplete resection. On long-term, prospective multicenter trials are required to improve patient care.

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“…The peak of incidence is between 40 and 60 years and women are more often affected (55–60%). In adults, the majority of ACC are sporadic [ 1 , 2 , 3 , 4 ]. Germline mutations of TP53 characterize 50–80% of children with ACC and 4% of adult patients; on the other hand, somatic mutations of TP53 are observed in more than 50% of adult ACC patients, associated with a more aggressive phenotype [ 5 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

“…Recent data suggest that combining other treatments such as mitotane may enhance the efficacy of immunotherapy [ 15 , 16 ]. By far, there are no standardized indications for the application of these treatments in recurrent and/or advanced ACC, and therapeutic options for advanced ACC often result in a poor prognosis [ 2 , 4 ]. The complexity of the disease requires a multidisciplinary approach that is an emerging model of treatment for rare or oncological diseases [ 17 ].…”

Section: Introductionmentioning

confidence: 99%

“…Recently, Daher et al reported in a large study that a multidisciplinary approach to treatment is critical in optimizing outcomes of ACC patients because of the likelihood of a complete resection or the chance to receive more lines of systemic therapy [ 4 ]. However, to the best of our knowledge, the impact of an established multidisciplinary team evaluation (MTE) on clinical practice, with a direct comparison of survival before and after MTE approach in patients with ACC, has not been reported so far.…”

Section: Introductionmentioning

confidence: 99%

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The Overall Survival and Progression-Free Survival in Patients with Advanced Adrenocortical Cancer Is Increased after the Multidisciplinary Team Evaluation

Tizianel

Caccese

Torresan

et al. 2022

Cancers

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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.

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Temporal Trends in Outcomes in Patients With Adrenocortical Carcinoma: A Multidisciplinary Referral-center Experience

Cited by 12 publications

References 40 publications

Prognostic Factors in Advanced Adrenocortical Carcinoma: Summary of a National Referral Center’s 20 years of Experience

Prognostic Factors in Advanced Adrenocortical Carcinoma: Summary of a National Referral Center’s 20 years of Experience

ENDOCRINE TUMOURS: Our experience with the management of patients with non-metastatic adrenocortical carcinoma

The Overall Survival and Progression-Free Survival in Patients with Advanced Adrenocortical Cancer Is Increased after the Multidisciplinary Team Evaluation

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