ACC
MCID: ADR015
MIFTS: 61

Adrenocortical Carcinoma (ACC) malady

Rare diseases, Cancer diseases, Cardiovascular diseases, Nephrological diseases, Reproductive diseases, Endocrine diseases categories
Download this MalaCard

Summaries for Adrenocortical Carcinoma

About this section


Fully expand this MalaCard
Disease Ontology:8 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 adrenal cortical carcinoma, is related to conn's syndrome and cushing's syndrome, and has symptoms including 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:65 Adrenocortical carcinoma, also adrenal cortical carcinoma (ACC) and adrenal cortex cancer, is an... more...

Description from OMIM:46 202300

Aliases & Classifications for Adrenocortical Carcinoma

About this section
Sources:
8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 48Orphanet, 62UMLS, 9diseasecard, 46OMIM, 44Novoseek, 34MeSH, 57SNOMED-CT, 39NCIt, 35MESH via Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet, 25ICD10
See all sources

Adrenocortical Carcinoma, Aliases & Descriptions:

Name: Adrenocortical Carcinoma 8 42 10 48 62
Adrenal Cortical Carcinoma 8 9 46
Carcinoma of the Adrenal Cortex 8 62
Agenesis of Corpus Callosum 62
Carcinoma, Adrenal Cortical 62
 
Carcinoma Adrenocortical 44
Adrenocortical Cancer 44
Adrenal Cortex Cancer 10
Acc 42


Classifications:



Characteristics (Orphanet epidemiological data):

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


External Ids:

Disease Ontology8 DOID:3948
MeSH34 D018268
NCIt39 C9325
OMIM46 202300
SNOMED-CT57 255035007, 2227007
MESH via Orphanet35 D018268
ICD10 via Orphanet26 C74.0
UMLS via Orphanet63 C0206686
ICD1025 C74.0

Related Diseases for Adrenocortical Carcinoma

About this section

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

(show top 50)    (show all 116)
idRelated DiseaseScoreTop Affiliating Genes
1conn's syndrome31.1CYP11B2
2cushing's syndrome30.9CYP11B2, INHA, NR0B1
3beckwith-wiedemann syndrome30.6TSPAN32, TSSC4, PHLDA2
4adrenal cortical adenoma30.3NR0B1, NR5A1, INHA
5pheochromocytoma30.3INHA, UTS2, NR5A1
6adenocarcinoma29.9NR5A1, TSPAN32, TP53, INHA
7rhabdomyosarcoma29.8TP53, INHA, UTS2
8adenoma29.8TP53, INHA, NR0B1, CYP11B2, FSHB, NR5A1
9obesity29.6INHA, NR5A1, NR0B1
10renal cell carcinoma10.5
1146xy sex reversal 310.4NR5A1
12adenoiditis10.4
13adrenal carcinoma10.4
14hyperaldosteronism10.4
15hypertension10.4
16premature ovarian failure10.3INHA
17li-fraumeni syndrome10.3
18thyroiditis10.3
19adrenocortical carcinoma with pure aldosterone hypersecretion10.3
20agenesis of the corpus callosum10.3
21peripheral vascular disease10.3
22vascular disease10.3
23aplasia cutis congenita10.3
24central serous chorioretinopathy10.3
25hepatitis10.3
2646,xy disorder of sex development and 46,xy complete gonadal dysgenesis10.3NR5A1, NR0B1
27x-linked adrenal hypoplasia congenita10.2NR0B1, NR5A1
28gonadal dysgenesis10.2NR5A1, NR0B1
29gestational trophoblastic neoplasm10.2PHLDA2, INHA
30myocardial infarction10.2
31hereditary motor and sensory neuropathy with agenesis of the corpus callosum10.2
32kallmann syndrome10.2NR0B1, NR5A1
33sex cord-gonadal stromal tumor10.2NR5A1, INHA
34hepatocellular carcinoma10.2
35choroid plexus carcinoma10.2
36choroiditis10.2
37osteosarcoma10.2
38peritonitis10.2
39thyroid cancer10.2
40turner syndrome10.2
41multiple endocrine neoplasia10.2
42hemihypertrophy10.2
43abrikosov's tumor10.2INHA, NR5A1
44nephroblastoma10.2TP53, NR5A1
45luteoma10.2
46von willebrand's disease10.2
47carney complex10.2
48angiosarcoma10.2
49adrenoleukodystrophy10.1NR0B1, NR5A1
50thalassemia10.1

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



Diseases related to adrenocortical carcinoma

Symptoms for Adrenocortical Carcinoma

About this section

Symptoms by clinical synopsis from OMIM:

202300

Clinical features from OMIM:

202300

HPO human phenotypes related to Adrenocortical Carcinoma:

id Description Frequency HPO Source Accession
1 autosomal recessive inheritance HP:0000007
2 adrenocortical carcinoma HP:0006744

Drugs & Therapeutics for Adrenocortical Carcinoma

About this section

Drug clinical trials:

Search ClinicalTrials for Adrenocortical Carcinoma

Search NIH Clinical Center for Adrenocortical Carcinoma

Genetic Tests for Adrenocortical Carcinoma

About this section

Anatomical Context for Adrenocortical Carcinoma

About this section

MalaCards organs/tissues related to Adrenocortical Carcinoma:

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

Animal Models for Adrenocortical Carcinoma or affiliated genes

About this section

MGI Mouse Phenotypes related to Adrenocortical Carcinoma:

36
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

About this section

Articles related to Adrenocortical Carcinoma:

(show top 50)    (show all 542)
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
Treatment patterns and outcomes for patients with adrenocortical carcinoma associated with hospital case volume in the United States. (25069860)
2014
4
Comparative diagnostic and prognostic performances of the hematoxylin-eosin and phospho-histone H3 mitotic count and Ki-67 index in adrenocortical carcinoma. (24434900)
2014
5
CYP2W1 is highly expressed in adrenal glands and is positively associated with the response to mitotane in adrenocortical carcinoma. (25144458)
2014
6
Giant nonfunctioning adrenocortical carcinoma: a case report and review of the literature. (25361816)
2014
7
Functioning Adrenocortical Carcinoma with Extension upto the Right Atrium Producing Cushing's Syndrome. (24083069)
2013
8
Adjuvant Therapies, Patient and Tumor Characteristics Associated with Survival of Adult Patients with Adrenocortical Carcinoma. (24302750)
2013
9
A rare coexistence of non-functional adrenocortical carcinoma and multicentric papillary thyroid microcarcinoma: a case report. (23889916)
2013
10
Mini-invasive surgery for adrenocortical carcinoma in children: is it safe? (23444873)
2013
11
Primary rhabdoid adrenocortical carcinoma: a clinicopathological and immunohistochemical study of three cases. (23445527)
2013
12
Serum inhibin pro-I+C is a tumor marker for adrenocortical carcinomas. (22127493)
2012
13
Childhood adrenocortical carcinoma as a sentinel cancer for detecting families with germline TP53 mutations. (22233476)
2012
14
Liposomal doxorubicin-based treatment in a preclinical model of adrenocortical carcinoma. (22407999)
2012
15
Adrenocortical carcinoma: effect of hospital volume on patient outcome. (22069043)
2012
16
Sunitinib in refractory adrenocortical carcinoma: a phase II, single-arm, open-label trial. (22837187)
2012
17
Adrenocortical carcinoma: past, present, and future. (22473597)
2012
18
Adrenocortical stem and progenitor cells: implications for adrenocortical carcinoma. (22266195)
2012
19
Surgery in adrenocortical carcinoma: Importance of national cooperation and centralized surgery. (22703895)
2012
20
A new mutation in the menin gene causes the multiple endocrine neoplasia type 1 syndrome with adrenocortical carcinoma. (21069576)
2011
21
Metronomic therapy concepts in the management of adrenocortical carcinoma. (21971765)
2011
22
Feminizing adrenocortical carcinoma with selective suppression of follicle-stimulating hormone secretion and disorganized steroidogenesis: a case report and literature review. (21720063)
2011
23
Significance of heparanase-1 and vascular endothelial growth factor in adrenocortical carcinoma angiogenesis: potential for therapy. (21706269)
2011
24
Rare pediatric adrenocortical carcinoma with oncocytic change: a cytologic dilemma. (21181300)
2011
25
Adrenocortical carcinoma presenting with a peritoneal effusion. (19941369)
2010
26
Fine needle aspiration cytology of adrenocortical carcinoma--case report. (20698150)
2010
27
Diagnosis: Novel molecular signatures for adrenocortical carcinoma. (19465894)
2009
28
Ectopic low-grade adrenocortical carcinoma in the spinal region: immunohistochemical and molecular cytogenetic study of a pediatric case. (18941403)
2009
29
Management of adrenocortical carcinoma. (19635227)
2009
30
Clinical management of adrenocortical carcinoma. (19500769)
2009
31
Percutaneous laser ablation of unresectable primary and metastatic adrenocortical carcinoma. (17498906)
2008
32
Bilateral primary adrenal non-Hodgkin's lymphoma and primary adrenocortical carcinoma--review of the literature preoperative differentiation of adrenal tumors. (18490838)
2008
33
Side population does not define stem cell-like cancer cells in the adrenocortical carcinoma cell line NCI h295R. (18063677)
2008
34
Alpha-fetoprotein (AFP)-producing adrenocortical carcinoma--long survival with various therapeutic strategies including a lung resection: report of a case. (18307006)
2008
35
Multiple malignant and benign lesions in the liver in a child with adrenocortical carcinoma. (18256815)
2008
36
Adjuvant mitotane in adrenocortical carcinoma. (17891836)
2007
37
Efficacy of adjuvant radiotherapy of the tumor bed on local recurrence of adrenocortical carcinoma. (16895957)
2006
38
Adrenocortical carcinoma extending into the inferior vena cava: presentation of a 15-patient series and review of the literature. (16364713)
2006
39
Etoposide, doxorubicin and cisplatin plus mitotane in the treatment of advanced adrenocortical carcinoma: a large prospective phase II trial. (16172198)
2005
40
Ruptured adrenocortical carcinoma as a cause of paediatric acute abdomen. (15711359)
2005
41
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
42
Management of adrenocortical carcinoma. (15008991)
2004
43
Pregnancy with functioning adrenocortical carcinoma. (12126365)
2002
44
Adrenocortical carcinoma: clinical, morphologic, and molecular characterization. (11844815)
2002
45
Virilising adrenocortical carcinoma. (12405338)
2002
46
Selective testosterone secreting adrenocortical carcinoma in an infant. (11237246)
2001
47
Special feature: pathological case of the month. Denouement and discussion: congenital adrenocortical carcinoma. (11115315)
2000
48
Incidentally found adrenocortical carcinoma. A study of 21 patients. (9893659)
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

