ACC
MCID: ADR015
MIFTS: 63

Adrenocortical Carcinoma (ACC) malady

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

Summaries for Adrenocortical Carcinoma

About this section
Sources:
8Disease Ontology, 63Wikipedia, 46OMIM, 32MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
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

About this section
Sources:
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
See all sources

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

About this section
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 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

About this section
Sources:
46OMIM
See all sources

Clinical features from OMIM:

202300

Clinical synopsis from OMIM:

202300

Drugs & Therapeutics for Adrenocortical Carcinoma

About this section
Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-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

About this section

Anatomical Context for Adrenocortical Carcinoma

About this section
Sources:
32MalaCards
See all sources

MalaCards organs/tissues related to Adrenocortical Carcinoma:

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

Animal Models for Adrenocortical Carcinoma or affiliated genes

About this section
Sources:
36MGI
See all sources

MGI Mouse Phenotypes related to Adrenocortical Carcinoma:

36
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

About this section
Sources:
50PubMed
See all sources

Articles related to Adrenocortical Carcinoma:

(show top 50)    (show all 547)
idTitleAuthorsYear
1
Myxoid variant of adrenocortical carcinoma: a report of two illustrative cases and a brief review of the literature. (24300732)
2014
2
Cytotoxic activity of gemcitabine, alone or in combination with mitotane, in adrenocortical carcinoma cell lines. (24018612)
2014
3
Adrenocortical carcinoma: a comprehensive immunohistochemical study of 40 cases. (23531850)
2014
4
Oncocytic adrenocortical carcinoma in a putty-nosed monkey (Cercopithecus nictitans) with hyperadrenocorticism. (23746483)
2013
5
A giant non-functional adrenocortical carcinoma presenting with acute kidney injury. (24375435)
2013
6
Solitary labial metastasis of adrenocortical carcinoma resembling a cystic tumor in a child. (24086826)
2013
7
Is there a role for laparoscopic adrenalectomy in patients with suspected adrenocortical carcinoma? A critical appraisal of the literature. (23315992)
2013
8
Childhood adrenocortical carcinoma as a sentinel cancer for detecting families with germline TP53 mutations. (22233476)
2012
9
Adrenocortical carcinoma in a dog with incomplete excision managed long-term with metastasectomy alone. (23033463)
2012
10
Adrenal gland: adrenocortical carcinoma-first RCT. (22614717)
2012
11
Worsening central sarcopenia and increasing intra-abdominal fat correlate with decreased survival in patients with adrenocortical carcinoma. (22526034)
2012
12
Metronomic therapy concepts in the management of adrenocortical carcinoma. (21971765)
2011
13
Adrenocortical carcinoma: the range of appearances on CT and MRI. (21606258)
2011
14
Adrenocortical carcinoma presenting as varicocele and renal vein thrombosis: a case report. (21806824)
2011
15
Fluorodesoxyglucose uptake in the remaining adrenal glands during the follow-up of patients with adrenocortical carcinoma: do not consider it as malignancy. (20921280)
2011
16
Perfluorinated compounds differentially affect steroidogenesis and viability in the human adrenocortical carcinoma (H295R) in vitro cell assay. (21641976)
2011
17
Metastatic adrenocortical carcinoma presenting simultaneously with Cushing's and Conn's syndromes: a case report. (21980052)
2011
18
The argument for mitotic rate-based grading for the prognostication of adrenocortical carcinoma. (21383612)
2011
19
Sunitinib Inhibits Cell Proliferation and Alters Steroidogenesis by Down-Regulation of HSD3B2 in Adrenocortical Carcinoma Cells. (22654799)
2011
20
Anti-CD10 (56C6) is expressed variably in adrenocortical tumors and cannot be used to discriminate clear cell renal cell carcinomas. (20390424)
2010
21
What is the best approach to an apparently nonmetastatic adrenocortical carcinoma? (20738315)
2010
22
Prognostic markers of survival after combined mitotane- and platinum-based chemotherapy in metastatic adrenocortical carcinoma. (20592067)
2010
23
Assessment of serum-free cortisol levels in patients with adrenocortical carcinoma treated with mitotane: a pilot study. (19473175)
2010
24
Oncocytic adrenocortical carcinoma. (20471567)
2010
25
Giant non-functioning adrenocortical carcinoma: A rare childhood tumor. (21209768)
2010
26
p53 Tetramerization domain mutations: germline R342X and R342P, and somatic R337G identified in pediatric patients with Li-Fraumeni syndrome and a child with adrenocortical carcinoma. (19714490)
2009
27
Wnt/betacatenin signaling in adrenocortical stem/progenitor cells: implications for adrenocortical carcinoma. (19286162)
2009
28
Management of endocrine manifestations and the use of mitotane as a chemotherapeutic agent for adrenocortical carcinoma. (19667279)
2009
29
Side population does not define stem cell-like cancer cells in the adrenocortical carcinoma cell line NCI h295R. (18063677)
2008
30
Newly developed hypertension as an early marker of recurrence of adrenocortical carcinoma with high renin expression. (18489644)
2008
31
Adrenocortical carcinoma survival rates correlated to genomic copy number variants. (18281524)
2008
32
Effect of epidermal growth factor and prostaglandin on the expression of aromatase (CYP19) in human adrenocortical carcinoma cell line NCI-H295R cells. (16394175)
2006
33
Aldosterone-producing adrenocortical carcinoma: an unusual cause of Conn's syndrome with an ominous clinical course. (15788646)
2005
34
Secondary hypertension in pregnancy due to an adrenocortical carcinoma. (15387874)
2004
35
Unusual double primary neoplasia: adrenocortical and ureteral carcinomas in werner syndrome. (14963361)
2004
36
Expression of the novel adrenocorticotropin-responsive gene selective Alzheimer's disease indicator-1 in the normal adrenal cortex and in adrenocortical adenomas and carcinomas. (15001630)
2004
37
Immunotherapy: new strategies for the treatment of adrenocortical carcinoma. (12931278)
2003
38
Large adrenocortical carcinoma extending into the inferior vena cava and right atrium. (12527494)
2003
39
Adrenocortical carcinoma: diagnosis, evaluation and treatment. (12478091)
2003
40
Selective testosterone secreting adrenocortical carcinoma in an infant. (11237246)
2001
41
Adrenocortical carcinoma. Clinical outcome at the end of the 20th century. (11571723)
2001
42
Adrenocortical carcinoma is characterized by a high frequency of chromosomal gains and high-level amplifications. (10824999)
2000
43
Evaluation of adrenocortical carcinoma in an infant using Ga-67 scintigraphy. (10795713)
2000
44
Clinical features and prognostic factors associated with adrenocortical carcinoma: Lahey Clinic Medical Center experience. (10651352)
2000
45
Adrenocortical carcinoma: clinical and laboratory observations. (10679640)
2000
46
Changes in neoplastic cell features and sensitivity to mitotane during mitotane-induced remission in a patient with recurrent, metastatic adrenocortical carcinoma. (10730906)
1999
47
Recurrent adrenocortical carcinoma in a child. (10383252)
1999
48
Mitotane associated with etoposide, doxorubicin, and cisplatin in the treatment of advanced adrenocortical carcinoma. Italian Group for the Study of Adrenal Cancer. (9827725)
1998
49
Overlapping expression of immunohistochemical markers and synaptophysin mRNA in pheochromocytomas and adrenocortical carcinomas. Implications for the differential diagnosis of adrenal gland tumors. (7723281)
1995
50
Favourable response of a virilizing adrenocortical carcinoma to preoperative treatment with ketoconazole and postoperative chemotherapy. (2031446)
1991

Genetic Variations for Adrenocortical Carcinoma

About this section

Expression for genes affiliated with Adrenocortical Carcinoma

About this section
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

About this section
Sources:
51QIAGEN, 37NCBI BioSystems Database, 29KEGG, 53Reactome, 12EMD Millipore
See all sources

Compounds for genes affiliated with Adrenocortical Carcinoma

About this section
Sources:
44Novoseek, 59Tocris Bioscience, 28IUPHAR, 11DrugBank, 24HMDB, 49PharmGKB, 2BitterDB
See all sources

Compounds related to Adrenocortical Carcinoma according to GeneCards/GeneDecks:

(show all 39)
idCompoundScoreTop Affiliating Genes
1acth4410.9NR0B1, CYP11B1, NR5A1, CYP11A1, INHA, ADM
2progesterone44 59 28 11 2414.9ADM, INHA, CYP11A1, CYP11B2, CYP11B1, NR0B1
33beta-hydroxysteroid4410.9CYP11B1, CYP11A1, NR5A1, INHA, CYP11B2
4cyclic amp44 2411.9INHA, NR5A1, ADM, CYP11A1, CYP11B2, CYP11B1
5corticosterone44 59 2412.9ADM, CYP11A1, NPW, NR5A1, CYP11B1, CYP11B2
6testosterone44 59 11 2413.9NR0B1, CYP11B1, CYP11B2, CYP11A1, INHA, ADM
7cholesterol44 28 11 2413.9ADM, NR5A1, NR0B1, CYP11B1, TSPAN32, CYP11B2
8doca4410.9CYP11A1, CYP11B2, CYP11B1
9steroid4410.9CYP11A1, NR0B1, NR5A1, CYP11B1, INHA, CYP11B2
10forskolin44 49 1112.9CYP11B2, CYP11B1, ADM
11oligonucleotide4410.9INHA, NR5A1, TP53, ADM, CYP11B2, CYP11B1
12aldosterone28 11 2412.9CYP11B1, CYP11B2, ADM
13androstenedione44 2411.9INHA, CYP11A1, CYP11B2, CYP11B1
14ribonucleic acid4410.8INHA, CYP11B1, NR5A1, CYP11A1, CYP11B2
1518-hydroxydeoxycorticosterone4410.8CYP11B2, CYP11B1
1618-oxocortisol4410.8CYP11B2, CYP11B1
1718-hydroxycortisol4410.8CYP11B1, CYP11B2
1817-hydroxyprogesterone44 2411.8CYP11B2, CYP11A1, CYP11B1
1911beta-hydroxysteroid4410.8CYP11B1, CYP11B2, CYP11A1
2021-deoxycortisol44 2411.8CYP11B2, CYP11B1
2118-hydroxycorticosterone44 2411.8CYP11B1, CYP11B2
2217beta-hydroxysteroid4410.8CYP11A1, NR5A1, CYP11B2
23dmrt4410.8NR5A1, NR0B1
24gnrh4410.8INHA, CYP11A1, NR0B1, NR5A1
25deoxycorticosterone28 2411.8CYP11B2, CYP11B1
26pcb 1264410.8CYP11B1, CYP11B2
27dhea4410.7CYP11A1, CYP11B2, NR5A1
28etomidate44 1111.7CYP11B2, CYP11B1
29cortisol28 2411.7CYP11B1, CYP11B2
30dbc-amp4410.7CYP11B2, ADM, CYP11A1
31estrogen4410.7ADM, CYP11A1, NR0B1, NR5A1, TP53, INHA
3211 deoxycortisol4410.6CYP11B2, CYP11B1
3322r-hydroxycholesterol44 2811.6NR5A1, CYP11A1
34pcbs4410.6CYP11B2, CYP11B1
35actinomycin d4410.6ADM, NR5A1, TP53, CYP11A1
36estradiol44 11 2412.5CYP11A1, INHA, CYP11B1, NR5A1
37hydrocortisone44 2 59 1113.5ADM, CYP11B2, CYP11B1
38dexamethasone44 49 28 1113.4ADM, CYP11B1, CYP11A1, CYP11B2, NR0B1
39potassium44 11 2412.2CYP11B2, ADM, NR5A1, CYP11B1

GO Terms for genes affiliated with Adrenocortical Carcinoma

About this section
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

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

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
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet