adrenocortical carcinoma

Summary

Summary: A malignant neoplasm of the ADRENAL CORTEX. Adrenocortical carcinomas are unencapsulated anaplastic (ANAPLASIA) masses sometimes exceeding 20 cm or 200 g. They are more likely to be functional than nonfunctional, and produce ADRENAL CORTEX HORMONES that may result in hypercortisolism (CUSHING SYNDROME); HYPERALDOSTERONISM; and/or VIRILISM.

Top Publications

  1. Sperone P, Ferrero A, Daffara F, Priola A, Zaggia B, Volante M, et al. Gemcitabine plus metronomic 5-fluorouracil or capecitabine as a second-/third-line chemotherapy in advanced adrenocortical carcinoma: a multicenter phase II study. Endocr Relat Cancer. 2010;17:445-53 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare neoplasm characterized by poor prognosis. First-line systemic treatments in advanced disease include mitotane, either alone or in combination with chemotherapy...
  2. Gaujoux S, Grabar S, Fassnacht M, Ragazzon B, Launay P, Libe R, et al. ?-catenin activation is associated with specific clinical and pathologic characteristics and a poor outcome in adrenocortical carcinoma. Clin Cancer Res. 2011;17:328-36 pubmed publisher
    Activation of the Wnt/?-catenin signaling pathway is frequent in adrenocortical carcinoma (ACC) and might be associated with a more aggressive phenotype...
  3. Ragazzon B, Libe R, Gaujoux S, Assie G, Fratticci A, Launay P, et al. Transcriptome analysis reveals that p53 and {beta}-catenin alterations occur in a group of aggressive adrenocortical cancers. Cancer Res. 2010;70:8276-81 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare disease with an overall poor but heterogeneous prognosis. This heterogeneity could reflect different mechanisms of tumor development...
  4. O Neill C, Spence A, Logan B, Suliburk J, Soon P, Learoyd D, et al. Adrenal incidentalomas: risk of adrenocortical carcinoma and clinical outcomes. J Surg Oncol. 2010;102:450-3 pubmed publisher
    ..The aim of this study is to determine the risk of malignancy in patients undergoing surgery for adrenal incidentaloma; and to compare clinical outcomes in those with adrenocortical carcinoma (ACC) based on the mode of presentation.
  5. Fassnacht M, Johanssen S, Fenske W, Weismann D, Agha A, Beuschlein F, et al. Improved survival in patients with stage II adrenocortical carcinoma followed up prospectively by specialized centers. J Clin Endocrinol Metab. 2010;95:4925-32 pubmed publisher
    Median survival in stage II adrenocortical carcinoma (ACC) differs widely in published series ranging between 23 and more than 60 months...
  6. Ronchi C, Sbiera S, Kraus L, Wortmann S, Johanssen S, Adam P, et al. Expression of excision repair cross complementing group 1 and prognosis in adrenocortical carcinoma patients treated with platinum-based chemotherapy. Endocr Relat Cancer. 2009;16:907-18 pubmed publisher
    Therapeutic progress in adrenocortical carcinoma (ACC) is severely hampered by its low incidence. Platinum-based chemotherapies are the most effective cytotoxic treatment regimens in ACC but response rates remain <50%...
  7. Haluska P, Worden F, Olmos D, Yin D, Schteingart D, Batzel G, et al. Safety, tolerability, and pharmacokinetics of the anti-IGF-1R monoclonal antibody figitumumab in patients with refractory adrenocortical carcinoma. Cancer Chemother Pharmacol. 2010;65:765-73 pubmed publisher
    ..through upregulation of the stimulatory ligand IGF-II has been implicated in the pathogenesis of adrenocortical carcinoma. As there is a paucity of effective therapies, this dose expansion cohort of a phase 1 study was ..
  8. Schmitz K, Helwig J, Bertram S, Sheu S, Suttorp A, Seggewiss J, et al. Differential expression of microRNA-675, microRNA-139-3p and microRNA-335 in benign and malignant adrenocortical tumours. J Clin Pathol. 2011;64:529-35 pubmed publisher
    ..The tremendous potential of microRNAs (miRNAs) as diagnostic biomarkers in surgical pathology has recently been shown in a broad variety of tumours...
  9. Russell Swetek A, West A, Mintern J, Jenkins J, Rodriguez Galindo C, Ribeiro R, et al. Identification of a novel TP53 germline mutation E285V in a rare case of paediatric adrenocortical carcinoma and choroid plexus carcinoma. J Med Genet. 2008;45:603-6 pubmed publisher
    ..These results further strengthen the association between germline TP53 mutations and childhood CPC, even when occurring in the absence of familial tumour susceptibility...

More Information

Publications89

  1. Gaujoux S, Al Ahmadie H, Allen P, Gonen M, Shia J, D Angelica M, et al. Resection of adrenocortical carcinoma liver metastasis: is it justified?. Ann Surg Oncol. 2012;19:2643-51 pubmed publisher
    b>Adrenocortical carcinoma (ACC) liver metastases (LM) represent a therapeutic challenge, and it is unclear whether resection is justified...
  2. Barreau O, Assie G, Wilmot Roussel H, Ragazzon B, Baudry C, Perlemoine K, et al. Identification of a CpG island methylator phenotype in adrenocortical carcinomas. J Clin Endocrinol Metab. 2013;98:E174-84 pubmed publisher
    ..This genome-wide methylation analysis reveals the existence of hypermethylated adrenocortical carcinomas, with a poorer prognosis. Hypermethylation in these tumors is important for silencing specific tumor suppressor genes. ..
  3. Grubbs E, Callender G, Xing Y, Perrier N, Evans D, Phan A, et al. Recurrence of adrenal cortical carcinoma following resection: surgery alone can achieve results equal to surgery plus mitotane. Ann Surg Oncol. 2010;17:263-70 pubmed publisher
    A recent nonrandomized interinstitutional study reported that adjuvant mitotane following surgery for adrenocortical carcinoma (ACC) was associated with decreased recurrence...
  4. Fassnacht M, Terzolo M, Allolio B, Baudin E, Haak H, Berruti A, et al. Combination chemotherapy in advanced adrenocortical carcinoma. N Engl J Med. 2012;366:2189-97 pubmed publisher
    b>Adrenocortical carcinoma is a rare cancer that has a poor response to cytotoxic treatment.
  5. Ikeda K, Saito T, Tojo K. Efonidipine, a Ca(2+)-channel blocker, enhances the production of dehydroepiandrosterone sulfate in NCI-H295R human adrenocortical carcinoma cells. Tohoku J Exp Med. 2011;224:263-71 pubmed
    ..NCI-H295R human adrenocortical carcinoma cells and HepG2 human hepatoma cells were cultured for 24 hours with or without a CCB (amlodipine, ..
  6. Fassnacht M, Johanssen S, Quinkler M, Bucsky P, Willenberg H, Beuschlein F, et al. Limited prognostic value of the 2004 International Union Against Cancer staging classification for adrenocortical carcinoma: proposal for a Revised TNM Classification. Cancer. 2009;115:243-50 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare malignancy, and it was only in 2004 that the International Union Against Cancer (UICC) defined TNM criteria and published the first staging classification...
  7. Simon D, Hammer G. Adrenocortical stem and progenitor cells: implications for adrenocortical carcinoma. Mol Cell Endocrinol. 2012;351:2-11 pubmed publisher
    ..stem/progenitor cell biology in the adrenal and/or other organs, are also implicated in the etiology of adrenocortical carcinoma. The rarity and heterogeneous presentation of ACC makes it difficult to identify the cellular origin and ..
  8. Ozata D, Caramuta S, Velazquez Fernandez D, Akçakaya P, Xie H, Höög A, et al. The role of microRNA deregulation in the pathogenesis of adrenocortical carcinoma. Endocr Relat Cancer. 2011;18:643-55 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is an aggressive tumor showing frequent metastatic spread and poor survival...
  9. Ismail A, Bateman A. Expression of TBX2 promotes anchorage-independent growth and survival in the p53-negative SW13 adrenocortical carcinoma. Cancer Lett. 2009;278:230-240 pubmed publisher
    ..Our findings identify TBX2 as a cell type-dependent survival factor under a p53-negative background, and are indicative of a potentially wider role for TBX2 in carcinogenesis than hitherto described. ..
  10. De Martino M, van Koetsveld P, Feelders R, Sprij Mooij D, Waaijers M, Lamberts S, et al. The role of mTOR inhibitors in the inhibition of growth and cortisol secretion in human adrenocortical carcinoma cells. Endocr Relat Cancer. 2012;19:351-64 pubmed publisher
    Patients with adrenocortical carcinoma (ACC) need new treatment options. The aim of this study was to evaluate the effects of the mTOR inhibitors sirolimus and temsirolimus on human ACC cell growth and cortisol production...
  11. Jani P, Nasr A, Demellawy D. Synchronous renal cell carcinoma and adrenocortical carcinoma: a rare case report and clinicopathologic approach. Can J Urol. 2008;15:4016-9 pubmed
    A case of synchronous adrenocortical carcinoma (ACC) and renal cell carcinoma (RCC) has not yet been described in the English medical literature, to our knowledge...
  12. Soon P, McDonald K, Robinson B, Sidhu S. Molecular markers and the pathogenesis of adrenocortical cancer. Oncologist. 2008;13:548-61 pubmed publisher
  13. Fassnacht M, Libe R, Kroiss M, Allolio B. Adrenocortical carcinoma: a clinician's update. Nat Rev Endocrinol. 2011;7:323-35 pubmed publisher
    b>Adrenocortical carcinoma is a rare heterogeneous neoplasm with an incompletely understood pathogenesis and a poor prognosis...
  14. Fassnacht M, Kroiss M, Allolio B. Update in adrenocortical carcinoma. J Clin Endocrinol Metab. 2013;98:4551-64 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is an orphan malignancy that has attracted increasing attention during the last decade. Here we provide an update on advances in the field since our last review published in this journal in 2006...
  15. Reibetanz J, Jurowich C, Erdogan I, Nies C, Rayes N, Dralle H, et al. Impact of lymphadenectomy on the oncologic outcome of patients with adrenocortical carcinoma. Ann Surg. 2012;255:363-9 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare malignancy with an unfavorable prognosis. The impact of a locoregional lymph node dissection (LND) has never been defined in this disease...
  16. Wang T, Cheung K, Roman S, Sosa J. A cost-effectiveness analysis of adrenalectomy for nonfunctional adrenal incidentalomas: is there a size threshold for resection?. Surgery. 2012;152:1125-32 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare, but aggressive, malignancy...
  17. Arlt W, Biehl M, Taylor A, Hahner S, Libe R, Hughes B, et al. Urine steroid metabolomics as a biomarker tool for detecting malignancy in adrenal tumors. J Clin Endocrinol Metab. 2011;96:3775-84 pubmed publisher
    Adrenal tumors have a prevalence of around 2% in the general population. Adrenocortical carcinoma (ACC) is rare but accounts for 2-11% of incidentally discovered adrenal masses...
  18. Schteingart D. Adjuvant mitotane therapy of adrenal cancer - use and controversy. N Engl J Med. 2007;356:2415-8 pubmed
  19. Ali A, Raphael S. Functional oncocytic adrenocortical carcinoma. Endocr Pathol. 2007;18:187-9 pubmed
    We present a case of oncocytic adrenocortical carcinoma in a 25-year-old man who presented with persistent hypertension, hypokalemia, and a large right adrenal mass...
  20. Wangberg B, Khorram Manesh A, Jansson S, Nilsson B, Nilsson O, Jakobsson C, et al. The long-term survival in adrenocortical carcinoma with active surgical management and use of monitored mitotane. Endocr Relat Cancer. 2010;17:265-72 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare tumour disease with sinister prognosis also after attempts to radical surgery; better prognosis is seen for low-stage tumours...
  21. Miller B, Ignatoski K, Daignault S, Lindland C, Doherty M, Gauger P, et al. Worsening central sarcopenia and increasing intra-abdominal fat correlate with decreased survival in patients with adrenocortical carcinoma. World J Surg. 2012;36:1509-16 pubmed publisher
    Accurate prediction of survival from adrenocortical carcinoma (ACC) is difficult and current staging models are unreliable. Central sarcopenia as part of the cachexia syndrome is a marker of frailty and predicts mortality...
  22. Giordano T. Molecular pathology of adrenal cortical tumors: separating adenomas from carcinomas. Endocr Pathol. 2006;17:355-63 pubmed
    ..Here, the relevant molecular pathology of adrenal cortical tumors is reviewed with special reference to those methods (e.g., DNA microarrays) that hold promise for improved diagnosis and prognosis, and prediction of therapeutic response...
  23. Soon P, Gill A, Benn D, Clarkson A, Robinson B, McDonald K, et al. Microarray gene expression and immunohistochemistry analyses of adrenocortical tumors identify IGF2 and Ki-67 as useful in differentiating carcinomas from adenomas. Endocr Relat Cancer. 2009;16:573-83 pubmed publisher
    ..The combination of IGF2 and Ki-67 IHC is also highly accurate in distinguishing between the two groups and is particularly helpful in ACTs with Weiss score of 3...
  24. Herrmann L, Heinze B, Fassnacht M, Willenberg H, Quinkler M, Reisch N, et al. TP53 germline mutations in adult patients with adrenocortical carcinoma. J Clin Endocrinol Metab. 2012;97:E476-85 pubmed publisher
    ..However, whereas such mutations have been detected with high frequency in childhood ACC, a large cohort of adult patients with ACC has never been investigated for TP53 germline mutations...
  25. Raymond V, Everett J, Furtado L, Gustafson S, Jungbluth C, Gruber S, et al. Adrenocortical carcinoma is a lynch syndrome-associated cancer. J Clin Oncol. 2013;31:3012-8 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is an endocrine malignancy with a poor prognosis. The association of adult-onset ACC with inherited cancer predisposition syndromes is poorly understood...
  26. Mwandila M, Waller H, Stott V, Mercer P. A case of a testosterone-secreting oncocytic adrenocortical carcinoma. N Z Med J. 2010;123:80-2 pubmed
    ..They are usually benign and non-functioning. We present a case of a testosterone-secreting oncocytic adrenocortical carcinoma in a 19-year-old female who presented with acne, hirsutism and irregular menses...
  27. Lawnicka H, Kowalewicz Kulbat M, Sicinska P, Kazimierczuk Z, Grieb P, Stepien H. Anti-neoplastic effect of protein kinase CK2 inhibitor, 2-dimethylamino-4,5,6,7-tetrabromobenzimidazole (DMAT), on growth and hormonal activity of human adrenocortical carcinoma cell line (H295R) in vitro. Cell Tissue Res. 2010;340:371-9 pubmed publisher
    ..Thus, CK2 kinase activity is probably involved in human ACC endocrine activity and growth...
  28. Butler C, Butler W, Rizvi A. Sustained remission with the kinase inhibitor sorafenib in stage IV metastatic adrenocortical carcinoma. Endocr Pract. 2010;16:441-5 pubmed publisher
    To report our experience using kinase inhibition therapy with sorafenib in a patient with advanced adrenocortical carcinoma.
  29. Kemp C, Ripley R, Mathur A, Steinberg S, Nguyen D, Fojo T, et al. Pulmonary resection for metastatic adrenocortical carcinoma: the National Cancer Institute experience. Ann Thorac Surg. 2011;92:1195-200 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare neoplasm with a high propensity for locoregional recurrences and distant metastases for which there are no effective systemic therapies...
  30. Zini L, Porpiglia F, Fassnacht M. Contemporary management of adrenocortical carcinoma. Eur Urol. 2011;60:1055-65 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare and typically aggressive malignancy. Available recommendations are based primarily on retrospective series or expert opinions, and only few prospective clinical studies have yet been published.
  31. Hermsen I, Fassnacht M, Terzolo M, Houterman S, Den Hartigh J, Leboulleux S, et al. 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. 2011;96:1844-51 pubmed publisher
    In patients with adrenocortical carcinoma (ACC) mitotane activity has been suggested to depend on plasma levels 14 mg/liter or greater and metabolite formation.
  32. Lin C, Whang E, Moalem J, Ruan D. Strategic combination therapy overcomes tyrosine kinase coactivation in adrenocortical carcinoma. Surgery. 2012;152:1045-50 pubmed publisher
    ..therapy could overcome tyrosine kinase coactivation and compensatory oncogenic signaling in patients with adrenocortical carcinoma (ACC)...
  33. Pinzani P, Scatena C, Salvianti F, Corsini E, Canu L, Poli G, et al. Detection of circulating tumor cells in patients with adrenocortical carcinoma: a monocentric preliminary study. J Clin Endocrinol Metab. 2013;98:3731-8 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare malignancy, the prognosis of which is mainly dependent on stage at diagnosis. The identification of disease-associated markers for early diagnosis and drug monitoring is mandatory...
  34. Assie G, Letouze E, Fassnacht M, Jouinot A, Luscap W, Barreau O, et al. Integrated genomic characterization of adrenocortical carcinoma. Nat Genet. 2014;46:607-12 pubmed publisher
    ..Thus, aggressive and indolent ACCs correspond to two distinct molecular entities driven by different oncogenic alterations...
  35. Terzolo M, Angeli A, Fassnacht M, Daffara F, Tauchmanova L, Conton P, et al. Adjuvant mitotane treatment for adrenocortical carcinoma. N Engl J Med. 2007;356:2372-80 pubmed
    b>Adrenocortical carcinoma is a rare neoplasm characterized by a high risk of recurrence after radical resection. Whether the use of mitotane is beneficial as an adjuvant treatment has been controversial...
  36. Yavascaoglu I, Yilmaz M, Kordan Y. Cardiac and caval invasion of left adrenocortical carcinoma. Urol Int. 2008;81:244-6 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare and highly malignant neoplasm...
  37. Volante M, Buttigliero C, Greco E, Berruti A, Papotti M. Pathological and molecular features of adrenocortical carcinoma: an update. J Clin Pathol. 2008;61:787-93 pubmed publisher
    The pathological diagnosis of adrenocortical carcinoma (ACC), which is based on gross and microscopic criteria, is subjective...
  38. Stephan E, Chung T, Grant C, Kim S, Von Hoff D, Trent J, et al. Adrenocortical carcinoma survival rates correlated to genomic copy number variants. Mol Cancer Ther. 2008;7:425-31 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare endocrine malignancy accounting for between 0.02% and 0.2% of all cancer deaths. Surgical removal offers the only current potential for cure...
  39. Ragazzon B, Cazabat L, Rizk Rabin M, Assie G, Groussin L, Fierrard H, et al. Inactivation of the Carney complex gene 1 (protein kinase A regulatory subunit 1A) inhibits SMAD3 expression and TGF beta-stimulated apoptosis in adrenocortical cells. Cancer Res. 2009;69:7278-84 pubmed publisher
    ..This cross-talk between the PKA and the TGFbeta signaling pathways reveals a new mechanism of endocrine tumorigenesis...
  40. Ko J, Lee H, Hong J, Hwang J. Virilizing adrenocortical carcinoma in a child with Turner syndrome and somatic TP53 gene mutation. Eur J Pediatr. 2010;169:501-4 pubmed publisher
    Virilizing adrenocortical carcinoma and Turner syndrome have opposite clinical manifestations in some aspects. Here, we report on the first case of virilizing adrenocortical carcinoma in a girl with Turner syndrome...
  41. Veytsman I, Nieman L, Fojo T. Management of endocrine manifestations and the use of mitotane as a chemotherapeutic agent for adrenocortical carcinoma. J Clin Oncol. 2009;27:4619-29 pubmed publisher
    ..Recommendations are provided to help manage patients with this difficult disease and to improve the quality of their lives...
  42. Senthil R, Mittal B, Kashyap R, Bhattacharya A, Radotra B, Bhansali A. ¹?F FDG PET/CT demonstration of IVC and right atrial involvement in adrenocortical carcinoma. Jpn J Radiol. 2012;30:281-3 pubmed publisher
    b>Adrenocortical carcinoma is a rare and aggressive tumour. Inferior vena caval (IVC) involvement by the tumour thrombus is a rare phenomenon, and extension into the right atrium is even more rare...
  43. Bilimoria K, Shen W, Elaraj D, Bentrem D, Winchester D, Kebebew E, et al. Adrenocortical carcinoma in the United States: treatment utilization and prognostic factors. Cancer. 2008;113:3130-6 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare tumor with a relatively poor prognosis...
  44. Volante M, Bollito E, Sperone P, Tavaglione V, Daffara F, Porpiglia F, et al. Clinicopathological study of a series of 92 adrenocortical carcinomas: from a proposal of simplified diagnostic algorithm to prognostic stratification. Histopathology. 2009;55:535-43 pubmed publisher
    Pathological diagnosis of adrenocortical carcinoma relies on several microscopic features commonly used in combination in different scoring systems that are sometimes subjective and/or time consuming...
  45. Berruti A, Sperone P, Ferrero A, Germano A, Ardito A, Priola A, et al. Phase II study of weekly paclitaxel and sorafenib as second/third-line therapy in patients with adrenocortical carcinoma. Eur J Endocrinol. 2012;166:451-8 pubmed publisher
    There is a strong rationale in the use of antiangiogenic therapy in the management of adrenocortical carcinoma (ACC). Metronomic administration of chemotherapy and antiangiogenic drugs can be synergistic in targeting endothelial cells.
  46. Barlaskar F, Spalding A, Heaton J, Kuick R, Kim A, Thomas D, et al. Preclinical targeting of the type I insulin-like growth factor receptor in adrenocortical carcinoma. J Clin Endocrinol Metab. 2009;94:204-12 pubmed publisher
    Drug therapy for adrenocortical carcinoma (ACC), a rare and lethal malignancy, is largely empirical and ineffective. New treatments directed at molecular targets critical to the pathophysiology of ACC may prove more efficacious.
  47. Costa R, Wesolowski R, Raghavan D. Chemotherapy for advanced adrenal cancer: improvement from a molecular approach?. BJU Int. 2011;108:1546-54 pubmed publisher
    ..Advanced adrenal carcinoma constitutes an orphan disease, with a high mortality rate, and merits investment in clinical trials...
  48. Fassnacht M, Allolio B. Clinical management of adrenocortical carcinoma. Best Pract Res Clin Endocrinol Metab. 2009;23:273-89 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare and heterogeneous malignancy, and most of the diagnostic and therapeutic strategies are not fully established according to criteria of evidence-based medicine...
  49. Lu C, Yen T, Hsieh M, Chen Y. Spontaneous rupture of adrenocortical carcinoma: a coincidence or a tendency?. Clin Nephrol. 2009;72:147-50 pubmed
    We report a rare case of adrenocortical carcinoma spontaneously rupturing. To our knowledge, this is the sixth reported case in literature that is not related to any preceding traumatic incidents or predisposing disease...
  50. Tacon L, Prichard R, Soon P, Robinson B, Clifton Bligh R, Sidhu S. Current and emerging therapies for advanced adrenocortical carcinoma. Oncologist. 2011;16:36-48 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare but aggressive malignancy with a poor prognosis...
  51. Kroiss M, Quinkler M, Johanssen S, van Erp N, Lankheet N, Pöllinger A, et al. Sunitinib in refractory adrenocortical carcinoma: a phase II, single-arm, open-label trial. J Clin Endocrinol Metab. 2012;97:3495-503 pubmed publisher
    Treatment of refractory adrenocortical carcinoma (ACC) is not established. Animal experiments pointed toward adrenal toxicity of sunitinib.
  52. Chouairy C, Abdul Karim F, MacLennan G. Adrenocortical carcinoma. J Urol. 2008;179:323 pubmed
  53. Naing A, LoRusso P, Fu S, Hong D, Chen H, Doyle L, et al. Insulin growth factor receptor (IGF-1R) antibody cixutumumab combined with the mTOR inhibitor temsirolimus in patients with metastatic adrenocortical carcinoma. Br J Cancer. 2013;108:826-30 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare and aggressive endocrine malignancy without an available effective systemic chemotherapy...
  54. Kerkhofs T, Verhoeven R, van der Zwan J, Dieleman J, Kerstens M, Links T, et al. Adrenocortical carcinoma: a population-based study on incidence and survival in the Netherlands since 1993. Eur J Cancer. 2013;49:2579-86 pubmed publisher
    The reported annual incidence of adrenocortical carcinoma (ACC) is 0.5-2.0 cases per million individuals. Updated population-based studies on incidence are lacking...
  55. Papotti M, Volante M, Duregon E, Delsedime L, Terzolo M, Berruti A, et al. Adrenocortical tumors with myxoid features: a distinct morphologic and phenotypical variant exhibiting malignant behavior. Am J Surg Pathol. 2010;34:973-83 pubmed publisher
    ..second (4 cases) was characterized by focal myxoid changes in tumors otherwise similar to conventional adrenocortical carcinoma, with large atypical cells having an eosinophilic cytoplasm and a diffuse or nodular architecture...
  56. Martarelli D, Pompei P, Baldi C, Mazzoni G. Mebendazole inhibits growth of human adrenocortical carcinoma cell lines implanted in nude mice. Cancer Chemother Pharmacol. 2008;61:809-17 pubmed
    b>Adrenocortical carcinoma is a rare tumor of the adrenal gland which requires new therapeutic approaches as its early diagnosis is difficult and prognosis poor despite therapies used...
  57. Ohwada S, Izumi M, Kawate S, Hamada K, Toya H, Togo N, et al. Surgical outcome of stage III and IV adrenocortical carcinoma. Jpn J Clin Oncol. 2007;37:108-13 pubmed
    b>Adrenocortical carcinoma (ACC) is a rare tumor usually diagnosed at an advanced stage on invasion of or adherence to adjacent organs. We report surgical outcome of stage III and IV ACCs.
  58. Fonseca A, Kugelberg J, Starker L, Scholl U, Choi M, Hellman P, et al. Comprehensive DNA methylation analysis of benign and malignant adrenocortical tumors. Genes Chromosomes Cancer. 2012;51:949-60 pubmed publisher
    ..Genes with altered DNA methylation patterns were identified of putative importance to benign and malignant adrenocortical tumor development...
  59. Lehmann T, Wrzesinski T. The molecular basis of adrenocortical cancer. Cancer Genet. 2012;205:131-7 pubmed publisher
    Adrenocortical tumors (ACTs) are common, and most are benign adrenocortical adenomas (ACAs). Malignant adrenocortical carcinoma (ACC) is a rare tumor type and is observed at the rate of one or two cases per million annually...
  60. Ehrlund A, Jonsson P, Vedin L, Williams C, Gustafsson J, Treuter E. Knockdown of SF-1 and RNF31 affects components of steroidogenesis, TGF?, and Wnt/?-catenin signaling in adrenocortical carcinoma cells. PLoS ONE. 2012;7:e32080 pubmed publisher
    ..for adrenogonadal development, maintenance of adrenocortical progenitor cells and the development of adrenocortical carcinoma. Finally, the SF-1 gene profile can be used to distinguish malignant from benign adrenocortical tumors, a ..
  61. Caramuta S, Lee L, Ozata D, Akçakaya P, Xie H, Höög A, et al. Clinical and functional impact of TARBP2 over-expression in adrenocortical carcinoma. Endocr Relat Cancer. 2013;20:551-64 pubmed publisher
    ..This is the first study to demonstrate the deregulation of miRNA-processing factors in adrenocortical tumors and to show the clinical and biological impact of TARBP2 over-expression in this tumor type. ..
  62. Waldmann J, Feldmann G, Slater E, Langer P, Buchholz M, Ramaswamy A, et al. Expression of the zinc-finger transcription factor Snail in adrenocortical carcinoma is associated with decreased survival. Br J Cancer. 2008;99:1900-7 pubmed publisher
    ..19). In conclusion, we describe for the first time that Snail is expressed in a large subset of ACCs. Furthermore, Snail expression is associated with decreased survival, advanced disease and higher risk of developing distant metastases...
  63. Nakamura M, Miki Y, Akahira J, Morimoto R, Satoh F, Ishidoya S, et al. An analysis of potential surrogate markers of target-specific therapy in archival materials of adrenocortical carcinoma. Endocr Pathol. 2009;20:17-23 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare neoplasm but some of the cases are highly malignant. Clinical outcome of the patients with advanced ACC still remained poor or dismal despite recent development of aggressive antitumor therapies...
  64. Doghman M, Cazareth J, Douguet D, Madoux F, Hodder P, Lalli E. Inhibition of adrenocortical carcinoma cell proliferation by steroidogenic factor-1 inverse agonists. J Clin Endocrinol Metab. 2009;94:2178-83 pubmed publisher
    ..SF-1 amplification and overexpression are found in most cases of childhood adrenocortical tumors (ACTs)...
  65. Kroiss M, Quinkler M, Lutz W, Allolio B, Fassnacht M. Drug interactions with mitotane by induction of CYP3A4 metabolism in the clinical management of adrenocortical carcinoma. Clin Endocrinol (Oxf). 2011;75:585-91 pubmed publisher
    ..o,p'-DDD)] is the only drug approved for the treatment for adrenocortical carcinoma (ACC) and has also been used for various forms of glucocorticoid excess...
  66. Dehner L, Hill D. Adrenal cortical neoplasms in children: why so many carcinomas and yet so many survivors?. Pediatr Dev Pathol. 2009;12:284-91 pubmed publisher
    ..A risk assessment system is proposed that incorporates tumor weight, localization of tumor to the gland without invasion into the surrounding tissues or organs, and absence of metastasis...
  67. Ohtake H, Kawamura H, Matsuzaki M, Yokoyama E, Kitajima M, Onizuka S, et al. Oncocytic adrenocortical carcinoma. Ann Diagn Pathol. 2010;14:204-8 pubmed publisher
    Only 17 cases of oncocytic adrenocortical carcinoma have been reported in the English literature. Here, we report an incidental case of oncocytic adrenocortical carcinoma...
  68. Miller B, Ammori J, Gauger P, Broome J, Hammer G, Doherty G. Laparoscopic resection is inappropriate in patients with known or suspected adrenocortical carcinoma. World J Surg. 2010;34:1380-5 pubmed publisher
    ..Use of laparoscopy has been questioned in patients with ACC. This study compares the outcomes of patients undergoing laparoscopic versus open resection (OR) for ACC...
  69. Lombardi C, Raffaelli M, Boniardi M, De Toma G, Marzano L, Miccoli P, et al. Adrenocortical carcinoma: effect of hospital volume on patient outcome. Langenbecks Arch Surg. 2012;397:201-7 pubmed publisher
    Optimal management of adrenocortical carcinoma (ACC) involves a detailed diagnostic workup, radical surgery, and appropriate adjuvant therapy...
  70. Ayala Ramirez M, Jasim S, Feng L, Ejaz S, Deniz F, Busaidy N, et al. Adrenocortical carcinoma: clinical outcomes and prognosis of 330 patients at a tertiary care center. Eur J Endocrinol. 2013;169:891-899 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare malignancy with a poor prognosis. Herein, we describe the clinical features and outcomes for a large series of ACC patients.
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    No standard therapy for advanced adrenocortical carcinoma (ACC) is established by any randomized trial but a consensus conference 2003 recommended mitotane as monotherapy or combined with etoposide, doxorubicin and cisplatin or with ..
  72. Suyama K, Beppu T, Isiko T, Sugiyama S, Matsumoto K, Doi K, et al. Spontaneous rupture of adrenocortical carcinoma. Am J Surg. 2007;194:77-8 pubmed
    Massive hemorrhage from an adrenocortical carcinoma seldom occurs in the retroperitoneal or abdominal cavity. We report a case of spontaneous rupture of primary adrenocortical carcinoma occurring in an adolescent...
  73. Mermejo L, Elias Junior J, Saggioro F, Tucci Junior S, Castro M, Moreira A, et al. Giant adrenal myelolipoma associated with 21-hydroxylase deficiency: unusual association mimicking an androgen-secreting adrenocortical carcinoma. Arq Bras Endocrinol Metabol. 2010;54:419-24 pubmed
    ..Biochemical correlation has brought concerns about differential diagnosis with adrenocortical carcinoma, and surgical excision of the left adrenal mass was indicated...
  74. Sabolch A, Feng M, Griffith K, Hammer G, Doherty G, Ben Josef E. Adjuvant and definitive radiotherapy for adrenocortical carcinoma. Int J Radiat Oncol Biol Phys. 2011;80:1477-84 pubmed publisher
    To evaluate the impact of both adjuvant and definitive radiotherapy on local control of adrenocortical carcinoma.
  75. Quinkler M, Hahner S, Wortmann S, Johanssen S, Adam P, Ritter C, et al. Treatment of advanced adrenocortical carcinoma with erlotinib plus gemcitabine. J Clin Endocrinol Metab. 2008;93:2057-62 pubmed publisher
    b>Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis...
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    b>Adrenocortical carcinoma (ACC) is a rare malignancy, and patients with ACC have a poor prognosis. Even after radical surgery, up to 85% of patients develop recurrent disease. Systemic treatment options still have limited efficacy...
  77. Raparia K, Ayala A, Sienko A, Zhai Q, Ro J. Myxoid adrenal cortical neoplasms. Ann Diagn Pathol. 2008;12:344-8 pubmed publisher
    ..The usual clinical and histological features can be applied to classify the lesions as benign, borderline tumor, or malignant. In our series, there was no case with frank malignant tumor...
  78. Fenske W, Völker H, Adam P, Hahner S, Johanssen S, Wortmann S, et al. Glucose transporter GLUT1 expression is an stage-independent predictor of clinical outcome in adrenocortical carcinoma. Endocr Relat Cancer. 2009;16:919-28 pubmed publisher
    Owing to the rarity of adrenocortical carcinoma (ACC) no prognostic markers have been established beyond stage and resection status...
  79. Cerquetti L, Bucci B, Marchese R, Misiti S, de Paula U, Miceli R, et al. Mitotane increases the radiotherapy inhibitory effect and induces G2-arrest in combined treatment on both H295R and SW13 adrenocortical cell lines. Endocr Relat Cancer. 2008;15:623-34 pubmed publisher
    ..The same irreversible block on G2 phase, induced by IR/o,p'-DDD treatment, happened in H295R cells with restored wild-type p53 suggesting that this mechanism is not mediated by p53 pathway...
  80. Sakata R, Tsuchiya F, Osaka K, Fujikawa A, Ouchi H, Iwasaki A. [Adrenocortical carcinoma detected by retroperitoneal hemorrhage : a case report]. Hinyokika Kiyo. 2012;58:149-53 pubmed
    ..We report a case of spontaneous rupture of a primary adrenocortical carcinoma that occurred in a 79-year-old man. He visited our hospital with left abdominal pain...