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Sofia S Pereira, Tiago Morais, Madalena M Costa, Mariana P Monteiro and Duarte Pignatelli

rate of between 16 and 38% (1, 3, 4, 5) . The correct diagnosis of adrenocortical tumors (ACTs) is, therefore, understandably of growing importance. ACTs can be divided into functioning and nonfunctioning tumors based on whether they secrete steroids

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Monica F Stecchini, Zilda Braid, Candy B More, Davi C Aragon, Margaret Castro, Ayrton C Moreira and Sonir R Antonini

conditions associated with androgen excess, usually after initiation of treatment of the underlying disease ( 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 ). In this context, androgen-secreting adrenocortical tumors (ACTs), which

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Sofia S Pereira, Mariana P Monteiro, Sonir R Antonini and Duarte Pignatelli

Adrenocortical tumors Adrenal cortex tumors (ACT) are common tumors with a reported prevalence above 4% in most populations ( 1 ). However, the majority of ACT are benign, non-functioning and incidentally discovered during imaging studies

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Milène Tetsi Nomigni, Sophie Ouzounian, Alice Benoit, Jacqueline Vadrot, Frédérique Tissier, Sylvie Renouf, Hervé Lefebvre, Sophie Christin-Maitre and Estelle Louiset

-secreting ovarian tumors such as Leydig–Sertoli tumors (9, 10, 11) . In contrast, high levels of DHEAS are indicative of adrenocortical adenoma of zona reticularis (ZR). In most cases, adrenal androgen-secreting tumors are malignant. In particular, concomitant

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Weixi Wang, Rulai Han, Lei Ye, Jing Xie, Bei Tao, Fukang Sun, Ran Zhuo, Xi Chen, Xiaxing Deng, Cong Ye, Hongyan Zhao and Shu Wang

offspring of a consanguineous marriage. Four of the eight mutation carriers were evaluated for MEN1-related tumors and three members were affected by adrenal tumors. Two of which had adrenocortical adenomas (ACAs) and the proband’s daughter was affected with

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Maria Cristina De Martino, Richard A Feelders, Claudia Pivonello, Chiara Simeoli, Fortuna Papa, Annamaria Colao, Rosario Pivonello and Leo J Hofland

Introduction Adrenocortical carcinomas (ACCs) are rare tumors with scant treatment options for which new treatments are required ( 1 , 2 , 3 ). The limited efficacy of conventional antineoplastic treatment in ACCs increases the need for

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Anne Jouinot, Bernard Royer, Etienne Chatelut, Sotheara Moeung, Guillaume Assié, Audrey Thomas-Schoemann, Jérôme Bertherat, François Goldwasser and Benoit Blanchet

patients’ recruitment and samples collection. References 1 Jouinot A Bertherat J. Management of endocrine disease: adrenocortical carcinoma: differentiating the good from the poor prognosis tumors . European Journal of Endocrinology 2018 178

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Jung Soo Lim, Seung-Eun Lee, Jung Hee Kim and Jae Hyeon Kim

Purpose: To evaluate the clinical characteristics and prognostic factors in patients with adrenocortical carcinoma (ACC) in South Korea.

Methods: A nationwide, registry-based survey was conducted to identify pathologically proven ACC at 25 tertiary care centers in South Korea between 2000 and 2014. Cox proportional hazard model and log-rank test were adopted for survival analysis.

Results: Two hundred four patients with ACC were identified, with a median follow-up duration of 20 months (IQR 5-52 months). The median age at diagnosis was 51.5 years (IQR 40-65.8 years), and ACC was prevalent in women (n=110, 53.9%). Abnormal pain was the most common clinical symptom (n=70, 40.2%), and ENSAT stage 2 was most common (n=62, 30.4%) at the time of diagnosis. One hundred sixty-nine patients underwent operation, while 17 were treated with other modalities. The remission rate was 48%, and median recurrence-free survival time was 46 months. Estimated 5-year recurrence-free rate was 44.7%. There were more women, large tumor, atypical mitosis, venous invasion, and higher mitotic count in cancer recurrence group. Estimated 5-year overall survival and disease-specific survival rates were 64.5% and 70.6%, respectively. Higher ENSAT stage and advanced pathologic characteristics were risk factors for all-cause mortality of ACC. Large tumor size and cortisol-secreting tumor were additional risk factors for ACC-specific death.

Conclusions: We report the first epidemiologic study regarding ACC in an Asian population. ENSAT stage 4; lymph node involvement; non-operative group; and invasion of vein, sinusoid, or capsule were associated with an increased risk for all-cause mortality.

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Deborah Cosentini, Salvatore Grisanti, Alberto Dalla Volta, Marta Laganà, Chiara Fiorentini, Paola Perotti, Sandra Sigala and Alfredo Berruti

Editorial Adrenocortical carcinoma (ACC) is a rare endocrine neoplasia characterized by an overall dismal prognosis and its clinical manifestations are the consequence of either steroid excess or tumor mass progression ( 1 ). Surgery is the

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Wafaa M Rashed, Anas Saad, Muneer Al-Husseini, Ahmed Mahmoud Galal, Assem Mohamed Ismael, Ahmed M Al-Tayep, Ayman El Shafie, Mahmoud Ahmed Ali and Ahmad Samir Alfaar

adrenal gland tumors and BRCA1 or BRCA2 mutations. These genetic associations should be investigated in large longitudinal study of primary breast cancer and adrenal gland tumor as a second malignancy. The incidence of adrenocortical carcinomas (ACCs) as