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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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
Functioning Adrenocortical Carcinoma with Extension upto the Right Atrium Producing Cushing's Syndrome. (24083069)
2013
2
Insulin growth factor receptor (IGF-1R) antibody cixutumumab combined with the mTOR inhibitor temsirolimus in patients with metastatic adrenocortical carcinoma. (23412108)
2013
3
Detection of circulating tumor cells in patients with adrenocortical carcinoma: a monocentric preliminary study. (23836937)
2013
4
Diagnosis of adrenocortical carcinoma via endosonography-assisted fine-needle aspiration of inferior vena cava thrombosis: first case in the literature. (23363322)
2013
5
TOP2A is overexpressed and is a therapeutic target for adrenocortical carcinoma. (23533247)
2013
6
Adrenocortical carcinoma: effect of hospital volume on patient outcome. (22069043)
2012
7
Sunitinib in refractory adrenocortical carcinoma: a phase II, single-arm, open-label trial. (22837187)
2012
8
Surgery in adrenocortical carcinoma: Importance of national cooperation and centralized surgery. (22703895)
2012
9
Imaging findings of pelvic tumor thrombosis extending from sacral bone metastasis of adrenocortical carcinoma. (23326744)
2012
10
Childhood adrenocortical carcinoma: Case report and review. (22629514)
2012
11
Adrenocortical Carcinoma: Current Therapeutic State-of-the-Art. (23125857)
2012
12
Virilizing adrenocortical carcinoma in a child: A rare enigma. (22837928)
2012
13
Human adrenocortical carcinoma cell lines. (21924324)
2012
14
A 12-year-old chinese girl with Cushing syndrome and virilization due to adrenocortical carcinoma. (21648291)
2011
15
Pregnancy in a patient with adrenocortical carcinoma during treatment with Mitotane - a case report. (21528483)
2011
16
Deficits in the management of patients with adrenocortical carcinoma in Germany. Statements cannot be substantiated. (21637638)
2011
17
Chemopreventive actions by enterolactone and 13 VIOXX-related lactone derivatives in H295R human adrenocortical carcinoma cells. (19913079)
2010
18
Response to radiation therapy in adrenocortical carcinoma. (20220294)
2010
19
Cancer: Second-line therapy for adrenocortical carcinoma proves beneficial. (20583342)
2010
20
A case of a testosterone-secreting oncocytic adrenocortical carcinoma. (21317966)
2010
21
Management of adrenocortical carcinoma. (19635227)
2009
22
Limited prognostic value of the 2004 International Union Against Cancer staging classification for adrenocortical carcinoma: proposal for a revised TNM classification. (19827149)
2009
23
Mutational analysis of the BRAF, RAS and EGFR genes in human adrenocortical carcinomas. (19190079)
2009
24
Limited prognostic value of the 2004 International Union Against Cancer staging classification for adrenocortical carcinoma: proposal for a Revised TNM Classification. (19025987)
2009
25
Close examination of steroidogenesis disorders in a DOC- and progesterone-producing adrenocortical carcinoma. (18985457)
2009
26
Comparative effects of prolactin versus ACTH, estradiol, progesterone, testosterone, and dihydrotestosterone on cortisol release and proliferation of the adrenocortical carcinoma cell line H295R. (18484195)
2008
27
Identification of biomarkers of adrenocortical carcinoma using genomewide gene expression profiling. (18794420)
2008
28
Concomitant secretion of glucocorticoid, androgens, and mineralocorticoid by an adrenocortical carcinoma: case report and review of literature. (17669719)
2007
29
Adrenocortical carcinoma presenting as a retroperitoneal abscess: an unusual presentation of a rare tumor. (17872359)
2007
30
Effects of brominated flame retardants and brominated dioxins on steroidogenesis in H295R human adrenocortical carcinoma cell line. (17447562)
2007
31
Effect of insulin-like growth factor and its receptor-I antibody on growth of human adrenocortical carcinoma SW-13 cell lines in vitro]. (17259156)
2007
32
Effects of PCBs and MeSO2-PCBs on adrenocortical steroidogenesis in H295R human adrenocortical carcinoma cells. (16216300)
2006
33
Adrenocortical carcinoma: contrast washout characteristics on CT. (16794135)
2006
34
Adrenocortical carcinoma. (16882496)
2006
35
Emerging treatment strategies for adrenocortical carcinoma: a new hope. (16234301)
2006
36
Shift from Conn's syndrome to Cushing's syndrome in a recurrent adrenocortical carcinoma. (16260420)
2005
37
Three-dimensional sonography in diagnosis of retroperitoneal hemorrhage from adrenocortical carcinoma. (15448327)
2004
38
Radiofrequency ablation of adrenal tumors and adrenocortical carcinoma metastases. (12548596)
2003
39
Adrenocortical carcinoma: a 15-year survival after complete resection and repeated resection. A retrospective study in a patient with an expected poor prognosis. (12926137)
2003
40
Effects of 3-MeSO2-DDE and some CYP inhibitors on glucocorticoid steroidogenesis in the H295R human adrenocortical carcinoma cell line. (11869873)
2002
41
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. (12015757)
2002
42
Special feature: pathological case of the month. Denouement and discussion: congenital adrenocortical carcinoma. (11115315)
2000
43
Recurrence of giant adrenocortical carcinoma in the contralateral adrenal gland 6 years after surgery: report of a case. (10752787)
2000
44
Metastatic congenital adrenocortical carcinoma: a case report with tumor remission at 3 1/2 years. (11095414)
2000
45
Secretion of endothelin-1 and adrenomedullin by SW-13 human adrenocortical carcinoma cells. (11078430)
2000
46
Lack of response to octreotide in Cushing's syndrome due to metastatic adrenocortical carcinoma. (10448470)
1999
47
Case 3: Adrenocortical carcinoma. (9725326)
1998
48
Synchronous testicular seminoma and adrenocortical carcinoma: a case report. (9855136)
1998
49
Uneventful delivery following series of successive treatments for virilized Cushing syndrome due to adrenocortical carcinoma. (2219621)
1990
50
Primary hyperaldosteronism caused by adrenocortical carcinoma. (3751090)
1986

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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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