References
Fassnacht M, Libé R, Kroiss M et al (2011) Adrenocortical carcinoma: a clinician’s update. Nat Rev Endocrinol 7:323–335
Else T, Kim AC, Sabolch A et al (2014) Adrenocortical carcinoma. Endocr Rev 35:282–326
Swiglo BA, Murad MH, Schünemann HJ et al (2008) A case for clarity, consistency, and helpfulness: state of-the-art clinical practice guidelines in endocrinology using the grading of recommendations, assessment, development, and evaluation system. J Clin Endocrinol Metabol 93:666–673
Fassnacht M, Kenn W, Allolio B (2004) Adrenal tumors: how to establish malignancy? J Endocrinol Invest 27:387–399
Tauchmanova L, Colao A, Marzano LA et al (2004) Adrenocortical carcinomas: twelve-year prospective experience. World J Surg 28:896–903
Arlt W, Biehl M, Taylor AE et al (2011) Urine steroid metabolomics as a biomarker tool for detecting malignancy in adrenal tumors. J Clin Endocrinol Metab 96:3775–3784
Nieman LK, Biller BMK, Findling JW et al (2008) The diagnosis of Cushing’s syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metabol 93:1526–1540
Terzolo M, Bovio S, Pia A et al (2009) Management of adrenal incidentaloma. Best Pract Res Clin Endocrinol Metabol 23:233–243
Chiodini I (2011) Clinical review: diagnosis and treatment of subclinical hypercortisolism. J Clin Endocrinol Metab 96:1223–1236
Terzolo M, Pia A, Reimondo G (2012) Subclinical Cushing’s syndrome: definition and management. Clin Endocrinol 76:12–18
Vassiliadi DA, Tsagarakis S (2011) Endocrine incidentalomas––challenges imposed by incidentally discovered lesions. Nat Rev Endocrinol 7:668–680
Terzolo M, Stigliano A, Chiodini I et al (2011) AME position statement on adrenal incidentaloma. Eur J Endocrinol 164:851–870
Morelli V, Masserini B, Salcuni AS et al (2010) Subclinical hypercortisolism: correlation between biochemical diagnostic criteria and clinical aspects. Clin Endocrinol 73:161–166
Eller-Vainicher C, Morelli V, Salcuni AS et al (2010) Accuracy of several parameters of hypothalamic-pituitary-adrenal axis activity in predicting before surgery the metabolic effects of the removal of an adrenal incidentaloma. Eur J Endocrinol 163:925–935
Seccia TM, Fassina A, Nussdorfer G et al (2005) Aldosterone-producing adrenocortical carcinoma: an unusual cause of Conn’s syndrome with an ominous clinical course. End Relat Cancer 12:149–159
Fischer E, Beuschlein F, Bidlingmaier M, Reincke M (2011) Commentary on the Endocrine Society Practice Guidelines: consequences of adjustment of antihypertensive medication in screening of primary aldosteronism. Rev Endocr Metab Disord 12:43–48
Funder JW, Carey RM, Fardella C et al (2008) Case detection, diagnosis, and treatment of patients with primary aldosteronism: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab 93:3266–3281
Libè R, Fratticci A, Bertherat J (2007) Adrenocortical cancer: pathophysiology and clinical management. Endocr Relat Cancer 14:13–28
Terzolo M, Alì A, Osella G et al (2000) The value of dehydroepiandrosterone sulfate measurement in the differentiation between benign and malignant adrenal masses. Eur J Endocrinol 142:611–617
Lenders JWM, Duh QY, Eisenhofer G et al (2014) Pheochromocytoma and paraganglioma: an Endocrine Society clinical practice guideline. J Clin Endocr Metab 99:1915–1942
Fishman EK, Deutch BM, Hartman DS et al (1987) Primary adrenocortical carcinoma: CT evaluation with clinical correlation. AJR 148:531–535
Zhang HM, Perrier ND, Grubbs EG et al (2012) CT features and quantification of the characteristics of adrenocortical carcinomas on unenhanced and contrast-enhanced studies. Clin Radiol 67:38–46
Park BK, Kim CK, Kwon GY et al (2007) Re-evaluation of pheochromocytomas on delayed contrast enhanced CT: washout enhancement and other imaging features. Eur Radiol 17:2804–2809
Copeland PM (1983) The incidentally discovered adrenal mass. Ann Intern Med 98:940–945
Terzolo M, Ali A, Osella G et al (1997) Prevalence of adrenal carcinoma among incidentally discovered adrenal masses. A retrospective study from 1989 to 1994. Gruppo Piemontese Incidentalomi Surrenalici. Arch Surg 132:914–919
