ACC
MCID: ADR015
MIFTS: 60

Adrenocortical Carcinoma (ACC) malady

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

Summaries for Adrenocortical Carcinoma

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9Disease Ontology, 66Wikipedia, 48OMIM, 34MalaCards
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Wikipedia:66 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 conn'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.

Disease Ontology:9 An adrenal cortex cancer that forms in the outer layer of tissue of the adrenal gland.

Description from OMIM:48 202300

Aliases & Classifications for Adrenocortical Carcinoma

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9Disease Ontology, 10diseasecard, 44NIH Rare Diseases, 48OMIM, 11DISEASES, 46Novoseek, 50Orphanet, 63UMLS, 59SNOMED-CT, 41NCIt, 36MeSH, 37MESH via Orphanet, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet, 26ICD10
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Classifications:



Characteristics (Orphanet epidemiological data):

50
adrenocortical carcinoma:
Inheritance: Sporadic; Prevalence: 1-9/1000000; Age of onset: Childhood


Aliases & Descriptions:

adrenocortical carcinoma 9 44 11 50 63
adrenal cortical carcinoma 9 10 48
carcinoma of the adrenal cortex 9
carcinoma adrenocortical 46
adrenocortical cancer 46
adrenal cortex cancer 11
acc 44


External Ids:

Disease Ontology9 DOID:3948
NCIt41 C9325
MeSH36 D018268
OMIM48 202300
MESH via Orphanet37 D018268
SNOMED-CT59 255035007, 2227007
ICD10 via Orphanet27 C74.0, E24.8
SNOMED-CT via Orphanet60 2227007
UMLS via Orphanet64 C0206686
ICD1026 C74.0

Related Diseases for Adrenocortical Carcinoma

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Sources:
18GeneCards, 19GeneDecks
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Diseases related to Adrenocortical Carcinoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 109)
idRelated DiseaseScoreTop Affiliating Genes
1cushing's syndrome30.8CYP11B2, INHA, NR0B1
2conn's syndrome30.5CYP11B2
3adenoma30.4TP53, INHA, NR0B1, CYP11B2, FSHB, NR5A1
4pheochromocytoma30.4INHA, UTS2, NR5A1
5beckwith-wiedemann syndrome30.3TSPAN32, TSSC4, PHLDA2
6adenocarcinoma30.1NR5A1, TSPAN32, TP53, INHA
7rhabdomyosarcoma30.0TP53, INHA, UTS2
8obesity29.8INHA, NR5A1, NR0B1
9adenoid cystic carcinoma10.5
10adenoiditis10.4
11adrenal carcinoma10.4
12hyperaldosteronism10.4
13renal cell carcinoma10.4
14hypertension10.4
15li-fraumeni syndrome10.3
16adrenocortical carcinoma with pure aldosterone hypersecretion10.3
17peripheral vascular disease10.3
18vascular disease10.3
19corpus callosum agenesis10.3
20central serous chorioretinopathy10.3
21hepatitis10.3
22thyroiditis10.3
23myocardial infarction10.2
24hereditary motor and sensory neuropathy with agenesis of the corpus callosum10.2
25choroiditis10.2
26choroid plexus carcinoma10.2
27hepatocellular carcinoma10.2
28osteosarcoma10.2
29peritonitis10.2
30thyroid cancer10.2
31turner syndrome10.2
32multiple endocrine neoplasia10.2
33hemihypertrophy10.2
34luteoma10.1
35von willebrand's disease10.1
36carney complex10.1
37alpha thalassemia10.1
38thalassemia10.1
39circumscribed cutaneous aplasia of the vertex10.1
40partial agenesis of corpus callosum10.1
41overuse syndrome10.1
4246xy sex reversal 310.1NR5A1
43premature ovarian failure10.1INHA
4446,xy disorder of sex development and 46,xy complete gonadal dysgenesis10.1NR5A1, NR0B1
45x-linked adrenal hypoplasia congenita10.1NR0B1, NR5A1
46gonadal dysgenesis10.1NR5A1, NR0B1
47gestational trophoblastic neoplasm10.0PHLDA2, INHA
48kallmann syndrome10.0NR0B1, NR5A1
49sex cord-gonadal stromal tumor10.0NR5A1, INHA
50abrikosov's tumor10.0INHA, NR5A1

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



Diseases related to adrenocortical carcinoma

Symptoms for Adrenocortical Carcinoma

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Sources:
48OMIM
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Symptoms by clinical synopsis from OMIM:

202300

Clinical features from OMIM:

202300

Drugs & Therapeutics for Adrenocortical Carcinoma

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-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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34MalaCards
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MalaCards organs/tissues related to Adrenocortical Carcinoma:

34
Cortex, Adrenal cortex, Adrenal gland, Liver, Thyroid, Kidney, Lung, Bone, Ovary, T cells, Endothelial

Animal Models for Adrenocortical Carcinoma or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Adrenocortical Carcinoma:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053858.5TBX2, NR5A1, TP53, CYP11B2, INHA
2MP:00053898.1NR0B1, INHA, NR5A1, TP53, FSHB
3MP:00053787.9INHA, TP53, NR5A1, TBX2, PHLDA2, CYP11B2
4MP:00053797.9FSHB, CYP11B2, INHA, NR0B1, NR5A1, TP53
5MP:00053767.5TBX2, NR0B1, CYP11B2, NR5A1, FSHB, TP53

Publications for Adrenocortical Carcinoma

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Sources:
53PubMed
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Articles related to Adrenocortical Carcinoma:

(show top 50)    (show all 519)
idTitleAuthorsYear
1
Molecular markers and targeted therapies for adrenocortical carcinoma. (24304415)
2014
2
Myxoid variant of adrenocortical carcinoma: a report of two illustrative cases and a brief review of the literature. (24300732)
2014
3
Comparative diagnostic and prognostic performances of the hematoxylin-eosin and phospho-histone H3 mitotic count and Ki-67 index in adrenocortical carcinoma. (24434900)
2014
4
Functioning Adrenocortical Carcinoma with Extension upto the Right Atrium Producing Cushing's Syndrome. (24083069)
2013
5
Adjuvant Therapies, Patient and Tumor Characteristics Associated with Survival of Adult Patients with Adrenocortical Carcinoma. (24302750)
2013
6
Mini-invasive surgery for adrenocortical carcinoma in children: is it safe? (23444873)
2013
7
Primary rhabdoid adrenocortical carcinoma: a clinicopathological and immunohistochemical study of three cases. (23445527)
2013
8
Prevalence of germline TP53 mutations in a prospective series of unselected patients with adrenocortical carcinoma. (23175693)
2013
9
Serum inhibin pro-I+C is a tumor marker for adrenocortical carcinomas. (22127493)
2012
10
Childhood adrenocortical carcinoma as a sentinel cancer for detecting families with germline TP53 mutations. (22233476)
2012
11
Liposomal doxorubicin-based treatment in a preclinical model of adrenocortical carcinoma. (22407999)
2012
12
Adrenocortical carcinoma: effect of hospital volume on patient outcome. (22069043)
2012
13
Sunitinib in refractory adrenocortical carcinoma: a phase II, single-arm, open-label trial. (22837187)
2012
14
Adrenocortical carcinoma: past, present, and future. (22473597)
2012
15
Adrenocortical stem and progenitor cells: implications for adrenocortical carcinoma. (22266195)
2012
16
Surgery in adrenocortical carcinoma: Importance of national cooperation and centralized surgery. (22703895)
2012
17
A new mutation in the menin gene causes the multiple endocrine neoplasia type 1 syndrome with adrenocortical carcinoma. (21069576)
2011
18
Metronomic therapy concepts in the management of adrenocortical carcinoma. (21971765)
2011
19
Feminizing adrenocortical carcinoma with selective suppression of follicle-stimulating hormone secretion and disorganized steroidogenesis: a case report and literature review. (21720063)
2011
20
Significance of heparanase-1 and vascular endothelial growth factor in adrenocortical carcinoma angiogenesis: potential for therapy. (21706269)
2011
21
Rare pediatric adrenocortical carcinoma with oncocytic change: a cytologic dilemma. (21181300)
2011
22
Adrenocortical carcinoma presenting with a peritoneal effusion. (19941369)
2010
23
Fine needle aspiration cytology of adrenocortical carcinoma--case report. (20698150)
2010
24
Adrenocortical carcinoma and synchronous malignancies. (20842232)
2010
25
Diagnosis: Novel molecular signatures for adrenocortical carcinoma. (19465894)
2009
26
Ectopic low-grade adrenocortical carcinoma in the spinal region: immunohistochemical and molecular cytogenetic study of a pediatric case. (18941403)
2009
27
Management of adrenocortical carcinoma. (19635227)
2009
28
Clinical management of adrenocortical carcinoma. (19500769)
2009
29
Percutaneous laser ablation of unresectable primary and metastatic adrenocortical carcinoma. (17498906)
2008
30
Bilateral primary adrenal non-Hodgkin's lymphoma and primary adrenocortical carcinoma--review of the literature preoperative differentiation of adrenal tumors. (18490838)
2008
31
Side population does not define stem cell-like cancer cells in the adrenocortical carcinoma cell line NCI h295R. (18063677)
2008
32
Alpha-fetoprotein (AFP)-producing adrenocortical carcinoma--long survival with various therapeutic strategies including a lung resection: report of a case. (18307006)
2008
33
Multiple malignant and benign lesions in the liver in a child with adrenocortical carcinoma. (18256815)
2008
34
Adjuvant mitotane in adrenocortical carcinoma. (17891836)
2007
35
Efficacy of adjuvant radiotherapy of the tumor bed on local recurrence of adrenocortical carcinoma. (16895957)
2006
36
Adrenocortical carcinoma extending into the inferior vena cava: presentation of a 15-patient series and review of the literature. (16364713)
2006
37
Etoposide, doxorubicin and cisplatin plus mitotane in the treatment of advanced adrenocortical carcinoma: a large prospective phase II trial. (16172198)
2005
38
Ruptured adrenocortical carcinoma as a cause of paediatric acute abdomen. (15711359)
2005
39
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
40
A case of postpartum hypopituitarism accompanied by Cushing's syndrome as a result of an adrenocortical carcinoma. (15863951)
2005
41
Management of adrenocortical carcinoma. (15008991)
2004
42
Pregnancy with functioning adrenocortical carcinoma. (12126365)
2002
43
Adrenocortical carcinoma: clinical, morphologic, and molecular characterization. (11844815)
2002
44
Virilising adrenocortical carcinoma. (12405338)
2002
45
Selective testosterone secreting adrenocortical carcinoma in an infant. (11237246)
2001
46
Special feature: pathological case of the month. Denouement and discussion: congenital adrenocortical carcinoma. (11115315)
2000
47
Incidentally found adrenocortical carcinoma. A study of 21 patients. (9893659)
1998
48
Simultaneous adrenocortical carcinoma and ganglioneuroblastoma in a child with Turner syndrome and germline p53 mutation. (9598730)
1998
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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Expression for genes affiliated with Adrenocortical Carcinoma

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Sources:
2BioGPS, 16Gene 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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Sources:
51PathCards, 54QIAGEN, 56Reactome, 39NCBI BioSystems Database
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Pathways related to Adrenocortical Carcinoma according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8TBX2, TP53
2
Show member pathways
Nuclear Receptors39
9.6NR0B1, NR5A1
39.5NR5A1, INHA
4
Show member pathways
9.3FSHB, INHA

Compounds for genes affiliated with Adrenocortical Carcinoma

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Sources:
46Novoseek, 62Tocris Bioscience, 12DrugBank, 52PharmGKB, 25HMDB, 30IUPHAR
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Compounds related to Adrenocortical Carcinoma according to GeneCards/GeneDecks:

(show all 25)
idCompoundScoreTop Affiliating Genes
117beta-hydroxysteroid469.9CYP11B2, NR5A1
2dmrt469.9NR5A1, NR0B1
3dhea469.8NR5A1, CYP11B2
4ptx1469.7FSHB, NR5A1
5radicicol46 62 1211.6TP53, UTS2
6proline469.5TP53, NR5A1, CYP11B2
73beta-hydroxysteroid469.5NR5A1, CYP11B2, INHA
8actinomycin d469.4FSHB, NR5A1, TP53
9forskolin46 52 1211.3INHA, NR5A1, CYP11B2
10leucine469.2CYP11B2, NR0B1, NR5A1
11estradiol46 25 1211.1INHA, FSHB, NR5A1
12cyclic amp46 2510.0CYP11B2, NR0B1, NR5A1, INHA
13thymidine46 2510.0TP53, UTS2, NR5A1
14ribonucleic acid468.9NR5A1, FSHB, CYP11B2, INHA
15gnrh468.8FSHB, INHA, NR0B1, NR5A1
16sodium46 259.8CYP11B2, UTS2, NR5A1
17oligonucleotide468.6CYP11B2, NR5A1, FSHB, TP53, INHA
18estrogen468.5NR0B1, NR5A1, FSHB, TP53, INHA
19acth468.5NR5A1, NR0B1, FSHB, INHA, CYP11B2
20testosterone46 62 25 1211.4NR0B1, NR5A1, CYP11B2, FSHB, INHA
21steroid468.4CYP11B2, INHA, FSHB, NR5A1, NR0B1
22cysteine468.3UTS2, NR0B1, TSPAN32, FSHB
23cholesterol46 30 25 1211.2CYP11B2, UTS2, NR5A1, NR0B1, TSPAN32
24progesterone46 30 62 25 1212.2NR5A1, TP53, INHA, CYP11B2, NR0B1, FSHB
25lipid468.0UTS2, CYP11B2, TSPAN32, NR5A1

GO Terms for genes affiliated with Adrenocortical Carcinoma

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17Gene Ontology
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Biological processes related to Adrenocortical Carcinoma according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1cell agingGO:00756910.0TP53, TBX2
2adrenal gland developmentGO:0303259.8NR5A1, NR0B1
3protein localizationGO:0081049.6NR0B1, TP53
4intracellular receptor signaling pathwayGO:0305229.6NR5A1, NR0B1
5negative regulation of transcription, DNA-templatedGO:0458929.4TBX2, NR0B1, TP53
6male gonad developmentGO:0085849.3NR0B1, INHA, NR5A1
7cell-cell signalingGO:0072679.1INHA, TSPAN32, NR5A1
8cell differentiationGO:0301549.0INHA, TP53, NR5A1

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

idNameGO IDScoreTop Affiliating Genes
1steroid hormone receptor activityGO:0037079.6NR0B1, NR5A1
2ligand-activated sequence-specific DNA binding RNA polymerase II transcription factor activityGO:0048799.3NR0B1, NR5A1
3hormone activityGO:0051798.4FSHB, UTS2, INHA

Products for genes affiliated with Adrenocortical Carcinoma

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  • Antibodies
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Sources for Adrenocortical Carcinoma

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet