Influence of hormonal functional status on survival in adrenocortical carcinoma: systematic review and meta-analysis

Vanbrabant, T; Fassnacht, Martin; Assié, Guillaume; Dekkers, Olaf M.

doi:10.1530/eje-18-0450

Cited by 74 publications

(55 citation statements)

References 32 publications

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“…In a formal comparison, the ENSAT staging (29) was slightly superior to the UICC staging (88). Additionally, the association between hypercortisolism and mortality was consistent and remained with a positive hazard ratio after adjustments for tumor stage HR: 1.71, 95% CI: 1.18-2.47 (35). Ki67 was studied in five publications, showing worse prognosis with increasing Ki67 in all studies.…”

Section: Clinical Questionmentioning

confidence: 64%

“…In summary, we included 18 publications for clinical question 1 (diagnostics for ACC), 35 studies for clinical question 2 (prognosis), 10 publications for clinical question 3 (adjuvant therapy) and 48 publications for clinical question 4 (recurrent/advanced disease). The review of hormonal overproduction as prognostic factor was published as stand-alone paper (35). For question 3, we included one study after having been provided with baseline characteristics and adjusted estimates for mitotane therapy not reported in the original publication (36).…”

Section: Description Of Search and Selection Of Literaturementioning

confidence: 99%

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European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors

Fassnacht

Dekkers

Else

et al. 2018

European Journal of Endocrinology

690

867

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Adrenocortical carcinoma (ACC) is a rare and in most cases steroid hormone-producing tumor with variable prognosis. The purpose of these guidelines is to provide clinicians with best possible evidence-based recommendations for clinical management of patients with ACC based on the GRADE (Grading of Recommendations Assessment, Development and Evaluation) system. We predefined four main clinical questions, which we judged as particularly important for the management of ACC patients and performed systematic literature searches: (A) What is needed to diagnose an ACC by histopathology? (B) Which are the best prognostic markers in ACC? (C) Is adjuvant therapy able to prevent recurrent disease or reduce mortality after radical resection? (D) What is the best treatment option for macroscopically incompletely resected, recurrent or metastatic disease? Other relevant questions were discussed within the group. Selected Recommendations: (i) We recommend that all patients with suspected and proven ACC are discussed in a multidisciplinary expert team meeting. (ii) We recommend that every patient with (suspected) ACC should undergo careful clinical assessment, detailed endocrine work-up to identify autonomous hormone excess and adrenal-focused imaging. (iii) We recommend that adrenal surgery for (suspected) ACC should be performed only by surgeons experienced in adrenal and oncological surgery aiming at a complete en bloc resection (including resection of oligo-metastatic disease). (iv) We suggest that all suspected ACC should be reviewed by an expert adrenal pathologist using the Weiss score and providing Ki67 index. (v) We suggest adjuvant mitotane treatment in patients after radical surgery that have a perceived high risk of recurrence (ENSAT stage III, or R1 resection, or Ki67 >10%). (vi) For advanced ACC not amenable to complete surgical resection, local therapeutic measures (e.g. radiation therapy, radiofrequency ablation, chemoembolization) are of particular value. However, we suggest against the routine use of adrenal surgery in case of widespread metastatic disease. In these patients, we recommend either mitotane monotherapy or mitotane, etoposide, doxorubicin and cisplatin depending on prognostic parameters. In selected patients with a good response, surgery may be subsequently considered. (vii) In patients with recurrent disease and a disease-free interval of at least 12 months, in whom a complete resection/ablation seems feasible, we recommend surgery or alternatively other local therapies. Furthermore, we offer detailed recommendations about the management of mitotane treatment and other supportive therapies. Finally, we suggest directions for future research.

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Section: Clinical Questionmentioning

confidence: 64%

Section: Description Of Search and Selection Of Literaturementioning

confidence: 99%

European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors

Fassnacht

Dekkers

Else

et al. 2018

European Journal of Endocrinology

690

867

View full text Add to dashboard Cite

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“…The association between cortisol-producing ACC and mortality shows a positive hazard ratio of 1.71 (95% confidence interval of 1.18-2.47) after adjusting for tumor stage. Further, ENSAT guidelines define cortisol-producing ACC as a poor prognostic factor [9,10]. However, the mechanism by which patients with cortisol-producing ACC have a poor prognosis has not been elucidated.…”

Section: Introductionmentioning

confidence: 99%

CCNB2 and AURKA overexpression may cause atypical mitosis in Japanese cortisol-producing adrenocortical carcinoma with TP53 somatic variant

et al. 2020

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Background Many genomic analyses of cortisol-producing adrenocortical carcinoma (ACC) have been reported, but very few have come from East Asia. The first objective of this study is to verify the genetic difference with the previous reports by analyzing targeted deep sequencing of 7 Japanese ACC cases using next-generation sequencing (NGS). The second objective is to compare the somatic variant findings identified by NGS analysis with clinical and pathological findings, aiming to acquire new knowledge about the factors that contribute to the poor prognosis of ACC and to find new targets for the treatment of ACC. Method DNA was extracted from ACC tissue of seven patients and two reference blood samples. Targeted deep sequencing was performed using the MiSeq system for 12 genes, and the obtained results were analyzed using MuTect2. The hypothesis was obtained by integrating the somatic variant findings with clinical and pathological data, and it was further verified using The Cancer Genome Atlas (TCGA) dataset for ACC. Results Six possible pathogenic and one uncertain significance somatic variants including a novel PRKAR1A (NM_002734.4):c.545C>A (p.T182K) variant were found in five of seven cases. By integrating these data with pathological findings, we hypothesized that cases with TP53 variants were more likely to show atypical mitotic figures. Using TCGA dataset, we found

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“…Generally a poor prognosis is expected in this type of malignancy despite the progress of technology and therapy (20). Cortisol producing subtype of adrenal cancer has the highest rate of mortality (as was this case) when compared to the subtypes with other endocrine profile (21).…”

Section: Poor Prognosis Of Adrenal Cancermentioning

confidence: 77%

Pale skin: An adrenal cancer?

Valea¹,

Cârșote²,

Петрова³

et al. 2019

Ro J Med Pract.

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Adrenal carcinoma is a complex orphan disease with a very poor prognosis. The evolution is rapid and in cases with suggestive symptoms the diagnosis may be established earlier in order to improve the outcome. One third of the patients are negative for a clear hormonal expression or they have an atypical presentation. We introduce an adult patient diagnosed with adrenal carcinoma and a rapid lethal outcome who initially presented with pale skin and non-specific abdominal pain. A multidisciplinary team is essential for adequate diagnosis and prompt intervention. Severe bleeding at the level of the adrenal carcinoma causing symptomatic anaemia is a rare event. The prognosis in adrenocortical carcinoma is severe and the presence of humoral hypercalcemia represents a sign of short survival time.

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Influence of hormonal functional status on survival in adrenocortical carcinoma: systematic review and meta-analysis

Cited by 74 publications

References 32 publications

European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors

European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors

CCNB2 and AURKA overexpression may cause atypical mitosis in Japanese cortisol-producing adrenocortical carcinoma with TP53 somatic variant

Pale skin: An adrenal cancer?

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