Tobias Else1, Andrew R Williams, Aaron Sabolch, Shruti Jolly, Barbra S Miller, Gary D Hammer. 1. Department of Internal Medicine (T.E., G.D.H.), Division of Metabolism, Endocrinology, and Diabetes, Department of Radiation Oncology (A.S., S.J.), and Division of Endocrine Surgery (B.S.M.), Section of General Surgery, University of Michigan Hospital and Health Systems, Ann Arbor, Michigan 48106; and University of Michigan Medical School (A.R.W.), Ann Arbor, Michigan 48109.
Abstract
CONTEXT: Adrenocortical carcinoma is a rare malignant endocrine neoplasia. Studies regarding outcome and prognostic factors rely on fairly small studies. Here we summarize the experience with patients with a diagnosis of adrenocortical carcinoma from a large tertiary referral center. OBJECTIVE: The objective of the study was to identify prognostic factors in patients with adrenocortical carcinoma and evaluate adjuvant treatment strategies. DESIGN: Patient data were collected in a retrospective single-center study. Epidemiological, patient, and tumor characteristics were analyzed for prognostic factors regarding overall and recurrence-free survival in Cox regression models (multivariable and univariable). RESULTS: Three hundred ninety-one adult patients with the diagnosis of adrenocortical carcinoma were identified. Median overall survival was 35.2 months. Cortisol production [hazard ratio (HR) 1.4, HR 1.5], tumor stage (HR stage 3 of 2.1 and 2.1, HR stage 4 of 4.8), and tumor grade (HR 2.4 and 2.0) were identified as negative prognostic factors (HR for death, HR for recurrence). Mitotane therapy increases recurrence-free survival, an effect that was significantly further improved by adjuvant radiation therapy but did not impact overall survival. Patients with open adrenalectomy had improved overall survival. CONCLUSIONS: This study increases the evidence for adverse risk factors (cortisol production, high tumor stage, and high tumor grade) and suggests the following therapy approach: adrenocortical carcinoma patients should be treated with open adrenalectomy. Adjuvant therapy, particularly mitotane therapy in conjunction with radiation, should be considered to delay tumor recurrence.
CONTEXT: Adrenocortical carcinoma is a rare malignant endocrine neoplasia. Studies regarding outcome and prognostic factors rely on fairly small studies. Here we summarize the experience with patients with a diagnosis of adrenocortical carcinoma from a large tertiary referral center. OBJECTIVE: The objective of the study was to identify prognostic factors in patients with adrenocortical carcinoma and evaluate adjuvant treatment strategies. DESIGN:Patient data were collected in a retrospective single-center study. Epidemiological, patient, and tumor characteristics were analyzed for prognostic factors regarding overall and recurrence-free survival in Cox regression models (multivariable and univariable). RESULTS: Three hundred ninety-one adult patients with the diagnosis of adrenocortical carcinoma were identified. Median overall survival was 35.2 months. Cortisol production [hazard ratio (HR) 1.4, HR 1.5], tumor stage (HR stage 3 of 2.1 and 2.1, HR stage 4 of 4.8), and tumor grade (HR 2.4 and 2.0) were identified as negative prognostic factors (HR for death, HR for recurrence). Mitotane therapy increases recurrence-free survival, an effect that was significantly further improved by adjuvant radiation therapy but did not impact overall survival. Patients with open adrenalectomy had improved overall survival. CONCLUSIONS: This study increases the evidence for adverse risk factors (cortisol production, high tumor stage, and high tumor grade) and suggests the following therapy approach: adrenocortical carcinomapatients should be treated with open adrenalectomy. Adjuvant therapy, particularly mitotane therapy in conjunction with radiation, should be considered to delay tumor recurrence.
Authors: F Fausto Palazzo; Frederic Sebag; Mauricio Sierra; Giuseppe Ippolito; Philippe Souteyrand; Jean-François Henry Journal: World J Surg Date: 2006-05 Impact factor: 3.352
Authors: M Clarke; R Collins; S Darby; C Davies; P Elphinstone; V Evans; J Godwin; R Gray; C Hicks; S James; E MacKinnon; P McGale; T McHugh; R Peto; C Taylor; Y Wang Journal: Lancet Date: 2005-12-17 Impact factor: 79.321
Authors: Alfredo Berruti; Massimo Terzolo; Paola Sperone; Anna Pia; Silvia Della Casa; David J Gross; Carlo Carnaghi; Paolo Casali; Francesco Porpiglia; Franco Mantero; Giuseppe Reimondo; Alberto Angeli; Luigi Dogliotti Journal: Endocr Relat Cancer Date: 2005-09 Impact factor: 5.678
Authors: J P Luton; S Cerdas; L Billaud; G Thomas; B Guilhaume; X Bertagna; M H Laudat; A Louvel; Y Chapuis; P Blondeau Journal: N Engl J Med Date: 1990-04-26 Impact factor: 91.245
Authors: Isobel C Mouat; Kei Omata; Andrew S McDaniel; Namita G Hattangady; Debnita Talapatra; Andi K Cani; Daniel H Hovelson; Scott A Tomlins; William E Rainey; Gary D Hammer; Thomas J Giordano; Tobias Else Journal: Endocr Relat Cancer Date: 2019-02 Impact factor: 5.678
Authors: Siyuan Zheng; Andrew D Cherniack; Ninad Dewal; Richard A Moffitt; Ludmila Danilova; Bradley A Murray; Antonio M Lerario; Tobias Else; Theo A Knijnenburg; Giovanni Ciriello; Seungchan Kim; Guillaume Assie; Olena Morozova; Rehan Akbani; Juliann Shih; Katherine A Hoadley; Toni K Choueiri; Jens Waldmann; Ozgur Mete; A Gordon Robertson; Hsin-Ta Wu; Benjamin J Raphael; Lina Shao; Matthew Meyerson; Michael J Demeure; Felix Beuschlein; Anthony J Gill; Stan B Sidhu; Madson Q Almeida; Maria C B V Fragoso; Leslie M Cope; Electron Kebebew; Mouhammed A Habra; Timothy G Whitsett; Kimberly J Bussey; William E Rainey; Sylvia L Asa; Jérôme Bertherat; Martin Fassnacht; David A Wheeler; Gary D Hammer; Thomas J Giordano; Roel G W Verhaak Journal: Cancer Cell Date: 2016-05-09 Impact factor: 31.743
Authors: Georgios Antonios Margonis; Yuhree Kim; Jason D Prescott; Thuy B Tran; Lauren M Postlewait; Shishir K Maithel; Tracy S Wang; Douglas B Evans; Ioannis Hatzaras; Rivfka Shenoy; John E Phay; Kara Keplinger; Ryan C Fields; Linda X Jin; Sharon M Weber; Ahmed Salem; Jason K Sicklick; Shady Gad; Adam C Yopp; John C Mansour; Quan-Yang Duh; Natalie Seiser; Carmen C Solorzano; Colleen M Kiernan; Konstantinos I Votanopoulos; Edward A Levine; George A Poultsides; Timothy M Pawlik Journal: Ann Surg Oncol Date: 2015-08-19 Impact factor: 5.344