ACC
MCID: ADR015
MIFTS: 63

Adrenocortical Carcinoma (ACC) malady

Cancer diseases, Cardiovascular diseases, Nephrological diseases, Endocrine diseases categories

Summaries for Adrenocortical Carcinoma

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8Disease Ontology, 63Wikipedia, 46OMIM, 32MalaCards
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Wikipedia:63 Adrenocortical carcinoma, also adrenal cortical carcinoma (ACC) and adrenal cortex cancer, is an... more...

MalaCards: Adrenocortical Carcinoma, also known as adrenal cortical carcinoma, is related to cushing's syndrome and adenoma. 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 Glucuronidation. The compounds acth and progesterone have been mentioned in the context of this disorder. Affiliated tissues include cortex, adrenal cortex and adrenal gland, and related mouse phenotypes are growth/size and endocrine/exocrine gland.

Disease Ontology:8 An adrenal carcinoma that forms in the outer layer of tissue of the adrenal gland.

Description from OMIM:46 202300

Aliases & Classifications for Adrenocortical Carcinoma

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8Disease Ontology, 9diseasecard, 42NIH Rare Diseases, 46OMIM, 10DISEASES, 44Novoseek, 48Orphanet, 60UMLS, 39NCIt, 34MeSH, 56SNOMED-CT, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet, 25ICD10
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Classifications:



Characteristics (Orphanet epidemiological data):

48
adrenocortical carcinoma:
Inheritance: Sporadic; Prevalence: 1-9/1000000; Age of onset: Childhood


Aliases & Descriptions:

adrenocortical carcinoma 8 42 10 48 60
adrenal cortical carcinoma 8 9 46
carcinoma of the adrenal cortex 8
carcinoma adrenocortical 44
adrenocortical cancer 44
adrenal cortex cancer 10
acc 42


External Ids:

Disease Ontology8 DOID:3948
NCIt39 C9325
MeSH34 D018268
OMIM46 202300
MESH via Orphanet35 D018268
SNOMED-CT56 2227007, 255035007
ICD10 via Orphanet26 C74.0, E24.8
SNOMED-CT via Orphanet57 2227007
UMLS via Orphanet61 C0206686
ICD1025 C74.0

Related Diseases for Adrenocortical Carcinoma

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17GeneCards, 18GeneDecks
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Diseases related to Adrenocortical Carcinoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 113)
idRelated DiseaseScoreTop Affiliating Genes
1cushing's syndrome30.7INHA, CYP11A1, CYP11B2, CYP11B1, NR0B1
2adenoma30.6NR5A1, NR0B1, CYP11B1, CYP11B2, CYP11A1, INHA
3hypertension30.5CYP11B1, CYP11B2, ADM
4pheochromocytoma30.4NR5A1, INHA, ADM
5conn's syndrome30.3CYP11B1, CYP11B2
6beckwith-wiedemann syndrome30.2PHLDA2, NAP1L4, SLC22A18, TSSC4, TSPAN32
7adrenal adenoma30.1ADM, CYP11A1, CYP11B2, CYP11B1
8adenocarcinoma30.1NR5A1, INHA, TSPAN32, ADM, TP53
9rhabdomyosarcoma29.9INHA, SLC22A18, TP53
10obesity29.8NPB, NR5A1, NR0B1, CYP11A1, INHA
11adrenal carcinoma10.7
12adenoid cystic carcinoma10.4
13adenoiditis10.4
14renal cell carcinoma10.4
15hyperaldosteronism10.4
16li-fraumeni syndrome10.3
17thyroiditis10.3
18adrenocortical carcinoma with pure aldosterone hypersecretion10.3
19hepatitis10.2
20peripheral vascular disease10.2
21vascular disease10.2
22corpus callosum agenesis10.2
23myocardial infarction10.1
24bone carcinoma10.1
25neuroendocrine carcinoma10.1
26papillary carcinoma10.1
27choroiditis10.1
28choroid plexus carcinoma10.1
29hepatocellular carcinoma10.1
30lynch syndrome10.1
31osteosarcoma10.1
32papillary thyroid carcinoma10.1
33peritonitis10.1
34thyroid cancer10.1
35turner syndrome10.1
36von willebrand's disease10.1
37carney complex10.1
38luteoma10.1
39adrenal cortical adenocarcinoma10.1
40pancreatic acinar cell adenocarcinoma10.1
41alpha thalassemia10.1
42cutaneous adenocystic carcinoma10.1
43lung adenoid cystic carcinoma10.1
44esophagitis10.1
45thalassemia10.1
46colorectal cancer10.1
47hereditary motor and sensory neuropathy with agenesis of the corpus callosum10.1
48circumscribed cutaneous aplasia of the vertex10.1
49hypoxia10.1
50wilms tumor10.0SLC22A18, INHA, NAP1L4, NR0B1, NR5A1

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



Diseases related to adrenocortical carcinoma

Clinical Features for Adrenocortical Carcinoma

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46OMIM
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Clinical features from OMIM:

202300

Clinical synopsis from OMIM:

202300

Drugs & Therapeutics for Adrenocortical Carcinoma

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Adrenocortical Carcinoma

Drug clinical trials:

Search ClinicalTrials for Adrenocortical Carcinoma

Search NIH Clinical Center for Adrenocortical Carcinoma

Search CenterWatch for Adrenocortical Carcinoma

Genetic Tests for Adrenocortical Carcinoma

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Anatomical Context for Adrenocortical Carcinoma

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32MalaCards
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MalaCards organs/tissues related to Adrenocortical Carcinoma:

32
Cortex, Adrenal cortex, Adrenal gland, Liver, Thyroid, Kidney, Bone, Lung, Breast, Ovary, Prostate, T cells, Endothelial, Colon, Pituitary

Animal Models for Adrenocortical Carcinoma or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Adrenocortical Carcinoma:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000537810.5PHLDA2, NR5A1, TBX2, NUP98, CYP11B2, CYP11A1
2MP:000537910.2TP53, NR5A1, NR0B1, CYP11B1, CYP11B2, CYP11A1

Publications for Adrenocortical Carcinoma

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50PubMed
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Articles related to Adrenocortical Carcinoma:

(show top 50)    (show all 547)
idTitleAuthorsYear
1
Adjuvant Therapies, Patient and Tumor Characteristics Associated with Survival of Adult Patients with Adrenocortical Carcinoma. (24302750)
2013
2
Treatment with docetaxel and cisplatin in advanced adrenocortical carcinoma, a phase II study. (23652308)
2013
3
Gaining traction in the treatment of adrenocortical carcinoma. (23293337)
2013
4
Role of radiotherapy in adrenocortical carcinoma. (23867507)
2013
5
Does laparoscopic adrenalectomy jeopardize oncologic outcomes for patients with adrenocortical carcinoma? (23765427)
2013
6
Adrenocortical carcinoma presenting with signs of acute abdomen. (23424707)
2013
7
Adrenocortical carcinoma: past, present, and future. (22473597)
2012
8
An unusual presentation of congenital adrenocortical carcinoma: a case report and review of the literature. (22542918)
2012
9
Aldosterone-producing adrenocortical carcinoma without hypertension. (22707896)
2012
10
The role of mTOR inhibitors in the inhibition of growth and cortisol secretion in human adrenocortical carcinoma cells. (22420007)
2012
11
Association of adrenocortical carcinoma with familial cancer susceptibility syndromes. (22209747)
2012
12
Efficacy of the novel dual PI3-kinase/mTOR inhibitor NVP-BEZ235 in a preclinical model of adrenocortical carcinoma. (22960230)
2012
13
Rare pediatric adrenocortical carcinoma with oncocytic change: a cytologic dilemma. (21181300)
2011
14
Potent inhibitory effect of the cyclolignan picropodophyllin (PPP) on human adrenocortical carcinoma cells proliferation. (21968616)
2011
15
Therapeutic management of advanced adrenocortical carcinoma: what do we know in 2011? (22161625)
2011
16
Improved survival in patients with stage II adrenocortical carcinoma followed up prospectively by specialized centers. (20668036)
2010
17
Re: David Brix, Bruno Allolio, Wiebke Fenske, et al. Laparoscopic versus open adrenalectomy for adrenocortical carcinoma: surgical and oncologic outcome in 152 patients. Eur Urol 2010;58:609-15. (20864252)
2010
18
Computed tomography of adrenocortical carcinoma containing macroscopic fat. (21067996)
2010
19
External validation of a nomogram predicting mortality in patients with adrenocortical carcinoma. (19493261)
2009
20
Osteopontin stimulates invasion of NCI-h295 cells but is not associated with survival in adrenocortical carcinoma. (19326399)
2009
21
Identification of a novel TP53 germline mutation in one child with adrenocortical carcinoma]. (19671334)
2009
22
Early detection of adrenocortical carcinoma in a child with Li-Fraumeni syndrome. (19101993)
2009
23
Exploratory analysis of potential risk factors of a rare disease: spatial distribution of adrenocortical carcinoma in Israel as a case study. (19042010)
2009
24
Alpha-fetoprotein (AFP)-producing adrenocortical carcinoma--long survival with various therapeutic strategies including a lung resection: report of a case. (18307006)
2008
25
Expression of the zinc-finger transcription factor Snail in adrenocortical carcinoma is associated with decreased survival. (19018264)
2008
26
Development of an adrenocorticotropin-responsive human adrenocortical carcinoma cell line. (18713819)
2008
27
Functional oncocytic adrenocortical carcinoma. (18058268)
2007
28
Adjuvant mitotane in adrenocortical carcinoma. (17891837)
2007
29
Prognostic parameters of metastatic adrenocortical carcinoma. (17062775)
2007
30
Efficacy of adjuvant radiotherapy of the tumor bed on local recurrence of adrenocortical carcinoma. (16895957)
2006
31
Adrenocortical carcinoma extending into the inferior vena cava: presentation of a 15-patient series and review of the literature. (16364713)
2006
32
Pigmented adrenocortical carcinoma: case report and review. (17308367)
2006
33
Recent advances in histopathology and immunohistochemistry of adrenocortical carcinoma. (17525483)
2006
34
Dendritic cells as potential adjuvant for immunotherapy in adrenocortical carcinoma. (16886963)
2006
35
Use of [18F]fluorodeoxyglucose positron emission tomography in evaluating locally recurrent and metastatic adrenocortical carcinoma. (16621901)
2006
36
Application of cardiopulmonary bypass for resection of renal cell carcinoma and adrenocortical carcinoma extending into the right atrium. (16643609)
2006
37
From incidentaloma to adrenocortical carcinoma: the surgical management of adrenal tumors. (15719374)
2005
38
Rhabdomyosarcoma, osteosarcoma, and adrenocortical carcinoma in a child with a germline p53 mutation. (15390294)
2004
39
Current perspective in the diagnosis and treatment of adrenocortical carcinoma. (11705136)
2001
40
Adrenocortical carcinoma: surgical progress or status quo? (11343545)
2001
41
Production and secretion of two vasoactive peptides, adrenomedullin and endothelin-1, by cultured human adrenocortical carcinoma cells. (10764953)
2000
42
The use of FDG-PET and CT for the staging of adrenocortical carcinoma in children. (10836591)
2000
43
Right atrial thrombus from an adrenocortical carcinoma. (10490573)
1999
44
Autocrine role of IGF-II in proliferation of human adrenocortical carcinoma NCI H295R cell line. (10425444)
1999
45
Adrenocortical insufficiency associated with long-term high-dose fosfestrol therapy for prostatic carcinoma. (10526944)
1999
46
YM116, 2-(1H-imidazol-4-ylmethyl)-9H-carbazole, decreases adrenal androgen synthesis by inhibiting C17-20 lyase activity in NCI-H295 human adrenocortical carcinoma cells. (10202857)
1999
47
Adrenocortical scintigraphy with 131I-6-beta-iodomethyl-norcholesterol (NP 59) in bilateral adrenocortical carcinoma. (9650214)
1998
48
Coexistence of an adrenocortical carcinoma with an abdominal ganglioneuroma in a child. (9569273)
1998
49
Mechanism of abnormal production of adrenal androgens in patients with adrenocortical adenomas and carcinomas. (8288710)
1994
50
Cytokeratin expression in adrenocortical neoplasia: an immunohistochemical and biochemical study with implications for the differential diagnosis of adrenocortical, hepatocellular, and renal cell carcinoma. (1371262)
1992

Genetic Variations for Adrenocortical Carcinoma

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Expression for genes affiliated with Adrenocortical Carcinoma

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Adrenocortical Carcinoma

Search GEO for disease gene expression data for Adrenocortical Carcinoma.

Pathways for genes affiliated with Adrenocortical Carcinoma

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51QIAGEN, 37NCBI BioSystems Database, 29KEGG, 53Reactome, 12EMD Millipore
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Compounds for genes affiliated with Adrenocortical Carcinoma

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44Novoseek, 59Tocris Bioscience, 28IUPHAR, 11DrugBank, 24HMDB, 49PharmGKB, 2BitterDB
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Compounds related to Adrenocortical Carcinoma according to GeneCards/GeneDecks:

(show all 39)
idCompoundScoreTop Affiliating Genes
1acth4410.9ADM, NR5A1, NR0B1, CYP11B1, CYP11B2, CYP11A1
2progesterone44 59 28 11 2414.9NR5A1, NR0B1, CYP11B1, CYP11B2, CYP11A1, INHA
33beta-hydroxysteroid4410.9NR5A1, CYP11B1, CYP11B2, CYP11A1, INHA
4cyclic amp44 2411.9NR5A1, NR0B1, CYP11B1, CYP11B2, CYP11A1, INHA
5corticosterone44 59 2412.9NPW, NR5A1, CYP11B1, CYP11B2, CYP11A1, ADM
6testosterone44 59 11 2413.9NR5A1, NR0B1, CYP11B1, CYP11B2, CYP11A1, INHA
7cholesterol44 28 11 2413.9NR5A1, NR0B1, CYP11B1, CYP11B2, CYP11A1, TSPAN32
8doca4410.9CYP11A1, CYP11B2, CYP11B1
9steroid4410.9NR5A1, NR0B1, CYP11B1, CYP11B2, CYP11A1, INHA
10forskolin44 49 1112.9CYP11B1, CYP11B2, ADM
11oligonucleotide4410.9NR5A1, NUP98, CYP11B1, CYP11B2, INHA, ADM
12aldosterone28 11 2412.9ADM, CYP11B2, CYP11B1
13androstenedione44 2411.9CYP11B1, CYP11B2, CYP11A1, INHA
14ribonucleic acid4410.8NR5A1, CYP11B1, CYP11B2, CYP11A1, INHA
1518-hydroxycortisol4410.8CYP11B2, CYP11B1
1618-oxocortisol4410.8CYP11B2, CYP11B1
1718-hydroxydeoxycorticosterone4410.8CYP11B2, CYP11B1
1817-hydroxyprogesterone44 2411.8CYP11B1, CYP11B2, CYP11A1
1911beta-hydroxysteroid4410.8CYP11A1, CYP11B2, CYP11B1
2018-hydroxycorticosterone44 2411.8CYP11B2, CYP11B1
2121-deoxycortisol44 2411.8CYP11B2, CYP11B1
2217beta-hydroxysteroid4410.8NR5A1, CYP11B2, CYP11A1
23dmrt4410.8NR0B1, NR5A1
24gnrh4410.8INHA, CYP11A1, NR0B1, NR5A1
25deoxycorticosterone28 2411.8CYP11B1, CYP11B2
26pcb 1264410.8CYP11B2, CYP11B1
27dhea4410.7CYP11A1, CYP11B2, NR5A1
28etomidate44 1111.7CYP11B2, CYP11B1
29cortisol28 2411.7CYP11B1, CYP11B2
30dbc-amp4410.7ADM, CYP11A1, CYP11B2
31estrogen4410.7NR5A1, NR0B1, CYP11A1, INHA, ADM, TP53
3211 deoxycortisol4410.6CYP11B2, CYP11B1
3322r-hydroxycholesterol44 2811.6NR5A1, CYP11A1
34pcbs4410.6CYP11B1, CYP11B2
35actinomycin d4410.6NR5A1, CYP11A1, ADM, TP53
36estradiol44 11 2412.5INHA, CYP11A1, CYP11B1, NR5A1
37hydrocortisone44 2 59 1113.5ADM, CYP11B2, CYP11B1
38dexamethasone44 49 28 1113.4NR0B1, CYP11B1, CYP11B2, CYP11A1, ADM
39potassium44 11 2412.2NR5A1, CYP11B1, CYP11B2, ADM

GO Terms for genes affiliated with Adrenocortical Carcinoma

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16Gene Ontology
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Biological processes related to Adrenocortical Carcinoma according to GeneCards/GeneDecks:

(show all 12)
idNameGO IDScoreTop Affiliating Genes
1C21-steroid hormone biosynthetic processGO:00670010.7CYP11A1, CYP11B2, CYP11B1
2sterol metabolic processGO:01612510.7CYP11A1, CYP11B2, CYP11B1
3cortisol biosynthetic processGO:03465110.7CYP11B1, CYP11B2
4aldosterone biosynthetic processGO:03234210.7CYP11B1, CYP11B2
5mineralocorticoid biosynthetic processGO:00670510.6CYP11B2, CYP11B1
6cellular response to potassium ionGO:03586510.6CYP11B2, CYP11B1
7steroid metabolic processGO:00820210.6CYP11B1, CYP11B2, CYP11A1
8progesterone biosynthetic processGO:00670110.5ADM, CYP11A1
9response to salt stressGO:00965110.5TP53, CYP11A1
10cell-cell signalingGO:00726710.5ADM, TSPAN32, INHA, NR5A1
11male gonad developmentGO:00858410.4NR5A1, NR0B1, INHA
12Leydig cell differentiationGO:03332710.2CYP11A1, NR0B1

Molecular functions related to Adrenocortical Carcinoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1heme bindingGO:02003710.5CYP11A1, CYP11B2, CYP11B1
2steroid 11-beta-monooxygenase activityGO:00450710.4CYP11B2, CYP11B1
3protein bindingGO:00551510.1CARS, NPW, NPBWR2, NPB, NR5A1, NR0B1

Products for genes affiliated with Adrenocortical Carcinoma

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  • Antibodies
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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
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet