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Adrenocortical Carcinoma

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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.

Also Known As:

Carcinoma, Adrenocortical; Adrenal Cortical Carcinoma; Adrenal Cortical Carcinomas; Adrenocortical Carcinomas; Carcinomas, Adrenal Cortical; Carcinomas, Adrenocortical; Carcinoma, Adrenal Cortical

Networked: 928 relevant articles (31 outcomes, 105 trials/studies)

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Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Cushing Syndrome
3.	Carcinoma (Carcinomatosis)
4.	Ectopic ACTH Syndrome
5.	Cardiac Arrhythmias (Arrythmia)

Experts

1.	Fassnacht, Martin: 33 articles (12/2015 - 01/2005)
2.	Allolio, Bruno: 28 articles (11/2015 - 01/2005)
3.	Terzolo, Massimo: 19 articles (04/2015 - 09/2005)
4.	Hahner, Stefanie: 18 articles (12/2015 - 01/2005)
5.	Sasano, Hironobu: 14 articles (06/2015 - 04/2006)
6.	Baudin, Eric: 13 articles (08/2015 - 03/2005)
7.	Beuschlein, Felix: 12 articles (10/2014 - 06/2003)
8.	Bertherat, Jérôme: 11 articles (12/2015 - 08/2003)
9.	Bertagna, Xavier: 11 articles (12/2015 - 08/2003)
10.	Kebebew, Electron: 11 articles (10/2015 - 08/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Adrenocortical Carcinoma:

1.	Mitotane (Lysodren)FDA Link 10/01/2014 - "Mitotane (o,p'DDD) is the most effective treatment of advanced adrenocortical carcinoma (ACC) but its mechanism of action remains unknown. " 07/01/2001 - "The excellent follow-up of these patients suggests that even in hopeless cases with metastatic adrenocortical carcinoma, mitotane should be administered for very long periods of time as it can be well-tolerated and may be beneficial in the long run." 01/01/1994 - "Complete remission of metastasized adrenocortical carcinoma under o,p'-DDD." 07/01/1990 - "Unlike previous reports of metastatic adrenal cortical carcinoma in which complete remission was obtained with high dose Mitotane treatment, the dose of Mitotane used in this patient was low. " 06/01/2009 - "Metastatic virilizing adrenocortical carcinoma: a rare case of cure with surgery and mitotane therapy."
2.	HormonesIBA 01/01/1984 - "This study concerns the history of a male patient with hormone-producing adrenal cortical carcinoma. " 06/01/2015 - "Steroid hormone related effects of marine persistent organic pollutants in human H295R adrenocortical carcinoma cells." 02/01/2013 - "Adrenocortical carcinomas (ACCs) originate in the steroid hormone-producing adrenal cortex. " 07/01/2012 - "Adrenocortical carcinomas can be functional or may be unassociated with syndromes of hormone overproduction. " 06/01/2011 - "Cyclic estrous-like behavior in a spayed cat associated with excessive sex-hormone production by an adrenocortical carcinoma."
3.	Suramin (Suramin Sodium)IBA 09/01/1994 - "Suramin should not be considered as first-line treatment for metastatic adrenocortical carcinoma. " 09/01/1994 - "(3) To improve treatment options in adrenocortical carcinoma as well as for further investigation on the usefulness of suramin, controlled prospective trials are urgently needed." 09/01/1994 - "(1) Suramin is of antineoplastic efficacy in the treatment of metastatic adrenocortical carcinoma. " 08/01/1990 - "We now confirm preferential accumulation of this compound in the normal adrenal gland, evaluate its in vitro effect against two human adrenocortical carcinoma cell lines (SW-13 and NCI-H295), and report the clinical activity of suramin in 17 patients with metastatic adrenocortical carcinoma. " 07/29/1989 - "Suramin for treatment of adrenocortical carcinoma."
4.	Phosphotransferases (Kinase)IBA 09/01/2008 - "Additionally, a selective IGF-IR kinase inhibitor had antitumor effects in adult and pediatric adrenocortical tumor cell lines, suggesting that IGF-IR inhibitors represent a promising therapy for human adrenocortical carcinoma." 05/01/2010 - "Sustained remission with the kinase inhibitor sorafenib in stage IV metastatic adrenocortical carcinoma." 11/25/2012 - "Efficacy of the novel dual PI3-kinase/mTOR inhibitor NVP-BEZ235 in a preclinical model of adrenocortical carcinoma." 05/01/2010 - "To report our experience using kinase inhibition therapy with sorafenib in a patient with advanced adrenocortical carcinoma. " 09/01/1977 - "Partial purification and characterization of the defective cyclic adenosine 3':5'-monophosphate binding protein kinase from adrenocortical carcinoma."
5.	Hydrocortisone (Cortisol)FDA LinkGeneric 06/01/2012 - "The role of mTOR inhibitors in the inhibition of growth and cortisol secretion in human adrenocortical carcinoma cells." 03/01/2010 - "Adrenocortical carcinoma cell proliferation, cortisol secretion, and cell invasion were measured using immunometric assays. " 07/01/2007 - "Cortisol as a possible marker of metastatic adrenocortical carcinoma: a case report with 3-year follow-up." 06/01/2004 - "VIP was shown in a dose-dependent manner to increase cortisol secretion in the NCI-H295R human adrenocortical carcinoma (H295) cell line (threshold dose 3.3x10(-10) M, maximal dose 10(-7) M), coupled with a parallel increase in cAMP accumulation. " 01/01/1998 - "Four months later the patient presented with a metastasizing cortisol- and androgen-producing adrenocortical carcinoma (ACC). "
6.	Cisplatin (Platino)FDA LinkGeneric 02/01/1987 - "Cisplatin-based treatment of adrenocortical carcinoma." 05/01/1985 - "[Cis-platinum used for the prevention of the recurrence of adrenal cortical carcinoma: report of a case]." 05/01/1983 - "Cisplatin for adrenal cortical carcinoma." 05/28/2013 - "Treatment with docetaxel and cisplatin in advanced adrenocortical carcinoma, a phase II study." 03/01/2000 - "The current study demonstrates that the combination of cisplatin and etoposide has minimal activity in advanced and metastatic adrenocortical carcinoma and other treatment strategies are warranted."
7.	Doxorubicin (Adriamycin)FDA LinkGeneric 05/01/2012 - "Liposomal doxorubicin-based treatment in a preclinical model of adrenocortical carcinoma." 12/01/1991 - "From November 1979 to July 1986, 52 patients (27 women and 25 men; median age 52 years) with advanced adrenocortical carcinoma entered a prospective, nonrandomized study evaluating moderate-dose mitotane and doxorubicin hydrochloride (Adriamycin). " 12/01/1991 - "Eastern Cooperative Oncology Group study 1879: mitotane and adriamycin in patients with advanced adrenocortical carcinoma." 01/01/2001 - "Cisplatin and etoposide as single therapy, or in combination with doxorubicin or etoposide, appear to be effective in adrenocortical carcinoma. " 12/01/2014 - "Mitotane enhances doxorubicin cytotoxic activity by inhibiting P-gp in human adrenocortical carcinoma cells."
8.	Etoposide (VP 16)FDA LinkGeneric 03/01/2000 - "The current study demonstrates that the combination of cisplatin and etoposide has minimal activity in advanced and metastatic adrenocortical carcinoma and other treatment strategies are warranted." 05/01/2003 - "Adrenocortical carcinoma in children: a role for etoposide and cisplatin adjuvant therapy? " 03/01/2000 - "Patients with advanced, unresectable, or metastatic adrenocortical carcinoma with objectively measurable disease or biochemical abnormalities received cisplatin, 50 mg/m(2), intravenously on Days 1 and 2, and etoposide, 100 mg/m(2), on Days 1, 2, and 3. Cycles were repeated every 21 days. " 08/01/1998 - "Cytotoxic therapy with etoposide and cisplatin in advanced adrenocortical carcinoma." 06/01/1996 - "Etoposide plus cisplatin may be an active combination for the treatment of adrenal cortical carcinoma."
9.	DNA (Deoxyribonucleic Acid)IBA 12/01/1987 - "Although adrenocortical carcinoma is in general markedly aggressive, the addition of nuclear DNA ploidy studies may help to identify certain groups of patients who have a relatively favorable prognosis." 04/01/2010 - "Analyzing the tumoral DNA revealed that the AIP wild-type allele was lost in the daughter's somatotropinoma and the mother's adrenocortical carcinoma. " 12/01/1995 - "Surgical management, DNA content, and patient survival in adrenal cortical carcinoma." 07/01/1995 - "DNA sequencing performed on the case showing overexpression of p53 protein revealed no mutation within exons 5 to 8. The associated adrenal cortical carcinoma of the same case also showed overexpression of p53 protein, but no mutation of the p53 gene. " 07/01/1993 - "We conclude that nuclear DNA content is related to (disease-free) survival of patients with adrenocortical carcinomas. "
10.	Ketoconazole (Nizoral)FDA LinkGeneric 01/01/1993 - "[Malignant adrenocortical carcinoma and ketoconazole]." 12/01/1991 - "Clinical utility of ketoconazole in cases of adrenocortical carcinoma." 08/01/1989 - "Use of ketoconazole in the treatment of a virilizing adrenocortical carcinoma." 07/15/1989 - "Preoperative ketoconazole therapy for adrenocortical carcinoma." 04/01/1991 - "Favourable response of a virilizing adrenocortical carcinoma to preoperative treatment with ketoconazole and postoperative chemotherapy."

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 03/01/2008 - "The CYP11B1-activated adrenocortical toxicant 3-methylsulphonyl-DDE (3-MeSO2-DDE) is proposed as a lead compound for an improved chemotherapy for adrenocortical carcinoma. " 01/01/2011 - "Reported responses to conventional cytotoxic chemotherapy have also been disappointing, and the rarity of ACC had hampered the ability to undertake randomized clinical studies until the establishment of the First International Randomized Trial in Locally Advanced and Metastatic Adrenocortical Carcinoma. " 07/01/2008 - "The objective of this study was to evaluate and compare the efficacies of conventional first-line chemotherapies for adrenocortical carcinoma. " 10/01/2015 - "Adrenocortical carcinoma is a rare malignancy with poor prognosis and limited response to chemotherapy. " 04/01/2015 - "Patients were randomly assigned (2:1) twice-daily 150 mg oral linsitinib or placebo via a web-based, centralised randomisation system and stratified according to previous systemic cytotoxic chemotherapy for adrenocortical carcinoma, Eastern Cooperative Oncology Group performance status, and use of one or more oral antihyperglycaemic therapy at randomisation. "
2.	Adrenalectomy 06/01/2015 - "The aim of the present meta-analysis was to investigate the safety and oncologic efficacy of laparoscopic adrenalectomy for stage I and II adrenocortical carcinoma. " 01/01/2015 - "Although waiting for further more exhaustive studies, we think that for suspected adrenocortical carcinoma, smaller than 8-10 cm and without pre or intraoperative evidence of local invasion, endoscopic adrenalectomy in a referral center seems to be an acceptable option." 07/01/2015 - "Laparoscopic adrenalectomy (LA) is performed, even for large adrenocortical carcinomas. " 07/01/2015 - "Laparoscopic Adrenalectomy for Large Adrenocortical Carcinoma." 06/01/2015 - "Laparoscopic versus Open Adrenalectomy for Stage I/II Adrenocortical Carcinoma: Meta-Analysis of Outcomes."
3.	Adjuvant Radiotherapy 01/01/2013 - "A retrospective cohort analysis of the efficacy of adjuvant radiotherapy after primary surgical resection in patients with adrenocortical carcinoma." 11/01/2006 - "Efficacy of adjuvant radiotherapy of the tumor bed on local recurrence of adrenocortical carcinoma."
4.	Transplants (Transplant) 09/01/2000 - "In a 29-year-old patient with adrenocortical carcinoma with metastases to the lung and bone, an adjuvant bone marrow graft using peripheral stem cells was unsuccessful. " 01/01/1966 - "Polycythemia in rats with adrenal cortical carcinoma transplants." 04/01/2006 - "Although this experience is limited, we suggest that TIP chemotherapy was effective for adrenocortical carcinoma, and a graft-versus-tumor effect after reduced-intensity stem cell transplantation may have contributed to the prolonged survival." 11/01/1969 - "The origin of erythropoietin in rats with transplants of an adrenal cortical carcinoma."
5.	Stem Cell Transplantation 04/01/2006 - "Although this experience is limited, we suggest that TIP chemotherapy was effective for adrenocortical carcinoma, and a graft-versus-tumor effect after reduced-intensity stem cell transplantation may have contributed to the prolonged survival."