Lee MJ, Hahn PF, Papanicolau N et al (1991) Benign and malignant adrenal masses: CT distinction with attenuation coefficients, size, and observer analysis. Radiology 179:415–418
Boland GW, Lee MJ, Gazelle GS et al (1998) Characterization of adrenal masses using unenhanced CT: an analysis of the CT literature. AJR 171:201–204
Blake MA, Kalra MK, Sweeney A et al (2006) Distinguishing benign from malignant adrenal masses: multidetector row CT protocol with 10-minute delay. Radiology 238:578–585
Blake MA, Cronin CG, Boland GW (2010) Adrenal imaging. AJR 194:1450–1460
Petersenn S, Richter PA, Broemel T et al (2015) Computed tomography criteria for discrimination of adrenal adenomas and adrenocortical carcinomas: analysis of the German ACC registry. Eur J Endocrin 172:415–422
Korobkin M, Brodeur FJ, Francis IR et al (1998) CT time-attenuation washout curves of adrenal adenomas and nonadenomas. AJR Am J Roentgenol 170:747–752
Peña CS, Boland GW, Hahn PF et al (2000) Characterization of indeterminate (lipid-poor) adrenal masses: use of washout characteristics at contrast-enhanced CT. Radiology 217:798–802
Caoili EM, Korobkin M, Francis IR et al (2002) Adrenal masses: characterization with combined unenhanced and delayed enhanced CT. Radiology 222:629–633
Szolar DH, Korobkin M, Reittner P et al (2005) Adrenocortical carcinomas and adrenal pheochromocytomas: mass and enhancement loss evaluation at delayed contrast-enhanced CT. Radiology 234:479–485
Sangwaiya MJ, Boland JW, Cronin CG et al (2010) Incidental adrenal lesions: accuracy of characterization with contrast-enhanced washout multidetector CT-10-minute delayed imaging protocol revisited in a large patient cohort. Radiology 256:504–510
Chiche L, Dousset B, Kieffer E, Chapuis Y (2006) Adrenocortical carcinoma extending into the inferior vena cava: Presentation of a 15-patient series and review of the literature Surgery 139:15–27
Park BK, Kim CK, Kim B, Lee JH (2007) Comparison of delayed enhanced CT and chemical shift MR for evaluating hyperattenuating incidental adrenal masses. Radiology 243:760–765
Ferrozzi F, Bova D (1995) CT and MR demonstration of fat within an adrenal cortical carcinoma. Abdom Imaging 20:272–274
Heye S, Woestenborghs H, Van Kerkhove F et al (2005) Adrenocortical carcinoma with fat inclusion: case report. Abdom Imaging 30:641–643
Marin D, Soher BJ, Dale BM et al (2010) Characterization of adrenal lesions: comparison of 2D and 3D dual gradient-echo MR imaging at 3 T-preliminary results. Radiology 254:179–184
Faria JF, Goldman SM, Szejnfeld J et al (2007) Adrenal masses: characterization with in vivo proton MR spectroscopy—initial experience. Radiology 245:788–797
Boland GW, Dwamena BA, Sangwaiya MJ et al (2011) Characterization of adrenal masses by using FDG PET: a systematic review and meta-analysis of diagnostic test performance. Radiology 259:117–126
Leboulleux S, Dromain C, Bonniaud G et al (2006) Diagnostic and prognostic value of 18-fluorodeoxyglucose positron emission tomography in adrenocortical carcinoma: a prospective comparison with computed tomography. J Clin Endocrinol Metab 91:920–925
Wong KK, Arabia M, Zerizerc I et al (2011) Role of positron emission tomography/computed tomography in adrenal and neuroendocrine tumors: fluorodeoxyglucose and non-fluorodeoxyglucose tracers. Nucl Med Commun 32:764–781
Leboulleux S, Deandreis D, Escourrou C et al (2011) Fluorodesoxyglucose uptake in the remaining adrenal glands during the follow-up of patients with adrenocortical carcinoma: do not consider it as malignancy. Eur J Endocrinol 164:89–94
Hennings J, Lindhe O, Bergstrom M et al (2006) [11C]metomidate positron emission tomography of adrenocortical tumors in correlation with histopathological findings. J Clin Endocrinol Metab 91:1410–1414
Hahner S, Kreissl MC, Fassnacht M et al (2013) Functional Characterization of Adrenal Lesions Using [123I]IMTO-SPECT/CT. J Clin Endocrinol Metab 98:1508–1518
Kreissl MC, Schirbel A, Fassnacht M et al (2013) [123I]Iodometomidate imaging in adrenocortical carcinoma. J Clin Endocrinol Metab 98:2755–2764
Tirabassi G, Kola B, Ferretti M et al (2012) Fine-needle aspiration cytology of adrenal masses: re-assessment with histological confirmation. J Endocrinol Invest 35:590–594
Sasano H, Shizawa S, Suzuki T et al (1995) Transcription factor adrenal 4 binding protein as a marker of adrenocortical malignancy. Hum Pathol 26:1154–1156
Sbiera S, Schmull S, Assie G et al (2010) High diagnostic and prognostic value of steroidogenic factor-1 expression in adrenal tumors. J Clin Endocrinol Metab 95:161–171
Sangoi AR, Fujiwara M, West RB et al (2011) Immunohistochemical distinction of primary adrenal cortical lesions from metastatic clear cell renal cell carcinoma: a study of 248 cases. Am J Surg Pathol 5:678–686
Duregon E, Fassina A, Volante M et al (2013) The reticulin algorithm for adrenocortical tumor diagnosis: a multicentric validation Study on 245 unpublished cases. Am J Surg Pathol 37:1433–1440
Weiss LM, Medeiros LJ, Vickery AL Jr (1989) Pathologic features of prognostic significance in adrenocortical carcinoma. Am J Surg Pathol 13:202–206
Tissier F, Aubert S, Leteurtre E et al (2012) Adrenocortical tumors: improving the practice of the Weiss system through virtual microscopy: a National Program of the French Network INCa-COMETE. Am J Surg Pathol 36:1194–1201
Macfarlane DA (1958) Cancer of the adrenal cortex: the natural history, prognosis and treatment in the study of fifty cases. Ann R Coll Surg Engl 109:613–618
Bradley EL 3rd (1975) Primary and adjunctive therapy in carcinoma of the adrenal cortex. Surg Gynecol Obstet 141:507–516
Sullivan M, Boileau M, Hodges CV (1978) Adrenal cortical carcinoma. J Urol 120:660–665
Berruti A, Baudin E, Gelderblom H et al (2012) Adrenal cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 23(Supplement 7):vii131–vii138
Terzolo M, Ardito A, Zaggia B et al (2012) Management of adjuvant mitotane therapy following resection of adrenal cancer. Endocrine 42:521–525
Bourdeau I, Mackenzie-Feder J, Lacroix A (2013) Recent advances in adrenocortical carcinoma in adults. Curr Opin Endocrinol Diabetes Obes 20:192–197
Icard P, Chapuis Y, Andreassian B et al (1992) Adrenocortical carcinoma in surgically treated patients: a retrospective study on 156 cases by the French Association of Endocrine Surgery. Surgery 112:972–979
Lee JE, Berger DH, el-Naggar AK et al (1995) Surgical management, DNA content, and patient survival in adrenal cortical carcinoma. Surgery 118:1090–1098
Delellis RA, Lloyd RV, Heitz PU, Eng C (eds) (2004) Pathology and genetics of tumours of endocrine organs. IARC, Lyon
Fassnacht M, Johanssen S, Quinkler M et al (2009) German adrenocortical carcinoma registry group; European Network for the Study of Adrenal Tumors. Limited prognostic value of the 2004 International Union Against Cancer staging classification for adrenocortical carcinoma: proposal for a Revised TNM Classification. Cancer 115:243–250
Lughezzani G, Sun M, Perrotte P et al (2010) The European Network for the Study of Adrenal Tumors staging system is prognostically superior to the International Union Against Cancer staging system: a North American validation. Eur J Cancer 46:713–719
Terzolo M, Daffara F, Ardito A et al (2014) Management of adrenal cancer: a 2013 update. J Endocrinol Invest 37:207–217
Morimoto R, Satoh F, Murakami O et al (2008) Immunohistochemistry of a proliferation marker Ki67/MIB1 in adrenocortical carcinomas: Ki67/MIB1 labeling index is a predictor for recurrence of adrenocortical carcinomas. Endocr J 55:49–55
van’t Sant HP, Bouvy ND, Kazemier G et al (2007) The prognostic value of two different histopathological scoring systems for adrenocortical carcinomas. Histopathol 51:239–245
Terzolo M, Angeli A, Fassnacht M et al (2007) Adjuvant mitotane treatment for adrenocortical carcinoma. New Engl Med 356:2372–2380
Volante M, Bollito E, Sperone P et al (2009) Clinicopathological study of a series of 92 adrenocortical carcinomas: from a proposal of simplified diagnostic algorithm to prognostic stratification. Histopathol 55:535–543
Papotti M, Libè R, Duregon E et al (2011) The Weiss score and beyond-histopathology for adrenocortical carcinoma. Horm canc 2:333–340
van Slooten H, Schaberg A, Smeenk D et al (1985) Morphologic characteristics of benign and malignant adrenocortical tumors. Cancer 55:766–773
Evans HL, Vassilopoulou-Sellin R (1996) Adrenal cortical neoplasms: a study of 56 cases. Am J Clin Pathol 105:76–86
Stojadinovic A, Ghossein RA, Hoos A et al (2002) Adrenocortical carcinoma: clinical, morphologic, and molecular characterization. J Clin Oncol 20:941–950
Assie G, Antoni G, Tissier F et al (2007) Prognostic parameters of metastatic adrenocortic carcinoma. J Clin Endocrinol Metab 92:148–154
Aiba M, Fujibayashi M (2005) Histopathological diagnosis and prognostic factors in adrenocortical carcinoma. Endocr Pathol 16:13–22
Giordano TJ (2011) The argument for mitotic rate-based grading for the prognostication of adrenocortical carcinoma. Am J Surg Pathol 35:471–473
Beuschlein F, Obracay J, Saeger W et al (2013) Prognostic value of histological markers in localized adrenocortical carcinoma after complete resection. Endocr Rev 34:23–29
Beuschlein F, Weigel J, Saeger W et al (2015) Major prognostic role of Ki67 in localized adrenocortical carcinoma after complete resection. J Clin Endocrinol Metab 100:841–849
Aubert S, Wacrenier A, Leroy X et al (2002) Weiss system revisited: a clinicopathologic and immunohistochemical study of 49 adrenocortical tumors. Am J Surg Pathol 26:1612–1619
Didolkar MS, Bescher RA, Elias EG, Moore RH (1981) Natural history of adrenal cortical carcinoma: a clinicopathologic study of 42 patients. Cancer 47:2153–2161
Luton JP, Cerdas S, Billaud L et al (1990) Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy. N Engl J Med 322:1195–1201
Wajchenberg BL, Albergaria Pereira MA, Medonca BB et al (2000) Adrenocortical carcinoma: clinical and laboratory observations. Cancer 88:711–736
Icard P, Goudet P, Charpenay C et al (2001) Adrenocortical carcinomas: surgical trends and results of a 253- patient series from the French Association of Endocrine Surgeons study group. World J Surg 25:891–897
Favia G, Lumachi F, D’Amico DF (2001) Adrenocortical carcinoma: is prognosis different in nonfunctioning tumors? Results of surgical treatment in 31 patients. World J Surg 25:735–738
Abiven G, Coste J, Groussin L et al (2006) Clinical and biological features in the prognosis of adrenocortical cancer: poor outcome of cortisol-secreting tumors in a series of 202 consecutive patients. J Clin Endocrinol Metab 91:2650–2655
Berruti A, Fassnacht M, Haak H et al (2014) Prognostic role of overt hypercortisolism in completely operated patients with adrenocortical cancer. Eur Urol 65:832–838
Berruti A, Terzolo M, Sperone P et al (2005) Etoposide, doxorubicin and cisplatin plus mitotane in the treatment of advanced adrenocortical carcinoma: a large prospective phase II trial. Endocr Relat Cancer 12:657–666
Ronchi CL, Sbiera S, Leich E et al (2012) Low SGK1 expression in human adrenocortical tumors is associated with ACTH-independent glucocorticoid secretion and poor prognosis. J Clin Endocrinol Metab 97:2251–2260
Giordano TJ, Kuick R, Else T et al (2009) Molecular classification and prognostication of adrenocortical tumors by transcriptome profiling. Clin Cancer Res 15:668–676
Laurell C, Velázquez-Fernández D, Lindsten K et al (2009) Transcriptional profiling enables molecular classification of adrenocortical tumours. Eur J Endocrinol 161:141–152
de Reyniès A, Assié G, Rickman DS et al (2009) Gene expression profiling reveals a new classification of adrenocortical tumors and identifies molecular predictors of malignancy and survival. J Clin Oncol 27:1108–1115
Ragazzon B, Libé R, Gaujoux S et al (2010) Transcriptome analysis reveals that p53 and{beta}-catenin alterations occur in a group of aggressive adrenocortical cancers. Cancer Res 70:8276–8281
Ragazzon B, Assié G, Bertherat J (2011) Transcriptome analysis of adrenocortical cancers: from molecular classification to the identification of new treatments. Endocr Relat Cancer 18:15–27
Assié G, Guillaud-Bataille M, Ragazzon B et al (2010) The pathophysiology, diagnosis and prognosis of adrenocortical tumors revisited by transcriptome analyses. Trends Endocrinol Metab 21:325–334
Fenske W, Völker HU, Adam P et al (2009) Glucose transporter GLUT1 expression is an stage-independent predictor of clinical outcome in adrenocortical carcinoma. Endocr Relat Cancer 1:919–928
Duregon E, Volante M, Giorcelli J et al (2013) Diagnostic and prognostic role of steroidogenic factor 1 in adrenocortical carcinoma: a validation study focusing on clinical and pathologic correlates. Hum Pathol 44:822–828
Soon PS, Tacon LJ, Gill AJ et al (2009) miR-195 and miR-483-5p identified as predictors of poor prognosis in adrenocortical cancer. Clin Cancer Res 15:7684–7692
Chabre O, Libé R, Assie G et al (2013) Serum miR-483-5p and miR-195 are predictive of recurrence risk in adrenocortical cancer patients. Endocr Relat Cancer 20:579–594
Pinzani P, Scatena C, Salvianti F et al (2013) Detection of circulating tumor cells in patients with adrenocortical carcinoma: a monocentric preliminary study. J Clin Endocrinol Metab 98:3731–3738
Assié G, Letouzé E, Fassnacht M et al (2014) Integrated genomic characterization of adrenocortical carcinoma. Nat Genet 46:607–20141
Donatini G, Caiazzo R, Do Cao C et al (2014) Long-term survival after adrenalectomy for stage I/II adrenocortical carcinoma (ACC): a retrospective comparative cohort study of laparoscopic versus open approach. Ann Surg Oncol 21:284–291
Fosså A, Røsok BI, Kazaryan AM et al (2013) Laparoscopic versus open surgery in stage I-III adrenocortical carcinoma—a retrospective comparison of 32 patients. Acta Oncol 52(8):1771–1777
Lombardi CP, Raffaelli M, De Crea C et al (2012) Open versus endoscopic adrenalectomy in the treatment of localized (stage I/II) adrenocortical carcinoma: results of a multiinstitutional Italian survey. Surgery 152:1158–1164
Bellantone R, Ferrante A, Boscherini et al (1997) Role of reoperation in recurrence of adrenal cortical carcinoma: results from 188 cases collected in the Italian National Registry for Adrenal Cortical Carcinoma. Surgery 122:1212–1218
Schulick RD, Brennan MF (1999) Long-term survival after complete resection and repeat resection in patients with adrenocortical carcinoma. Ann Surg Oncol 6:719–726
Vaughan ED Jr (2004) Diseases of the adrenal gland. Med Clin North Am 88:443–466
Huang H, Fojo T (2008) Adjuvant mitotane for adrenocortical cancer-a recurring controversy. J Clin Endocrinol Metab 93:3730–3732
Wängberg B, Khorram-Manesh A, Jansson S et al (2010) The long-term survival in adrenocortical carcinoma with active surgical management and use of monitored mitotane. Endocr Relat Cancer 17:265–272
Pommier RF, Brennan MF (1992) An eleven-year experience with adrenocortical carcinoma. Surgery 112:963–971
Crucitti F, Bellantone R, Ferrante A et al (1996) The italian registry for adrenal cortical carcinoma: analysis of a multi-institutional series of 129 patients. Surgery 119:161–170
Schteingart DE, Doherty GM, Gauger PG et al (2005) Management of patients with adrenal cancer: recommendations of an international consensus conference. End Relat Cancer 12:667–680
Fassnacht M, Johanssen S, Fenske W et al (2010) Improved survival in patients with stage II adrenocortical carcinoma followed up prospectively by specialized center. J Clin Endocr Metab 95:4925–4932
Porpiglia F, Fiori C, Daffara F et al (2010) Retrospective evaluation of the outcome of open versus laparoscopic adrenalectomy for stage I and II adrenocortical cancer. Eur Urol 57:873–878
Brix D, Allolio B, Fenske W et al (2010) Laparoscopic versus open adrenalectomy for adrenocortical carcinoma: surgical and oncologic outcome in 152 patients. Eur Urol 58:609–615
Reibetanz J, Jurowich C, Erdogan I et al (2012) Impact of lymphadenectomy on the oncologic outcome of patients with adrenocortical carcinoma. Ann Surg 25:363–369
Gaujoux S, Brennan MF (2012) Recommendation for standardized surgical management of primary adrenocortical carcinoma. Surgery 152:123–132
Bilimoria KY, Shen WT, Elaraj D et al (2008) Adrenocortical carcinoma in the United States: treatment utilization and prognostic factors. Cancer 113:3130–3136
Kemp CD, Ripley RT, Mathur A et al (2011) Pulmonary resection for metastatic adrenocortical carcinoma. Ann Thorac Surg 92:1195–1270
Ripley RT, Kemp CD, Davis JL et al (2011) Liver Resection and ablation for metastatic adrenocortical carcinoma. Ann Surg Oncol 18:1972–1979
Datrice NM, Langan RC, Ripley RT et al (2012) Operative management for recurrent and metastatic adrenocortical carcinoma. J Surg Oncol 105:709–713
Erdogan I, Deutschbein T, Jurowich C et al (2013) The role of surgery in the management of recurrent adrenocortical carcinoma. J Clin Endocr Metab 98:181–191
Veytsman I, Nieman L, Fojo T (2009) Management of endocrine manifestations and the use of mitotane as a chemotherapeutic agent for adrenocortical carcinoma. J Clin Oncol 27:4619–4629
Grubbs EG, Callender GG, Xing Y et al (2010) Recurrence of adrenal cortical carcinoma following resection: surgery alone can achieve results equal to surgery plus mitotane. Ann Surg Oncol 17:263–270
Terzolo M, Baudin E, Ardito A et al (2013) Mitotane levels predict the outcome of patients with adrenocortical carcinoma treated adjuvantly following radical. Eur J Endocrinol 169:263–270
Volante M, Terzolo M, Fassnacht M et al (2012) Ribonucleotide reductase large subunit (RRM1) gene expression may predict efficacy of adjuvant mitotane in adrenocortical cancer. Clin Cancer Res 18:3452–3461
Sabolch A, Feng M, Griffith K et al (2011) Adjuvant and definitive radiotherapy for adrenocortical carcinoma. Int J Radiat Oncol Biol Phys 80:1477–1484
Fassnacht M, Hahner S, Polat B et al (2006) Efficacy of adjuvant radiotherapy of the tumor bed on local recurrence of adrenocortical carcinoma. J Clin Endocrinol Metab 91:4501–4504
Habra MA, Ejaz S, Feng L et al (2013) A retrospective cohort analysis of the efficacy of adjuvant radiotherapy after primary surgical resection in patients with adrenocortical carcinoma. J Clin Endocrinol Metab 98:192–197
Polat B, Fassnacht M, Pfreundner L et al (2009) Radiotherapy in adrenocortical carcinoma. Cancer 115:2816–2823
Keskin S, Taş F, Vatansever S (2013) Adrenocortical carcinoma: clinicopathological features, prognostic factors and outcome. Urol Int 90:435–438
Khan TS, Imam H, Juhlin C et al (2000) Streptozocin and o, p’DDD in the treatment of adrenocortical cancer patients: long-term survival in its adjuvant use. Ann Oncol 11:1281–1287
Fujii Y, Kageyama Y, Kawakami S et al (2003) Successful long-term disease-free survival following multimodal treatments in a patients with a repeatedly recurrent refractory adrenal cortical carcinoma. Int J Urol 10:445–448
Touitou Y, Bogdan A, Luton JP (1978) Changes in corticosteroid synthesis of the human adrenal cortex in vitro, induced by treatment with o, p′-DDD for Cushing’s syndrome: evidence for the sites of action of the drug. J Ster Biochem 9:1217–1224
Lin CW, Chang YH, Pu HF (2012) Mitotane exhibits dual effects on steroidogenic enzymes gene transcription under basal and cAMP-stimulating microenvironments in NCI-H295 cells. Toxicol 298:14–23
Lehmann TP, Wrzesinski T, Jagodzinski PP (2013) The effect of mitotane on viability, steroidogenesis and gene expression in NCI-H295R adrenocortical cells. Mol Med Rep 7:893–900
Bergenstal DM, Hertz R, Lipsett MB, Moy RH (1960) Chemotherapy of adrenocortical cancer with o, p′-DDD. Ann Int Med 53:672–682
van Slooten H, Moolenaar AJ, van Seters AP, Smeenk D (1984) The treatment of adrenocortical carcinoma with o, p′-DDD: prognostic implications of serum level monitoring. Eur J Cancer Clin Oncol 20:47–53
Hermsen IG, Fassnacht M, Terzolo M et al (2011) Plasma concentrations of o, p′DDD, o, p′DDA, and o, p′DDE as predictors of tumor response to mitotane in adrenocortical carcinoma: results of a retrospective ENS@T multicenter study. J Clin Endocrinol Metab 96:1844–1851
Kerkhofs TM, Baudin E, Terzolo M et al (2013) Comparison of two mitotane starting dose regimens in patients with advanced adrenocortical carcinoma. J Clin Endocrinol Metab 98:4759–4767
Mauclère-Denost S, Leboulleux S, Borget I et al (2012) High-dose mitotane strategy in adrenocortical carcinoma: prospective analysis of plasma mitotane measurement during the first 3 months of follow-up. Eur J Endocrinol 166:261–268
van Erp NP, Guchelaar HJ, Ploeger BA et al (2011) Mitotane has a strong and a durable inducing effect on CYP3A4 activity. Eur J Endocrinol 164:621–626
Kroiss M, Quinkler M, Lutz WK et al (2011) Drug interactions with mitotane by induction of CYP3A4 metabolism in the clinical management of adrenocortical carcinoma. Clin End 75:585–591
Haak HR, Hermans J, van de Velde CJ et al (1994) Optimal treatment of adrenocortical carcinoma with mitotane: results in a consecutive series of 96 patients. Br J Cancer 69:947–951
Baudin E, Pellegriti G, Bonnay M et al (2001) Impact of monitoring plasma 1,1-dichlorodiphenildichloroetane (o, p′DDD) levels on the treatment of patients with adrenocortical carcinoma. Cancer 92:1385–1392
Baudin E, Leboulleux S, Al Ghuzlan A et al (2011) Therapeutic management of advanced adrenocortical carcinoma: what do we know in 2011? Horm Cancer 6:363–371
Bates SE, Shieh CY, Mickley LA et al (1991) Mitotane enhances cytotoxicity of chemotherapy in cell lines expressing a multidrug resistance gene (mdr-1/P-glycoprotein) which is also expressed by adrenocortical carcinomas. J Clin Endocrinol Metabol 73:18–29
Bukowski RM, Wolfe M, Levine HS et al (1993) Phase II trial of mitotane and cisplatin in patients with adrenal carcinoma: a Southwest Oncology Group study. J Clin Oncol 11:161–165
Schteingart DE (2000) Conventional and novel strategies in the treatment of adrenocortical cancer. Braz J Med Biol Res 33:1197–1200
Bonacci R, Gigliotti A, Baudin E et al (1998) Cytotoxic therapy with etoposide and cisplatin in advanced adrenocortical carcinoma. Brit J Cancer 78:546–549
Burgess MA, Legha SS, Sellin RV (1993) Chemotherapy with cisplatinum and etoposide (UP16) for patients with advanced adrenal cortical carcinoma (ACC). Proc Ann Soc Clin Oncol 12:188
Williamson SK, Lew D, Miller GJ et al (2000) Phase II evaluation of cisplatin and etoposide followed by mitotane at disease progression in patients with locally advanced or metastatic adrenocortical carcinoma: a Southwest Oncology Group Study. Cancer 88:1159–1165
Fassnacht M, Terzolo M, Allolio B et al (2012) Combination chemotherapy in advanced adrenocortical carcinoma. New Engl J Med 366:2189–2197
Barlaskar FM, Hammer GD (2007) The molecular genetics of adrenocortical carcinoma. Rev End Metab Dis 8:343–348
Barlaskar FM, Spalding AC, Heaton JH et al (2009) Preclinical targeting of the type I insulin-like growth factor receptor in adrenocortical carcinoma. J Clin Endocrin Metab 94:204–212
Carden CP, Frentzas S, Langham M et al (2009) Preliminary activity in adrenocortical tumor (ACC) in phase I dose escalation study of intermittent oral dosing of OSI-906, a small-molecule insulin-like growth factor-1 receptor (IGF- 1R) tyrosine kinase inhibitor in patients with advanced solid tumors. J Clin Oncol 27:3544
Haluska P, Worden F, Olmos D et al (2010) Safety, tolerability, and pharmacokinetics of the anti-IGF-1R monoclonal antibody figitumumab in patients with refractory adrenocortical carcinoma. Cancer Chem Pharm 65:765–773
Fassnacht M, Berruti A, Baudin E et al (2015) Linsitinib (OSI-906) versus placebo for patients with locally advanced or metastatic adrenocortical carcinoma: a double blind, randomized, phase 3 study. Lancet Oncology 16:426–435
Doghman M, El Wakil A, Cardinaudetal B (2010) Regulation of IGF-mTOR signalling by miRNA in childhood adrenocortical tumors. Canc Res 70:4666–4675
Naing A, Kurzrock R, Burger A et al (2011) Phase I trial of cixutumumab combined with temsirolimus in patients with advanced cancer. Clin Cancer Res 17:6052–6060
Tacon LJ, Prichard RS, Soon PSH et al (2011) Current and emerging therapies for advanced adrenocortical carcinoma. Oncologist 16:36–48
Wortmann S, Quinkler M, Ritter C et al (2010) Bevacizumab plus capecitabine as a salvage therapy in advanced adreno-cortical carcinoma. Eur J Endocrinol 162:349–356
Chaco R, Tossen G, Loria FS, Chaco M (2005) Response in a patient with metastatic adrenal cortical carcinoma with thalidomide. J Clin Oncol 23:1579–1580
Kroiss M, Quinkler M, Johanssen S et al (2012) Sunitinib in refractory adrenocortical carcinoma: a phase II, single-arm, open-label trial. J Clin Endocrinol Metab 97:3495–3503
Hong DS, Sebti SM, Newman RA et al (2009) Phase I trial of a combination of the multikinase inhibitor sorafenib and the farnesyltransferase inhibitor tipifarnib in advanced malignancies. Clin Cancer Res 15:7061–7068
Butler C, Butler WM, Rizvi AA (2010) Sustained remission with the kinase inhibitor sorafenib in stage IV metastatic adrenocortical carcinoma. End Pract 16:441–445
Berruti A, Sperone P, Ferrero A et al (2012) Phase II study of weekly paclitaxel and sorafenib as second/third-line therapy in patients with adrenocortical carcinoma. Eur J Endocrin 166:451–458
Lee JO, Lee KW, Kim CJ et al (2009) Metastatic adrenocortical carcinoma treated with sunitinib: a case report. Jpn J Clin Oncol 39:183–185
Gangadhar TC, Cohen EEW, Wu K et al (2011) Two drug interaction studies of sirolimus in combination with sorafenib or sunitinib in patients with advanced malignancies. Clin Cancer Res 17:1956–1963
Quinkler M, Hahner S, Wortmann S et al (2008) Treatment of advanced adrenocortical carcinoma with erlotinib plus gemcitabine. J Clin Endocrinol Metab 93:2057–2062
Samnotra V, Vassilopoulou-Sellin R, Fojo AT et al (2007) A phase II trial of gefitinib monotherapy in patients with unresectable adrenocortical carcinoma (ACC). J Clin Oncol 27:15527
Gross DJ, Munter G, Bitan M et al (2006) The role of imatinib mesylate (Glivec) for treatment of patients with malignant endocrine tumors positive for c-kit or PDGF-R. End Related Canc 13:535–540
Adam P, Hahner S, Hartmann M et al (2010) Epidermal growth factor receptor in adrenocortical tumors: analysis of gene sequence, protein expression and correlation with clinical outcome. Mod Pathol 23:1596–1604
Abraham J, Bakke S, Rutt A et al (2002) A phase II trial of combination chemotherapy and surgical resection for the treatment of metastatic adrenocortical carcinoma: continuous infusion doxorubicin, vincristine, and etoposide with daily mitotane as a P-glycoprotein antagonist. Cancer 94:2333–2343
Papewalis C, Fassnacht M, Willenberg HS et al (2006) Dendritic cells as potential adjuvant for immunotherapy in adrenocortical carcinoma. Clin Endocrinol 65:215–222
Wood BJ, Abraham J, Hvizda JL et al (2003) Radiofrequency ablation of adrenal tumors and adrenocortical carcinoma metastases. Cancer 97:554–560
Mayo-Smith WW, Dupuy DE (2004) Adrenal neoplasms: CT-guided radiofrequency ablation: preliminary results. Radiology 231:225–230
Tranberg KG (2004) Percutaneous ablation of liver tumours. Best Pract Res Clin Gastroenterol 18:125–145
Cazejust J, De Baere T, Auperin A et al (2010) Transcatheter arterial chemoembolization for liver metastases in patients with adrenocortical carcinoma. J Vasc Interv Radiol 21:1527–1532
Acknowledgments
The present statement is endorsed by the Adrenal Study Group of the Italian Society of Endocrinology (SIE).
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors AS, IC, RG, AF, LC, SDC, PL, ML, FM declare no conflicts of interest. MT declares the following conflicts of interest: Advisory board, HRA and Atterocor, Speaker honoraria HRA and Corcept.
Ethical approval
This article does not contain any studies with human participants performed by any of the authors.
Informed consent
For this type of study formal consent is not required.
Rights and permissions
About this article
Cite this article
Stigliano, A., Chiodini, I., Giordano, R. et al. Management of adrenocortical carcinoma: a consensus statement of the Italian Society of Endocrinology (SIE). J Endocrinol Invest 39, 103–121 (2016). https://doi.org/10.1007/s40618-015-0349-9
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s40618-015-0349-9