2015
DOI: 10.1016/j.ecl.2015.03.001
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Adrenocortical Carcinoma

Abstract: Recent developments in the treatment of adrenocortical carcinoma (ACC) include diagnostic and prognostic risk stratification algorithms, increasing evidence of the impact of historical therapies on overall survival, and emerging targets from integrated epigenomic and genomic analyses. Advances include proper clinical and molecular characterization of all patients with ACC, standardization of proliferative index analyses, referral of these patients to large cancer referral centers at the time of first surgery, … Show more

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Cited by 56 publications
(49 citation statements)
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References 129 publications
(206 reference statements)
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“…ACC is a rare malignancy occurring in about 0.7-2.0 cases per million population per year, and is responsible for 0.2% of all cancer deaths in the United States [ 26 ]. Currently, the main curative treatment for ACC is surgery, with an overall 5-year survival rate for all patients undergoing tumor resection of approximately 40% [ 27 - 29 ]. Surgery for removal of recurrent tumor, including metastatic lesions can also prolong survival ([ 26 ] and unpublished data).…”
Section: Introductionmentioning
confidence: 99%
“…ACC is a rare malignancy occurring in about 0.7-2.0 cases per million population per year, and is responsible for 0.2% of all cancer deaths in the United States [ 26 ]. Currently, the main curative treatment for ACC is surgery, with an overall 5-year survival rate for all patients undergoing tumor resection of approximately 40% [ 27 - 29 ]. Surgery for removal of recurrent tumor, including metastatic lesions can also prolong survival ([ 26 ] and unpublished data).…”
Section: Introductionmentioning
confidence: 99%
“…Overall survival was defined as the time interval between the date of treatment start and the date of death from any cause or the last known alive date. The mENSAT classification and GRAS parameters (22), as defined by grade (Weiss score <6 or >6 or Ki67 <20% or >20%), resection status of the primary, age younger than or older than 50 years, and absence or presence of tumor related or hormone-related symptoms at diagnosis, were used to assess prognosis. The CTCAE v4.03 score was used to assess toxicity.…”
Section: Study Design and Patient Characteristicsmentioning
confidence: 99%
“…Adrenocortical carcinoma (ACC) is an uncommon and heterogeneous endocrine malignant neoplasm with an estimated incidence of 0.5–2.0 cases per million per year (12). It usually occurs in adults with a peak incidence in the fifth decade of life; females are more commonly affected than males (3).…”
Section: Introductionmentioning
confidence: 99%
“…Patients with ACC generally show an unfavorable prognosis and a marked inter-individual variability in disease progression, recurrence, and overall survival (12). Because of the difficulty in differentiating the benign from malignant adrenocortical tumors, various multi-parametric diagnostic algorithms, such as the Weiss, Hough, van Slooten, modified Weiss scoring systems, and reticulin algorithm, have been used (3132).…”
Section: Introductionmentioning
confidence: 99%
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