performed for unrelated clinical reasons ( 1 , 2 ). On the other side, adrenocortical carcinomas (ACC) are rare but usually have an aggressive behavior and a poor prognosis ( 1 , 3 , 4 , 5 , 6 ). According to the ENSAT classification, the 5-year disease
Sofia S Pereira, Mariana P Monteiro, Sonir R Antonini and Duarte Pignatelli
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
cases and the majority is adrenocortical adenomas or hyperplasia. A small fraction of MEN1 patients developed adrenocortical carcinoma (ACC) ( 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 ). In fact, ACC in general is a rare malignancy with high mortality
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.
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
Sofia S Pereira, Tiago Morais, Madalena M Costa, Mariana P Monteiro and Duarte Pignatelli
prevalence of more than 4% in the adult population; in contrast, adrenocortical carcinomas (ACCs) are rare, having an incidence of between 0.5 and 2 per million, and are generally highly aggressive, with a poor prognosis that is expressed by a 5-year survival
Eric Seidel, Gudrun Walenda, Clemens Messerschmidt, Benedikt Obermayer, Mirko Peitzsch, Paal Wallace, Rohini Bahethi, Taekyeong Yoo, Murim Choi, Petra Schrade, Sebastian Bachmann, Gerhard Liebisch, Graeme Eisenhofer, Dieter Beule and Ute I Scholl
, Berlin) for bioinformatics support. References 1 Fassnacht M Kroiss M Allolio B . Update in adrenocortical carcinoma . Journal of Clinical Endocrinology and Metabolism 2013 4551 – 4564 . ( https://doi.org/10.1210/jc.2013-3020 ) 2
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
Anne Jouinot, Bernard Royer, Etienne Chatelut, Sotheara Moeung, Guillaume Assié, Audrey Thomas-Schoemann, Jérôme Bertherat, François Goldwasser and Benoit Blanchet
Introduction Adrenocortical carcinoma (ACC) is a rare malignancy (1 per million each year) with a poor prognosis ( 1 ). Even after complete surgery, up to 70% of patients will develop recurrence ( 1 ). Medical treatments show limited efficacy
Manjunath Goroshi, Swati S Jadhav, Vijaya Sarathi, Anurag R Lila, Virendra A Patil, Ravikumar Shah, Priya Hira, Rajaram Sharma, Shettepppa Goroshi, Gwendolyn Fernandes, Amey Rojekar, Abhay Dalvi, Ganesh Bakshi, Gagan Prakash, Nalini S Shah and Tushar R Bandgar
adrenocortical carcinoma (ACC, 8%), phaeochromocytoma (PCC, 7%) and adrenal metastasis (AM, 5%) are other less common masses ( 1 ). Most crucial step in management of an adrenal incidentaloma is to differentiate a benign ‘leave-alone’ lesion, from the one that
L Ghataore, I Chakraborti, S J Aylwin, K-M Schulte, D Dworakowska, P Coskeran and N F Taylor
Introduction Mitotane (o,p'-DDD) plays an important role in adjuvant therapy of adrenocortical carcinoma (ACC) and in advanced stage disease (1) . This application was first reported in 1959 by Bergenstal et al . (2) , but mitotane has only been