About this section

Expression for genes affiliated with Adrenocortical Carcinoma

About this section
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

About this section

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 Receptors37
9.6NR0B1, NR5A1
39.5NR5A1, INHA
4
Show member pathways
9.3FSHB, INHA

Compounds for genes affiliated with Adrenocortical Carcinoma

About this section

Compounds related to Adrenocortical Carcinoma according to GeneCards/GeneDecks:

(show all 25)
idCompoundScoreTop Affiliating Genes
117beta-hydroxysteroid449.9CYP11B2, NR5A1
2dmrt449.9NR5A1, NR0B1
3dhea449.8NR5A1, CYP11B2
4ptx1449.7FSHB, NR5A1
5radicicol44 61 1111.6TP53, UTS2
6proline449.5TP53, NR5A1, CYP11B2
73beta-hydroxysteroid449.5NR5A1, CYP11B2, INHA
8actinomycin d449.4FSHB, NR5A1, TP53
9forskolin44 50 1111.3INHA, NR5A1, CYP11B2
10leucine449.2CYP11B2, NR0B1, NR5A1
11estradiol44 24 1111.1INHA, FSHB, NR5A1
12cyclic amp44 2410.0CYP11B2, NR0B1, NR5A1, INHA
13thymidine44 2410.0TP53, UTS2, NR5A1
14ribonucleic acid448.9NR5A1, FSHB, CYP11B2, INHA
15gnrh448.8FSHB, INHA, NR0B1, NR5A1
16sodium44 249.8CYP11B2, UTS2, NR5A1
17oligonucleotide448.6CYP11B2, NR5A1, FSHB, TP53, INHA
18estrogen448.5NR0B1, NR5A1, FSHB, TP53, INHA
19acth448.5NR5A1, NR0B1, FSHB, INHA, CYP11B2
20testosterone44 61 24 1111.4NR0B1, NR5A1, CYP11B2, FSHB, INHA
21steroid448.4CYP11B2, INHA, FSHB, NR5A1, NR0B1
22cysteine448.3UTS2, NR0B1, TSPAN32, FSHB
23cholesterol44 28 24 1111.2CYP11B2, UTS2, NR5A1, NR0B1, TSPAN32
24progesterone44 28 61 24 1112.2NR5A1, TP53, INHA, CYP11B2, NR0B1, FSHB
25lipid448.0UTS2, CYP11B2, TSPAN32, NR5A1

GO Terms for genes affiliated with Adrenocortical Carcinoma

About this section

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:0303259.8NR0B1, NR5A1
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, NR5A1, INHA
7cell-cell signalingGO:0072679.1NR5A1, TSPAN32, INHA
8cell differentiationGO:0301549.0NR5A1, TP53, INHA

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

About this section
  • Antibodies
  • Proteins
  • Lysates

Sources for Adrenocortical Carcinoma

About this section
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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet