MCID: ADR015
MIFTS: 49

Adrenocortical Carcinoma malady

Rare diseases, Cancer diseases, Cardiovascular diseases, Nephrological diseases, Reproductive diseases, Endocrine diseases categories

Summaries for Adrenocortical Carcinoma

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Disease Ontology:9 An adrenal cortex cancer that forms in the outer layer of tissue of the adrenal gland and that has material basis in abnormally proliferating cells derives from epithelial cells.

MalaCards based summary: Adrenocortical Carcinoma, also known as malignant neoplasm of adrenal cortex, is related to conn's syndrome and cushing's syndrome. An important gene associated with Adrenocortical Carcinoma is TP53 (tumor protein p53), and among its related pathways are Inhibition of Ribosome Biogenesis by p14(ARF) and Nuclear Receptor transcription pathway. The compounds 17beta-hydroxysteroid and dmrt have been mentioned in the context of this disorder. Affiliated tissues include cortex, adrenal cortex and adrenal gland, and related mouse phenotypes are cardiovascular system and reproductive system.

Wikipedia:63 Adrenocortical carcinoma, also adrenal cortical carcinoma (ACC) and adrenal cortex cancer, is an... more...

Aliases & Classifications for Adrenocortical Carcinoma

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Sources:
9Disease Ontology, 41NIH Rare Diseases, 11DISEASES, 43Novoseek, 47Orphanet, 60UMLS, 38NCIt, 55SNOMED-CT, 33MeSH, 34MESH via Orphanet, 26ICD10 via Orphanet, 61UMLS via Orphanet, 25ICD10
See all sources

Adrenocortical Carcinoma, Aliases & Descriptions:

Name: Adrenocortical Carcinoma 9 41 11 47 60
Malignant Neoplasm of Adrenal Cortex 60
Carcinoma of the Adrenal Cortex 9
Agenesis of Corpus Callosum 60
Adrenal Cortical Carcinoma 9
 
Carcinoma Adrenocortical 43
Adrenocortical Cancer 43
Adrenal Cortex Cancer 11
Acc 41


Classifications:



Characteristics (Orphanet epidemiological data):

47
adrenocortical carcinoma:
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe); Age of onset: Childhood


External Ids:

Disease Ontology9 DOID:3948
NCIt38 C9325
MeSH33 D018268
Orphanet47 1501
SNOMED-CT55 255035007, 2227007
MESH via Orphanet34 D018268
ICD10 via Orphanet26 C74.0
UMLS via Orphanet61 C0206686
ICD1025 C74.0

Related Diseases for Adrenocortical Carcinoma

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Diseases related to Adrenocortical Carcinoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 112)
idRelated DiseaseScoreTop Affiliating Genes
1conn's syndrome31.1CYP11B2
2cushing's syndrome30.9NR0B1, INHA, CYP11B2
3beckwith-wiedemann syndrome30.8TSSC4, TSPAN32, PHLDA2
4adrenal cortical adenoma30.5NR0B1, NR5A1, INHA
5pheochromocytoma30.2NR5A1, UTS2, INHA
6adenocarcinoma29.9INHA, TP53, TSPAN32, NR5A1
7rhabdomyosarcoma29.8UTS2, TP53, INHA
8adenoma29.7CYP11B2, INHA, TP53, FSHB, NR5A1, NR0B1
9obesity29.6INHA, NR5A1, NR0B1
10renal cell carcinoma10.5
1146xy sex reversal 310.4NR5A1
12adenoiditis10.4
13adrenal cortical carcinoma10.4
14hyperaldosteronism10.4
15li-fraumeni syndrome10.4
16adrenocortical carcinoma with pure aldosterone hypersecretion10.4
17salivary gland adenoid cystic carcinoma10.4
18premature ovarian failure10.3INHA
19thyroiditis10.3
20agenesis of the corpus callosum10.3
21peripheral vascular disease10.3
22vascular disease10.3
23hepatitis10.3
24central serous chorioretinopathy10.3
2546,xy disorder of sex development and 46,xy complete gonadal dysgenesis10.2NR0B1, NR5A1
26adrenal hypoplasia, congenital, with hypogonadotropic hypogonadism10.2NR5A1, NR0B1
27gonadal dysgenesis10.2NR0B1, NR5A1
28myocardial infarction10.2
29hereditary motor and sensory neuropathy with agenesis of the corpus callosum10.2
30gestational trophoblastic neoplasm10.2INHA, PHLDA2
31kallmann syndrome10.2NR0B1, NR5A1
32sex cord-gonadal stromal tumor10.2NR5A1, INHA
33hepatocellular carcinoma10.2
34choroiditis10.2
35choroid plexus carcinoma10.2
36adrenal carcinoma10.2
37peritonitis10.2
38thyroid cancer10.2
39turner syndrome10.2
40multiple endocrine neoplasia10.2
41abrikosov's tumor10.2NR5A1, INHA
42luteoma10.2
43von willebrand's disease10.2
44angiosarcoma10.2
45wilms tumor10.1NR5A1, TP53
46adrenoleukodystrophy10.1NR0B1, NR5A1
47aplasia cutis congenita, nonsyndromic10.1
48thalassemia10.1
49overuse syndrome10.1
50congenital adrenal hyperplasia10.1CYP11B2, NR5A1, NR0B1

Graphical network of the top 20 diseases related to Adrenocortical Carcinoma:



Diseases related to adrenocortical carcinoma

Symptoms for Adrenocortical Carcinoma

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Drugs & Therapeutics for Adrenocortical Carcinoma

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Drug clinical trials:

Search ClinicalTrials for Adrenocortical Carcinoma

Search NIH Clinical Center for Adrenocortical Carcinoma

Genetic Tests for Adrenocortical Carcinoma

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Anatomical Context for Adrenocortical Carcinoma

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MalaCards organs/tissues related to Adrenocortical Carcinoma:

31
Cortex, Adrenal cortex, Adrenal gland, Liver, Thyroid, Kidney, Lung, Bone, Ovary, Prostate, Testes, T cells, Endothelial

Animal Models for Adrenocortical Carcinoma or affiliated genes

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MGI Mouse Phenotypes related to Adrenocortical Carcinoma:

35
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053858.5TBX2, NR5A1, TP53, INHA, CYP11B2
2MP:00053898.1INHA, TP53, FSHB, NR5A1, NR0B1
3MP:00053787.9CYP11B2, INHA, TP53, NR5A1, TBX2, PHLDA2
4MP:00053797.9NR0B1, NR5A1, FSHB, TP53, INHA, CYP11B2
5MP:00053767.5CYP11B2, INHA, TP53, FSHB, NR5A1, NR0B1

Publications for Adrenocortical Carcinoma

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Articles related to Adrenocortical Carcinoma:

(show top 50)    (show all 566)
idTitleAuthorsYear
1
Steroid hormone related effects of marine persistent organic pollutants in human H295R adrenocortical carcinoma cells. (25765474)
2015
2
Programmed death ligand-1 expression in adrenocortical carcinoma: an exploratory biomarker study. (25767716)
2015
3
Molecular markers and targeted therapies for adrenocortical carcinoma. (24304415)
2014
4
Myxoid variant of adrenocortical carcinoma: a report of two illustrative cases and a brief review of the literature. (24300732)
2014
5
Treatment patterns and outcomes for patients with adrenocortical carcinoma associated with hospital case volume in the United States. (25069860)
2014
6
Comparative diagnostic and prognostic performances of the hematoxylin-eosin and phospho-histone H3 mitotic count and Ki-67 index in adrenocortical carcinoma. (24434900)
2014
7
CYP2W1 is highly expressed in adrenal glands and is positively associated with the response to mitotane in adrenocortical carcinoma. (25144458)
2014
8
Giant nonfunctioning adrenocortical carcinoma: a case report and review of the literature. (25361816)
2014
9
Functioning Adrenocortical Carcinoma with Extension upto the Right Atrium Producing Cushing's Syndrome. (24083069)
2013
10
Adjuvant Therapies, Patient and Tumor Characteristics Associated with Survival of Adult Patients with Adrenocortical Carcinoma. (24302750)
2013
11
A rare coexistence of non-functional adrenocortical carcinoma and multicentric papillary thyroid microcarcinoma: a case report. (23889916)
2013
12
Mini-invasive surgery for adrenocortical carcinoma in children: is it safe? (23444873)
2013
13
Primary rhabdoid adrenocortical carcinoma: a clinicopathological and immunohistochemical study of three cases. (23445527)
2013
14
Serum inhibin pro-I+C is a tumor marker for adrenocortical carcinomas. (22127493)
2012
15
Childhood adrenocortical carcinoma as a sentinel cancer for detecting families with germline TP53 mutations. (22233476)
2012
16
Liposomal doxorubicin-based treatment in a preclinical model of adrenocortical carcinoma. (22407999)
2012
17
Adrenocortical carcinoma: effect of hospital volume on patient outcome. (22069043)
2012
18
Sunitinib in refractory adrenocortical carcinoma: a phase II, single-arm, open-label trial. (22837187)
2012
19
Adrenocortical carcinoma: past, present, and future. (22473597)
2012
20
Adrenocortical stem and progenitor cells: implications for adrenocortical carcinoma. (22266195)
2012
21
Surgery in adrenocortical carcinoma: Importance of national cooperation and centralized surgery. (22703895)
2012
22
A new mutation in the menin gene causes the multiple endocrine neoplasia type 1 syndrome with adrenocortical carcinoma. (21069576)
2011
23
Metronomic therapy concepts in the management of adrenocortical carcinoma. (21971765)
2011
24
Feminizing adrenocortical carcinoma with selective suppression of follicle-stimulating hormone secretion and disorganized steroidogenesis: a case report and literature review. (21720063)
2011
25
Significance of heparanase-1 and vascular endothelial growth factor in adrenocortical carcinoma angiogenesis: potential for therapy. (21706269)
2011
26
Rare pediatric adrenocortical carcinoma with oncocytic change: a cytologic dilemma. (21181300)
2011
27
Adrenocortical carcinoma presenting with a peritoneal effusion. (19941369)
2010
28
Fine needle aspiration cytology of adrenocortical carcinoma--case report. (20698150)
2010
29
Diagnosis: Novel molecular signatures for adrenocortical carcinoma. (19465894)
2009
30
Ectopic low-grade adrenocortical carcinoma in the spinal region: immunohistochemical and molecular cytogenetic study of a pediatric case. (18941403)
2009
31
Management of adrenocortical carcinoma. (19635227)
2009
32
Clinical management of adrenocortical carcinoma. (19500769)
2009
33
Percutaneous laser ablation of unresectable primary and metastatic adrenocortical carcinoma. (17498906)
2008
34
Bilateral primary adrenal non-Hodgkin's lymphoma and primary adrenocortical carcinoma--review of the literature preoperative differentiation of adrenal tumors. (18490838)
2008
35
Side population does not define stem cell-like cancer cells in the adrenocortical carcinoma cell line NCI h295R. (18063677)
2008
36
Alpha-fetoprotein (AFP)-producing adrenocortical carcinoma--long survival with various therapeutic strategies including a lung resection: report of a case. (18307006)
2008
37
Adjuvant mitotane in adrenocortical carcinoma. (17891836)
2007
38
Efficacy of adjuvant radiotherapy of the tumor bed on local recurrence of adrenocortical carcinoma. (16895957)
2006
39
Adrenocortical carcinoma extending into the inferior vena cava: presentation of a 15-patient series and review of the literature. (16364713)
2006
40
Etoposide, doxorubicin and cisplatin plus mitotane in the treatment of advanced adrenocortical carcinoma: a large prospective phase II trial. (16172198)
2005
41
Ruptured adrenocortical carcinoma as a cause of paediatric acute abdomen. (15711359)
2005
42
Neuropeptides B and W enhance the growth of human adrenocortical carcinoma-derived NCI-H295 cells by exerting MAPK p42/p44-mediated proliferogenic and antiapoptotic effects. (16273281)
2005
43
Management of adrenocortical carcinoma. (15008991)
2004
44
Pregnancy with functioning adrenocortical carcinoma. (12126365)
2002
45
Adrenocortical carcinoma: clinical, morphologic, and molecular characterization. (11844815)
2002
46
Virilising adrenocortical carcinoma. (12405338)
2002
47
Selective testosterone secreting adrenocortical carcinoma in an infant. (11237246)
2001
48
Special feature: pathological case of the month. Denouement and discussion: congenital adrenocortical carcinoma. (11115315)
2000
49
Novel splicing of an IGF2 polymorphic region in human adrenocortical carcinomas. (9367863)
1997
50
Detection of novel germ-line p53 mutations in diverse-cancer-prone families identified by selecting patients with childhood adrenocortical carcinoma. (1569604)
1992

Variations for Adrenocortical Carcinoma

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Cosmic variations for Adrenocortical Carcinoma:

7 (show all 64)
id Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Conf
110911TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
246274TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
310659TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
443755TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
51716843TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
6144153TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
7143792TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
810648TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
943963TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
1044816TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
111685349TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
121191164TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
1344033TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
1444288TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
1510769TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
1644082TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
1710722TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
1810645TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
1910725TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
2011524TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
2144091TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
2244081TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
2345995TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
2444623TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
256815TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
2610670TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
2796436TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
284169324TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
2910790TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
3011066TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
31133677TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
324169704TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
331717459TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
34329725TP53adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
3512639CTNNB1adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
3629398CTNNB1adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
375663CTNNB1adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
3885770CTNNB1adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
395664CTNNB1adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
4013153CTNNB1adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
4153039CTNNB1adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
4213154CTNNB1adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
435667CTNNB1adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
4428929EGFRadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
4513198EGFRadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
4628930EGFRadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
4712764EGFRadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
4828931EGFRadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
4928574EGFRadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
5013197EGFRadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
51574NRASadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
5228933NRASadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
53584NRASadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
544169712ATMadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
554169840ATMadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
564169841ATMadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
57476BRAFadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
58449BRAFadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
594169854APCadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
604169853CDKN2Aadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
61516KRASadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
62210753STK11adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
6329325PIK3CAadrenal gland,adrenal gland,adrenal cortical carcinoma,NS3
6423054MEN1adrenal gland,adrenal gland,adrenal cortical carcinoma,NS3

Expression for genes affiliated with Adrenocortical Carcinoma

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Search GEO for disease gene expression data for Adrenocortical Carcinoma.

Pathways for genes affiliated with Adrenocortical Carcinoma

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Pathways related to Adrenocortical Carcinoma according to GeneCards Suite gene sharing:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8TBX2, TP53
2
Show member pathways
Nuclear Receptors36
9.6NR0B1, NR5A1
39.5NR5A1, INHA
4
Show member pathways
9.3FSHB, INHA

Compounds for genes affiliated with Adrenocortical Carcinoma

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Compounds related to Adrenocortical Carcinoma according to GeneCards Suite gene sharing:

(show all 25)
idCompoundScoreTop Affiliating Genes
117beta-hydroxysteroid439.9CYP11B2, NR5A1
2dmrt439.9NR0B1, NR5A1
3dhea439.8NR5A1, CYP11B2
4ptx1439.7NR5A1, FSHB
5radicicol43 59 1211.6UTS2, TP53
6proline439.5CYP11B2, TP53, NR5A1
73beta-hydroxysteroid439.5NR5A1, INHA, CYP11B2
8actinomycin d439.4NR5A1, FSHB, TP53
9forskolin43 49 1211.3NR5A1, INHA, CYP11B2
10leucine439.2NR0B1, NR5A1, CYP11B2
11estradiol43 24 1211.1INHA, FSHB, NR5A1
12cyclic amp43 2410.0CYP11B2, INHA, NR5A1, NR0B1
13thymidine43 2410.0TP53, UTS2, NR5A1
14ribonucleic acid438.9NR5A1, FSHB, INHA, CYP11B2
15gnrh438.8INHA, FSHB, NR5A1, NR0B1
16sodium43 249.8NR5A1, UTS2, CYP11B2
17oligonucleotide438.6CYP11B2, INHA, TP53, FSHB, NR5A1
18estrogen438.5INHA, TP53, FSHB, NR5A1, NR0B1
19acth438.5NR0B1, NR5A1, FSHB, INHA, CYP11B2
20testosterone43 59 24 1211.4CYP11B2, INHA, FSHB, NR5A1, NR0B1
21steroid438.4CYP11B2, INHA, FSHB, NR5A1, NR0B1
22cysteine438.3NR0B1, FSHB, TSPAN32, UTS2
23cholesterol43 28 24 1211.2CYP11B2, UTS2, TSPAN32, NR5A1, NR0B1
24progesterone43 28 59 24 1212.2CYP11B2, INHA, TP53, FSHB, NR5A1, NR0B1
25lipid438.0CYP11B2, UTS2, TSPAN32, NR5A1

GO Terms for genes affiliated with Adrenocortical Carcinoma

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Biological processes related to Adrenocortical Carcinoma according to GeneCards Suite gene sharing:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1cell agingGO:000756910.0TP53, TBX2
2adrenal gland developmentGO:00303259.8NR0B1, NR5A1
3protein localizationGO:00081049.6NR0B1, TP53
4intracellular receptor signaling pathwayGO:00305229.6NR5A1, NR0B1
5negative regulation of transcription, DNA-templatedGO:00458929.4TBX2, NR0B1, TP53
6male gonad developmentGO:00085849.3NR0B1, NR5A1, INHA
7cell-cell signalingGO:00072679.1NR5A1, TSPAN32, INHA
8cell differentiationGO:00301549.0NR5A1, TP53, INHA

Molecular functions related to Adrenocortical Carcinoma according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1steroid hormone receptor activityGO:00037079.6NR0B1, NR5A1
2ligand-activated sequence-specific DNA binding RNA polymerase II transcription factor activityGO:00048799.3NR0B1, NR5A1
3hormone activityGO:00051798.4FSHB, UTS2, INHA

Products for genes affiliated with Adrenocortical Carcinoma

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Sources for Adrenocortical Carcinoma

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet