ACC
MCID: ADR015
MIFTS: 57

Adrenocortical Carcinoma (ACC) malady

Cancer, Cardiovascular, Nephrological, Endocrine categories

Summaries for Adrenocortical Carcinoma

Sources:
8Disease Ontology, 64Wikipedia, 47OMIM, 33MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
Wikipedia:64 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, colon and kidney, 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:47 202300

Aliases & Classifications for Adrenocortical Carcinoma

Sources:
8Disease Ontology, 43NIH Rare Diseases, 10DISEASES, 49Orphanet, 61UMLS, 9diseasecard, 47OMIM, 45Novoseek, 40NCIt, 35MeSH, 57SNOMED-CT, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet, 25ICD10
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer
Anatomical: Cardiovascular, Nephrological, Endocrine


Characteristics (Orphanet epidemiological data):

49
adrenocortical carcinoma:
Inheritance: Sporadic; Prevalence: 1-9/1000000; Age of onset: Childhood


Aliases & Descriptions:

adrenocortical carcinoma 8 43 10 49 61
adrenal cortical carcinoma 8 9 47
carcinoma of the adrenal cortex 8
carcinoma adrenocortical 45
adrenocortical cancer 45
adrenal cortex cancer 10
acc 43


External Ids:

Disease Ontology8 DOID:3948
NCIt40 C9325
MeSH35 D018268
OMIM47 202300
MESH via Orphanet36 D018268
SNOMED-CT57 2227007, 255035007
ICD10 via Orphanet26 C74.0, E24.8
SNOMED-CT via Orphanet58 2227007
UMLS via Orphanet62 C0206686
ICD1025 C74.0

Related Diseases for Adrenocortical Carcinoma

Sources:
17GeneCards, 18GeneDecks
See all sources

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

(show top 50)    (show all 103)
idRelated DiseaseScoreTop Affiliating Genes
1cushing's syndrome30.9NR0B1, CYP11B1, CYP11B2, CYP11A1, INHA
2adenoma30.7NR5A1, NR0B1, CYP11B1, CYP11B2, TP53, CYP11A1
3pheochromocytoma30.4ADM, INHA, NR5A1
4conn's syndrome30.3CYP11B2, CYP11B1
5beckwith-wiedemann syndrome30.2TSPAN32, TSSC4, NAP1L4, SLC22A18, PHLDA2
6adenocarcinoma30.2NR5A1, INHA, TSPAN32, ADM, TP53
7adrenal adenoma30.0CYP11B2, ADM, CYP11A1, CYP11B1
8adrenal carcinoma10.8
9adenoid cystic carcinoma10.4
10adrenocortical carcinoma with pure aldosterone hypersecretion10.3
11corpus callosum agenesis10.3
12central serous chorioretinopathy10.2
13multiple endocrine neoplasia10.2
14peripheral vascular disease10.2
15vascular disease10.2
16bone carcinoma10.2
17neuroendocrine carcinoma10.2
18papillary carcinoma10.2
19choroid plexus carcinoma10.2
20li-fraumeni syndrome10.2
21thyroid cancer10.2
22turner syndrome10.2
23hemihypertrophy10.2
24peritoneal carcinoma10.2
25carney complex10.1
26luteoma10.1
27adrenal cortical adenocarcinoma10.1
28pancreatic acinar cell adenocarcinoma10.1
29alpha thalassemia10.1
30cutaneous adenocystic carcinoma10.1
31lung adenoid cystic carcinoma10.1
32adenoiditis10.1
33thalassemia10.1
34tracheal cancer10.1
35colorectal cancer10.1
36hereditary motor and sensory neuropathy with agenesis of the corpus callosum10.1
37circumscribed cutaneous aplasia of the vertex10.1
38hypoxia10.1
39overuse syndrome10.1
40cholera10.0
41familial adenomatous polyposis10.0
42multicentric papillary thyroid carcinoma10.0
43familial retinoblastoma10.0
44diphtheria10.0
45hilar lung carcinoma10.0
46hypoglycemia10.0
47paracoccidioidomycosis10.0
48hypokalemia10.0
49thyroid medullary carcinoma10.0
50peutz-jeghers syndrome10.0

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



Diseases related to adrenocortical carcinoma

Clinical Features for Adrenocortical Carcinoma

Sources:
47OMIM
See all sources

Clinical features from OMIM:

202300

Clinical synopsis from OMIM:

202300

Drugs & Therapeutics for Adrenocortical Carcinoma

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

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

Anatomical Context for Adrenocortical Carcinoma

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Adrenocortical Carcinoma:

33
Cortex, Colon, Kidney, Liver, Lung, Thyroid, Adrenal gland, Breast, Ovary, Prostate, T cells, B cells, Endothelial, Adrenal cortex, Pituitary

Animal Models for Adrenocortical Carcinoma or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Adrenocortical Carcinoma:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000537810.5NPB, PHLDA2, NR5A1, TBX2, NUP98, CYP11B2
2MP:000537910.2NR5A1, NR0B1, CYP11B1, CYP11B2, CYP11A1, INHA

Publications for Adrenocortical Carcinoma

Sources:
51PubMed
See all sources

Articles related to Adrenocortical Carcinoma:

(show top 50)    (show all 549)
idTitleAuthorsYear
1
Molecular markers and targeted therapies for adrenocortical carcinoma. (24304415)
2014
2
Prevalence of germline TP53 mutations in a prospective series of unselected patients with adrenocortical carcinoma. (23175693)
2013
3
CpG island methylator phenotype in adrenocortical carcinoma: fact or fiction? (23293338)
2013
4
Mitotane levels predict the outcome of patients with adrenocortical carcinoma treated adjuvantly following radical resection. (23704714)
2013
5
Adrenocortical carcinoma initially presenting with hypokalemia and hypertension mimicking hyperaldosteronism: a case report. (24103295)
2013
6
Update on the management of unusual neuroendocrine tumors: pheochromocytoma and paraganglioma, medullary thyroid cancer and adrenocortical carcinoma. (23391119)
2013
7
Upregulated JAG1 enhances cell proliferation in adrenocortical carcinoma. (22427350)
2012
8
TP53 germline mutations in adult patients with adrenocortical carcinoma. (22170717)
2012
9
Phase II study of weekly paclitaxel and sorafenib as second/third-line therapy in patients with adrenocortical carcinoma. (22189997)
2012
10
Knockdown of SF-1 and RNF31 affects components of steroidogenesis, TGFI^, and Wnt/I^-catenin signaling in adrenocortical carcinoma cells. (22427816)
2012
11
Adrenocortical carcinoma extending into the inferior vena cava in a patient with right kidney agenesis: Surgical approach and review of literature. (22543230)
2012
12
Interleukin-13 receptor alpha2 is a novel therapeutic target for human adrenocortical carcinoma. (22570059)
2012
13
Mitotane treatment for adrenocortical carcinoma: an overview. (22382612)
2012
14
A new mutation in the menin gene causes the multiple endocrine neoplasia type 1 syndrome with adrenocortical carcinoma. (21069576)
2011
15
Feminizing adrenocortical carcinoma with selective suppression of follicle-stimulating hormone secretion and disorganized steroidogenesis: a case report and literature review. (21720063)
2011
16
Adrenocortical carcinoma: Report of two cases. (21897902)
2011
17
Toward a pathway-centered approach for the treatment of adrenocortical carcinoma. (21045685)
2011
18
Adrenocortical carcinoma and synchronous malignancies. (20842232)
2010
19
Adjuvant therapy in patients with adrenocortical carcinoma: a position of an international panel. (20567001)
2010
20
Virilizing adrenocortical carcinoma in a child with Turner syndrome and somatic TP53 gene mutation. (19701813)
2010
21
Adrenocortical carcinoma and pregnancy: clinical and biological features and prognosis. (20699382)
2010
22
Laparoscopic versus open adrenalectomy for adrenocortical carcinoma: surgical and oncologic outcome in 152 patients. (20580485)
2010
23
The long-term survival in adrenocortical carcinoma with active surgical management and use of monitored mitotane. (20026647)
2010
24
Multimodality imaging in a patient with adrenocortical carcinoma leading to peptide receptor radionuclide therapy. (19617743)
2009
25
Metastatic virilizing adrenocortical carcinoma: a rare case of cure with surgery and mitotane therapy. (19574489)
2009
26
Estrogen biosynthesis in human H295 adrenocortical carcinoma cells. (19026713)
2009
27
Preclinical targeting of the type I insulin-like growth factor receptor in adrenocortical carcinoma. (18854392)
2009
28
Cytotoxicity and gene expression profiling of two hydroxylated polybrominated diphenyl ethers in human H295R adrenocortical carcinoma cells. (19095052)
2009
29
Radiotherapy in adrenocortical carcinoma. (19402169)
2009
30
Bilateral primary adrenal non-Hodgkin's lymphoma and primary adrenocortical carcinoma--review of the literature preoperative differentiation of adrenal tumors. (18490838)
2008
31
Identification of a novel TP53 germline mutation E285V in a rare case of paediatric adrenocortical carcinoma and choroid plexus carcinoma. (18762572)
2008
32
Mebendazole inhibits growth of human adrenocortical carcinoma cell lines implanted in nude mice. (17581752)
2008
33
Usefulness of transcription factors Ad4BP/SF-1 and DAX-1 as immunohistologic markers for diagnosis of advanced adrenocortical carcinoma. (18824868)
2008
34
Adrenocortical carcinoma: role of laparoscopic surgery in treatment. (17892430)
2007
35
Alternative lengthening of telomeres in the human adrenocortical carcinoma cell line H295R. (16820888)
2006
36
Diagnostic and prognostic value of 18-fluorodeoxyglucose positron emission tomography in adrenocortical carcinoma: a prospective comparison with computed tomography. (16368753)
2006
37
Virilizing adrenocortical carcinoma with cavoatrial extension. (16860633)
2006
38
Etoposide, doxorubicin and cisplatin plus mitotane in the treatment of advanced adrenocortical carcinoma: a large prospective phase II trial. (16172198)
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
Myxoid adrenal cortical carcinoma--a rare variant of adrenocortical carcinoma. (16340152)
2005
41
Adrenocortical carcinoma with fat inclusion: case report. (15688105)
2005
42
Extension of adrenocortical carcinoma into the right atrium--echocardiographic diagnosis: a case report. (12777180)
2003
43
Fine needle aspiration cytology of metastatic hepatic adrenocortical carcinoma mimicking hepatocellular carcinoma: a case report. (11575658)
2001
44
2-Chloro-s-triazine herbicides induce aromatase (CYP19) activity in H295R human adrenocortical carcinoma cells: a novel mechanism for estrogenicity? (10746939)
2000
45
Anti-alpha-inhibin: marker of choice for the consistent distinction between adrenocortical carcinoma and renal cell carcinoma in fine-needle aspiration. (10385449)
1999
46
Is there a role for low doses of mitotane (o,p'-DDD) as adjuvant therapy in adrenocortical carcinoma? (9745410)
1998
47
Elevated levels of p53 protein in adrenocortical carcinomas from Costa Rica. (7695963)
1995
48
Insulin-like growth factors (IGFs) and their receptors in adrenal tumors: high IGF-II expression in functional adrenocortical carcinomas. (8370710)
1993
49
Neuroendocrine differentiation in adrenocortical carcinoma. New immunohistochemical findings supported by electron microscopy. (1735954)
1992
50
Molecular definition of the 11p15.5 region involved in Beckwith-Wiedemann syndrome and probably in predisposition to adrenocortical carcinoma. (2921038)
1989

Genetic Variations for Adrenocortical Carcinoma

Expression for genes affiliated with Adrenocortical Carcinoma

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
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

Sources:
52QIAGEN, 38NCBI BioSystems Database, 30KEGG, 54Reactome, 12EMD Millipore
See all sources

Compounds for genes affiliated with Adrenocortical Carcinoma

Sources:
45Novoseek, 60Tocris Bioscience, 29IUPHAR, 11DrugBank, 24HMDB, 50PharmGKB, 2BitterDB
See all sources

Compounds related to Adrenocortical Carcinoma according to GeneCards/GeneDecks:

(show all 39)
idCompoundScoreTop Affiliating Genes
1acth4510.9NR0B1, CYP11B1, NR5A1, CYP11A1, INHA, ADM
2progesterone45 60 29 11 2414.9ADM, INHA, CYP11A1, CYP11B2, CYP11B1, NR0B1
33beta-hydroxysteroid4510.9CYP11B1, CYP11A1, NR5A1, INHA, CYP11B2
4cyclic amp45 2411.9INHA, NR5A1, ADM, CYP11A1, CYP11B2, CYP11B1
5corticosterone45 60 2412.9ADM, CYP11A1, NPW, NR5A1, CYP11B1, CYP11B2
6testosterone45 60 11 2413.9NR0B1, CYP11B1, CYP11B2, CYP11A1, INHA, ADM
7cholesterol45 29 11 2413.9ADM, NR5A1, NR0B1, CYP11B1, TSPAN32, CYP11B2
8doca4510.9CYP11A1, CYP11B2, CYP11B1
9steroid4510.9CYP11A1, NR0B1, NR5A1, CYP11B1, INHA, CYP11B2
10forskolin45 50 1112.9CYP11B2, CYP11B1, ADM
11oligonucleotide4510.9INHA, NR5A1, TP53, ADM, CYP11B2, CYP11B1
12aldosterone29 11 2412.9CYP11B1, CYP11B2, ADM
13androstenedione45 2411.9INHA, CYP11A1, CYP11B2, CYP11B1
14ribonucleic acid4510.8INHA, CYP11B1, NR5A1, CYP11A1, CYP11B2
1518-hydroxydeoxycorticosterone4510.8CYP11B2, CYP11B1
1618-oxocortisol4510.8CYP11B2, CYP11B1
1718-hydroxycortisol4510.8CYP11B1, CYP11B2
1817-hydroxyprogesterone45 2411.8CYP11B2, CYP11A1, CYP11B1
1911beta-hydroxysteroid4510.8CYP11B1, CYP11B2, CYP11A1
2021-deoxycortisol45 2411.8CYP11B2, CYP11B1
2118-hydroxycorticosterone45 2411.8CYP11B1, CYP11B2
2217beta-hydroxysteroid4510.8CYP11A1, NR5A1, CYP11B2
23dmrt4510.8NR5A1, NR0B1
24gnrh4510.8INHA, CYP11A1, NR0B1, NR5A1
25deoxycorticosterone29 2411.8CYP11B2, CYP11B1
26pcb 1264510.8CYP11B1, CYP11B2
27dhea4510.7CYP11A1, CYP11B2, NR5A1
28etomidate45 1111.7CYP11B2, CYP11B1
29cortisol29 2411.7CYP11B1, CYP11B2
30dbc-amp4510.7CYP11B2, ADM, CYP11A1
31estrogen4510.7ADM, CYP11A1, NR0B1, NR5A1, TP53, INHA
3211 deoxycortisol4510.6CYP11B2, CYP11B1
3322r-hydroxycholesterol45 2911.6NR5A1, CYP11A1
34pcbs4510.6CYP11B2, CYP11B1
35actinomycin d4510.6ADM, NR5A1, TP53, CYP11A1
36estradiol45 11 2412.5CYP11A1, INHA, CYP11B1, NR5A1
37hydrocortisone45 2 60 1113.5ADM, CYP11B2, CYP11B1
38dexamethasone45 50 29 1113.4ADM, CYP11B1, CYP11A1, CYP11B2, NR0B1
39potassium45 11 2412.2CYP11B2, ADM, NR5A1, CYP11B1

GO Terms for genes affiliated with Adrenocortical Carcinoma

Sources:
16Gene Ontology
See all sources

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
3aldosterone biosynthetic processGO:03234210.7CYP11B2, CYP11B1
4cortisol biosynthetic processGO:03465110.7CYP11B2, CYP11B1
5cellular response to potassium ionGO:03586510.6CYP11B2, CYP11B1
6mineralocorticoid biosynthetic processGO:00670510.6CYP11B2, CYP11B1
7steroid metabolic processGO:00820210.6CYP11B2, CYP11B1, CYP11A1
8progesterone biosynthetic processGO:00670110.5ADM, CYP11A1
9response to salt stressGO:00965110.5CYP11A1, TP53
10cell-cell signalingGO:00726710.5ADM, TSPAN32, INHA, NR5A1
11male gonad developmentGO:00858410.4NR5A1, INHA, NR0B1
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

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Adrenocortical Carcinoma

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet