ACC
MCID: ADR016
MIFTS: 51

Adrenal Cortical Carcinoma (ACC)

Categories: Cancer diseases, Endocrine diseases, Rare diseases

Aliases & Classifications for Adrenal Cortical Carcinoma

MalaCards integrated aliases for Adrenal Cortical Carcinoma:

Name: Adrenal Cortical Carcinoma 41 12
Adrenocortical Carcinoma 41 12 75 53 44 15
Carcinoma of the Adrenal Cortex 12
Adrenal Cortex Carcinoma 17
Acc 53

Classifications:



External Ids:

Disease Ontology 12 DOID:3948
MeSH 44 D018268
NCIt 50 C9325
SNOMED-CT 68 2227007
ICD10 33 C74.0
UMLS 72 C0206686

Summaries for Adrenal Cortical Carcinoma

NIH Rare Diseases : 53 Adrenocortical carcinoma is a rare cancer affecting the outside of the adrenal glands (adrenal cortex). These glands are on top of each kidney and are responsible for producing certain hormones and keeping blood pressure at normal levels. Adrenocortical carcinoma is relatively frequent in children compared to many other cancers, although the cancer may also affect adults. Girls are more often affected than boys. Symptoms of adrenocortical carcinoma may include pain in the abdomen, hypertension, weight gain, frequent urination and possibly deepening of the voice. These symptoms are due to the tumors causing excess secretion of hormones from the adrenal glands. Adrenocortical carcinoma may develop by chance alone, but at least 50% of the cancers are thought to be hereditary. There are a number of genes that have changes (mutations) that can cause an adrenocortical carcinoma, including TP53 and IGF2. There have been reports of both autosomal dominant inheritance and autosomal recessive inheritance. An adrenocortical carcinoma is diagnosed based on urine tests for abnormal levels of cortisol, the hormone released by the adrenal glands. Blood tests can also be conducted to measure levels of potassium and sodium in the blood. A CT scan or MRI may be used to search for a visible tumor in the adrenal cortex. Treatment options include surgical removal of the tumor, which is important to achieve a good long-term outlook. Chemotherapy, specifically a drug called mitotane, can be used to try to remove any remaining cancer after surgery.

MalaCards based summary : Adrenal Cortical Carcinoma, also known as adrenocortical carcinoma, is related to adenoid cystic carcinoma and reticulum cell sarcoma. An important gene associated with Adrenal Cortical Carcinoma is HSD11B2 (Hydroxysteroid 11-Beta Dehydrogenase 2), and among its related pathways/superpathways are Aldosterone synthesis and secretion and Interleukin-4 and 13 signaling. The drugs Dopamine and Bromocriptine have been mentioned in the context of this disorder. Affiliated tissues include cortex, adrenal gland and adrenal cortex, and related phenotypes are cardiovascular system and behavior/neurological

Wikipedia : 75 Adrenocortical carcinoma (ACC) is an aggressive cancer originating in the cortex (steroid... more...

Related Diseases for Adrenal Cortical Carcinoma

Diseases related to Adrenal Cortical Carcinoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 619)
# Related Disease Score Top Affiliating Genes
1 adenoid cystic carcinoma 31.6 VIM TP53 S100B MUC1 CTNNB1 ACTC1
2 reticulum cell sarcoma 30.6 VIM MUC1 ACTC1
3 spindle cell sarcoma 30.4 VIM S100B MUC1 ACTC1
4 chordoma 30.4 VIM S100B MUC1
5 hemangioma 30.4 SYP CHGA ACTC1
6 acute adrenal insufficiency 30.4 POMC CYP11A1
7 leiomyoma 30.3 VIM IGF2 ACTC1
8 acinar cell carcinoma 30.2 TP53 CTNNB1 CHGA
9 small cell carcinoma 30.2 TP53 SYP CHGA
10 neurofibroma 30.1 SYP S100B MUC1
11 tubular adenocarcinoma 30.1 SYP MUC1 CHGA
12 horseshoe kidney 30.1 SYP CHGA ACTC1
13 rhabdomyosarcoma 30.1 VIM TP53 IGF2 ACTC1
14 teratoma 30.1 TP53 SYP ACTC1
15 gangliocytoma 30.0 SYP POMC
16 oncocytoma 30.0 VIM SYP MUC1
17 pleomorphic adenoma 29.8 VIM TP53 S100B MUC1 ACTC1
18 adenoma 29.6 TP53 POMC HSD11B2 CYP11B2 CTNNB1
19 hepatoblastoma 29.5 TP53 IGF2 CTNNB1
20 adrenal adenoma 29.5 POMC CYP11B2 CYP11B1 CYP11A1
21 conn's syndrome 29.4 POMC HSD11B2 CYP11B2 CYP11B1 CYP11A1
22 pancreatic endocrine carcinoma 29.4 SYP POMC
23 papilloma of choroid plexus 29.3 TP53 SYP S100B MUC1 IGF2
24 wilms tumor 1 29.1 TP53 NR5A1 IGF2 CTNNB1
25 adrenal cortical adenoma 29.0 SYP POMC NR5A1 CYP11B1 CYP11A1
26 carcinosarcoma 29.0 VIM TP53 MUC1 CTNNB1 ACTC1
27 primary pigmented nodular adrenocortical disease 29.0 SYP POMC CTNNB1 CHGA
28 gastrointestinal stromal tumor 28.9 VIM TP53 SYP S100B IGF2 CHGA
29 lipoid congenital adrenal hyperplasia 28.7 POMC NR5A1 CYP11B2 CYP11B1 CYP11A1
30 adrenal carcinoma 28.5 SYP POMC NR5A1 IGF2 CYP11B2 CYP11B1
31 adrenal rest tumor 28.4 SYP POMC NR5A1 CYP11B2 CYP11B1
32 endocrine gland cancer 28.4 TP53 SYP MUC1 IGF2 CTNNB1 CHGA
33 adrenocortical carcinoma, hereditary 26.6 TP53 SYP POMC NR5A1 IGF2 HSD11B2
34 adrenocortical carcinoma with pure aldosterone hypersecretion 12.5
35 corpus callosum, agenesis of 12.0
36 aplasia cutis congenita, nonsyndromic 12.0
37 agenesis of the corpus callosum with peripheral neuropathy 11.6
38 hereditary motor and sensory neuropathy with agenesis of the corpus callosum 11.5
39 aplasia cutis congenita 11.5
40 corpus callosum, agenesis of, with abnormal genitalia 11.5
41 aplasia cutis congenita with intestinal lymphangiectasia 11.3
42 epidermolysis bullosa junctionalis with pyloric atresia 11.2
43 aicardi syndrome 11.2
44 acetyl-coa carboxylase deficiency 11.2
45 vici syndrome 11.1
46 corpus callosum, partial agenesis of, x-linked 11.1
47 adams-oliver syndrome 2 11.1
48 adams-oliver syndrome 11.1
49 corpus callosum agenesis-intellectual disability-coloboma-micrognathia syndrome 11.1
50 ceruminous adenocarcinoma 11.1

Graphical network of the top 20 diseases related to Adrenal Cortical Carcinoma:



Diseases related to Adrenal Cortical Carcinoma

Symptoms & Phenotypes for Adrenal Cortical Carcinoma

MGI Mouse Phenotypes related to Adrenal Cortical Carcinoma:

46 (show all 11)
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.26 ACTC1 CHGA CTNNB1 CYP11A1 CYP11B1 CYP11B2
2 behavior/neurological MP:0005386 10.24 CTNNB1 CYP11A1 CYP11B1 HSD11B2 IGF2 NR5A1
3 homeostasis/metabolism MP:0005376 10.22 ACTC1 CHGA CTNNB1 CYP11A1 CYP11B1 CYP11B2
4 cellular MP:0005384 10.2 ACTC1 CTNNB1 CYP11A1 CYP11B1 HSD11B2 IGF2
5 mortality/aging MP:0010768 10.15 ACTC1 CHGA CTNNB1 CYP11A1 CYP11B1 HSD11B2
6 endocrine/exocrine gland MP:0005379 10.13 CHGA CTNNB1 CYP11A1 CYP11B1 CYP11B2 IGF2
7 nervous system MP:0003631 10 CHGA CTNNB1 CYP11A1 CYP11B2 IGF2 NR5A1
8 muscle MP:0005369 9.97 ACTC1 CHGA CTNNB1 CYP11A1 CYP11B1 HSD11B2
9 normal MP:0002873 9.76 ACTC1 CTNNB1 CYP11A1 HSD11B2 NR5A1 S100B
10 renal/urinary system MP:0005367 9.56 CHGA CTNNB1 CYP11B1 CYP11B2 HSD11B2 IGF2
11 reproductive system MP:0005389 9.23 CHGA CTNNB1 CYP11A1 CYP11B2 IGF2 NR5A1

Drugs & Therapeutics for Adrenal Cortical Carcinoma

Drugs for Adrenal Cortical Carcinoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 149)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dopamine Approved Phase 4 51-61-6, 62-31-7 681
2
Bromocriptine Approved, Investigational Phase 4 25614-03-3 31101
3 Dopamine Agents Phase 4
4 Antiparkinson Agents Phase 4
5 Dopamine agonists Phase 4
6
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
7
Mecasermin Approved, Investigational Phase 3 68562-41-4
8
Streptozocin Approved, Investigational Phase 3 18883-66-4 29327
9
Lenograstim Approved, Investigational Phase 3 135968-09-1
10
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
11
Etoposide Approved Phase 3 33419-42-0 36462
12
Mitotane Approved Phase 3 53-19-0 4211
13
Cisplatin Approved Phase 3 15663-27-1 84093 441203 2767
14 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
15
Liposomal doxorubicin Phase 3 31703
16 Antibiotics, Antitubercular Phase 3
17 Anti-Bacterial Agents Phase 3
18 Central Nervous System Depressants Phase 3
19 Anesthetics, General Phase 3
20 Anesthetics Phase 3
21 Anesthetics, Intravenous Phase 3
22 Adjuvants, Immunologic Phase 3
23 Immunologic Factors Phase 3
24 Cola Phase 3
25 Liver Extracts Phase 3
26 Analgesics Phase 3
27 Narcotics Phase 3
28 Analgesics, Opioid Phase 3
29 Adjuvants, Anesthesia Phase 3
30 Tubulin Modulators Phase 3
31 Antineoplastic Agents, Phytogenic Phase 3
32 Antineoplastic Agents, Hormonal Phase 3
33 Topoisomerase Inhibitors Phase 3
34 Antimitotic Agents Phase 3
35 Etoposide phosphate Phase 3
36 Keratolytic Agents Phase 3
37 Dermatologic Agents Phase 3
38 Podophyllotoxin Phase 3 518-28-5
39
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
40
Parathyroid hormone Approved, Investigational Phase 2 9002-64-6
41
Ritonavir Approved, Investigational Phase 2 155213-67-5 392622
42
Indinavir Approved Phase 2 150378-17-9 5362440
43
Vincristine Approved, Investigational Phase 2 57-22-7, 2068-78-2 5978
44
Etomidate Approved Phase 1, Phase 2 33125-97-2 36339 667484
45
Gefitinib Approved, Investigational Phase 2 184475-35-2 123631
46
acetic acid Approved Phase 2 64-19-7 176
47
Sunitinib Approved, Investigational Phase 2 557795-19-4, 341031-54-7 5329102
48
Tamoxifen Approved Phase 2 10540-29-1 2733526
49
Sodium citrate Approved, Investigational Phase 2 68-04-2
50
Oxaliplatin Approved, Investigational Phase 2 61825-94-3 5310940 9887054 43805 6857599

Interventional clinical trials:

(show top 50) (show all 74)
# Name Status NCT ID Phase Drugs
1 The Therapeutic Effect of Bromocriptin in Patients With Primary Aldosteronism Unknown status NCT00451672 Phase 4 bromocriptine
2 A Randomized, Double-blind, Placebo-controlled, Phase 3 Study of OSI-906 in Patients With Locally Advanced or Metastatic Adrenocortical Carcinoma Completed NCT00924989 Phase 3 OSI-906
3 First International Randomized Trial in Locally Advanced and Metastatic Adrenocortical Carcinoma Treatment Completed NCT00094497 Phase 3 Etoposide;Doxorubicin;Cisplatin;Streptozotocin;Mitotane
4 Treatment of Adrenocortical Tumors With Surgery Plus Lymph Node Dissection and Multiagent Chemotherapy: A Groupwide Phase III Study Completed NCT00304070 Phase 3 doxorubicin hydrochloride;cisplatin;mitotane;etoposide
5 A Randomized, Double-blind, Placebo-controlled Multi-center Study to Evaluate the Safety and Efficacy of Fentanyl Sublingual Spray (Fentanyl SL Spray) for the Treatment of Breakthrough Cancer Pain Completed NCT00538850 Phase 3 Fentanyl sublingual spray;Placebo
6 Mitotane With or Without Cisplatin and Etoposide After Surgery in Treating Participants With Stage I-III Adrenocortical Cancer With High Risk of Recurrence (ADIUVO-2) Recruiting NCT03583710 Phase 3 Cisplatin;Etoposide;Mitotane
7 Efficacy of Adjuvant Mitotane Treatment in Prolonging Recurrence-free Survival in Patients With Adrenocortical Carcinoma at Low-intermediate Risk of Recurrence Recruiting NCT00777244 Phase 3 MITOTANE
8 Mitotane With or Without Cisplatin and Etoposide After Surgery in Treating Participants With Stage I-III Adrenocortical Cancer With High Risk of Recurrence Recruiting NCT03723941 Phase 3 Cisplatin plus Etoposide
9 Sorafenib Plus Paclitaxel Metronomic Chemotherapy in Adreno-Cortical-Carcinoma Patients Progressing After 1st or 2nd Line Cytotoxic Chemotherapy Unknown status NCT00786110 Phase 2 Sorafenib;Paclitaxel
10 Radiation Therapy in Combination With Indinavir / Ritonavir (Crixivan / Norvir) for the Treatment of Brain Metastases: a Randomized Phase II Study Unknown status NCT00637637 Phase 2 indinavir sulfate;ritonavir
11 A Study of Combination Chemotherapy and Surgical Resection in the Treatment of Adrenocortical Carcinoma: Continuous Infusion Doxorubicin, Vincristine and Etoposide With Daily Mitotane Before and After Surgical Resection Completed NCT00001339 Phase 2 doxorubicin, vincristine, and etoposide with mitotane
12 Phase II Trial With Taxotere and Cisplatin in Non-operable Adrenocortical Carcinoma Completed NCT00324012 Phase 2 cisplatin, taxotere
13 Phase II Clinical Trial of Dovitinib (TKI-258) in First-line Metastatic or Locally Advanced Non-resectable Adrenocortical Carcinoma Completed NCT01514526 Phase 2 Dovitinib
14 Evaluation of 123I-Iodometomidate for Adrenal Scintigraphy Completed NCT00454103 Phase 1, Phase 2 123I-Iodometomidate
15 A Phase 1/2 Dose Escalation Study to Determine the Safety, Pharmacokinetics, and Pharmacodynamics of Intravenous TKM-080301 in Patients With Advanced Solid Tumors Completed NCT01262235 Phase 1, Phase 2 TKM-080301
16 A Phase II Trial of ZD1839 (Iressa) in Patients With Nonresectable Adrenocortical Carcinoma (ACC) Completed NCT00215202 Phase 2 Iressa (ZD1839)
17 A Phase II Study of the Orally Administered Negative Enantiomer of Gossypol (AT-101) in Patients With Advanced Adrenocortical Carcinoma (ACC) Completed NCT00848016 Phase 2 R-(-)-gossypol acetic acid
18 Sunitinib in Refractory Adrenocortical-Carcinoma Patients Progressing After Cytotoxic Chemotherapy Completed NCT00453895 Phase 2 Sunitinib
19 Cisplatin, Doxorubicin and Tamoxifen in the Treatment of Incurable Soft Tissue and Endocrine Malignancies Completed NCT00002608 Phase 2 cisplatin;doxorubicin hydrochloride;tamoxifen citrate
20 Phase II Trial of Surgical Resection and Heated Intraperitoneal Peritoneal Chemotherapy (HIPEC) for Adrenocortical Carcinoma Completed NCT01833832 Phase 2 Cisplatin;sodium thiosulfate
21 An Open-Label Phase 2a Study in Subjects With N-Cadherin Positive, Advanced or Recurrent Solid Tumors to Investigate the Safety and Efficacy of ADH-1 Administered Intravenously as a Single Agent (Adherex Protocol Number AHX-01-201) Completed NCT00264433 Phase 2 ADH -1 (Exherin™)
22 A Phase II, Open-label, Dose Titration, Multi-center Study to Assess the Safety/Tolerability and Efficacy of Osilodrostat in Patients With All Types of Endogenous Cushing's Syndrome Except Cushing's Disease Completed NCT02468193 Phase 2 Osilodrostat
23 A Phase II Study of Oxaliplatin in Children With Recurrent Solid Tumors Completed NCT00091182 Phase 2 oxaliplatin
24 Phase 2 Study of the Safety and Efficacy of CORT125134 in the Treatment of Endogenous Cushing's Syndrome Completed NCT02804750 Phase 2 CORT125134
25 A Phase II Study of IMC-A12 (Anti-IGF-I Receptor Monoclonal Antibody, NSC #742460) in Children With Relapsed/Refractory Solid Tumors Completed NCT00831844 Phase 2
26 Phase II Study of Axitinib (AG-013736) With Evaluation of the VEGF-Pathway in Metastatic, Recurrent or Primary Unresectable Adrenocortical Cancer Completed NCT01255137 Phase 2 Axitinib
27 A Study of Combination Chemotherapy & Surgical Resection in the Tx of Adrenocortical Cancer: Mitotane & Continuous Infusion Doxorubicin, Vincristine & Etoposide w/the P-glycoprotein Antagonist, Tariquidar (XR9576), Before & After Surgical Resection Completed NCT00071058 Phase 2 XR9576 (Tariquidar)
28 Phase II Study to Evaluate the Effects of Cabozantinib in Patients With Unresectable/Metastatic Adrenocortical Carcinoma Recruiting NCT03612232 Phase 2 Cabozantinib-s-malate
29 Phase 2 Trial of XL184 (Cabozantinib) an Oral Small-Molecule Inhibitor of Multiple Kinases, in Children and Young Adults With Refractory Sarcomas, Wilms Tumor, and Other Rare Tumors Recruiting NCT02867592 Phase 2 Cabozantinib;Cabozantinib S-malate
30 Phase II Trial of Surgical Resection and Heated Intraperitoneal Peritoneal Chemotherapy (HIPEC) for Adrenocortical Carcinoma Recruiting NCT03127774 Phase 2 Cisplatin;Sodium thiosulfate
31 A Phase II Study to Evaluate the Effects of Cabozantinib in Patients With Unresectable/Metastatic Adrenocortical Carcinoma Recruiting NCT03370718 Phase 2 Cabozantinib
32 A Phase II Study of Nivolumab Combined With Ipilimumab for Patients With Advanced Rare Genitourinary Tumors Recruiting NCT03333616 Phase 2 Ipilimumab;Nivolumab
33 An Open-Label Extension Study of the Safety of Relacorilant in the Treatment of the Signs and Symptoms of Cushing Syndrome Recruiting NCT03604198 Phase 2 relacorilant
34 Multicenter, Prospective, Non-randomized, Phase II Trial Designed to Evaluate the Activity of Cabazitaxel in Patients With Advanced Adreno-Cortical- Carcinoma Progressing After Previous Chemotherapy Lines Recruiting NCT03257891 Phase 2 Cabazitaxel
35 Phase II Study for the Evaluation of Efficacy of Pembrolizumab (MK-3475) in Patients With Rare Tumors Recruiting NCT02721732 Phase 2
36 MetronomIc CApecitabine and DOcetaxel as Second-line Chemotherapy for Advanced Gastric Cancer Patients Previously Treated With Fluoropyrimidine and Platinum Agents MiCADO Study Recruiting NCT02007148 Phase 2 capecitabine;Docetaxel
37 DART: Dual Anti-CTLA-4 and Anti-PD-1 Blockade in Rare Tumors Recruiting NCT02834013 Phase 2
38 A Phase II Clinical Trial of Single Agent Pembrolizumab in Subjects With Advanced Adrenocortical Carcinoma Active, not recruiting NCT02673333 Phase 2 Pembrolizumab
39 Activity of Abiraterone Acetate in the Management of Cushing's Syndrome in Patients With Adrenocortical Carcinoma Active, not recruiting NCT03145285 Phase 2 Abiraterone Acetate
40 Evaluation of Suramin in Advanced Adrenal Cortical Carcinoma, Phase II Terminated NCT00002921 Phase 2 suramin;therapeutic hydrocortisone
41 Phase II Study of Nivolumab (Anti-PD-1 Antibody) for Treatment of Metastatic Adrenocortical Carcinoma Terminated NCT02720484 Phase 2 Nivolumab
42 Phase II Study of Antineoplastons A10 and AS2-1 In Patients With Carcinoma of the Adrenal Gland Terminated NCT00003453 Phase 2 Antineoplaston therapy (Atengenal + Astugenal)
43 Multi-Institutional Phase II Study of IMC-A12, a Recombinant Human IgG1 Monoclonal Antibody Directed at the Type I Insulin-Like Growth Factor Receptor IGF1R, in Adrenocortical Carcinoma: IMC-A12 With Mitotane vs Mitotane Alone Terminated NCT00778817 Phase 2 mitotane
44 A Phase II Study of Bevacizumab in Patients With Nonresectable Cancer of the Adrenal Cortex. Withdrawn NCT00469469 Phase 2 Bevacizumab
45 Phase 1 Study of the Safety and Tolerability of ATR-101 in Adrenocortical Carcinoma Completed NCT01898715 Phase 1 ATR-101
46 A Phase I Trial of Suramin With Sequential Doxorubicin in Patients With Advanced Solid Tumors Completed NCT00003038 Phase 1 doxorubicin hydrochloride;suramin
47 A Phase 1 Dose Escalation Study of Seneca Valley Virus (NTX-010), A Replication-Competent Picornavirus, in Relapsed/Refractory Pediatric Patients With Neuroblastoma, Rhabdomyosarcoma, or Rare Tumors With Neuroendocrine Features Completed NCT01048892 Phase 1 cyclophosphamide
48 A Phase I, Pharmacokinetic, and Biologic Correlative Study of R115777 (NSC 702818) and Herceptin in Patients With Advanced Cancer Completed NCT00005842 Phase 1 tipifarnib
49 A Pilot Study of Metformin in Patients With a Diagnosis of Li-Fraumeni Syndrome Completed NCT01981525 Phase 1 Metformin
50 A Phase I Trial of Herceptin and Interleukin-12 Completed NCT00004074 Phase 1

Search NIH Clinical Center for Adrenal Cortical Carcinoma

Cochrane evidence based reviews: adrenocortical carcinoma

Genetic Tests for Adrenal Cortical Carcinoma

Anatomical Context for Adrenal Cortical Carcinoma

MalaCards organs/tissues related to Adrenal Cortical Carcinoma:

41
Cortex, Adrenal Gland, Adrenal Cortex, Testes, Liver, Kidney, Lung

Publications for Adrenal Cortical Carcinoma

Articles related to Adrenal Cortical Carcinoma:

(show top 50) (show all 2739)
# Title Authors PMID Year
1
In vitro modulation of multidrug resistance by pregnane steroids and in vivo inhibition of tumour development by 7α-OBz-11α(R)-OTHP-5β-pregnanedione in K562/R7 and H295R cell xenografts. 38
30777494 2019
2
Adrenocortical carcinoma - towards genomics guided clinical care. 38
31147626 2019
3
Adjuvant Radiation Improves Recurrence-Free Survival and Overall Survival in Adrenocortical Carcinoma. 38
31220287 2019
4
Insulin-Like Growth Factor and SLC12A7 Dysregulation: A Novel Signaling Hallmark of Non-Functional Adrenocortical Carcinoma. 38
31034883 2019
5
Rare cancers in childhood and adolescence in Brazil: First report of data from 19 population-based cancer registries. 38
31013367 2019
6
Imprint cytology of adrenal cortical carcinoma. A valuable surrogate for frozen sections. 38
31381263 2019
7
A phase II study of the orally administered negative enantiomer of gossypol (AT-101), a BH3 mimetic, in patients with advanced adrenal cortical carcinoma. 38
31172443 2019
8
SIRT6 abrogation promotes adrenocortical carcinoma through activation of NF-κB signaling. 38
30989475 2019
9
Giant nonfunctional ectopic adrenocortical carcinoma on the anterior abdominal wall: A case report. 38
31423440 2019
10
IGF2 role in adrenocortical carcinoma biology. 38
31378849 2019
11
[Diagnosis and surgical treatment of massive adrenal area tumor with tumor thrombus]. 38
31420622 2019
12
The incidence of endogenous Cushing's syndrome in the modern era. 38
31094003 2019
13
Metastatic Adrenal Cortical Carcinoma Responding to Octreotide: A Case Report. 38
31073023 2019
14
Adrenocortical carcinoma xenograft in zebrafish embryos as model to study the in vivo cytotoxicity of abiraterone acetate. 38
31397841 2019
15
LARGE ADRENAL INCIDENTALOMAS REQUIRE A DEDICATED DIAGNOSTIC PROCEDURE. 38
30865539 2019
16
Second follow-up of a German cohort on childhood cancer incidence after exposure to postnatal diagnostic X-ray. 38
31342929 2019
17
A method for the minimally invasive drug monitoring of mitotane by means of volumetric absorptive microsampling for a home-based therapeutic drug monitoring. 38
31093700 2019
18
Composite Adrenocortical Carcinoma and Neuroblastoma in an Infant With a TP53 Germline Mutation: A Case Report and Literature Review. 38
29746440 2019
19
Adrenocortical carcinoma arising from an adrenal adenoma in a young adult female. 38
31308928 2019
20
Nivolumab in metastatic adrenocortical carcinoma: results of a phase II trial. 38
31276163 2019
21
EZH2 cooperates with E2F1 to stimulate expression of genes involved in adrenocortical carcinoma aggressiveness. 38
31363169 2019
22
National Treatment Practice for Adrenocortical Carcinoma: Have they changed and have we made any progress? 38
31361313 2019
23
Identification of important invasion and proliferation related genes in adrenocortical carcinoma. 38
31321566 2019
24
Clinicopathological features and outcomes of adrenocortical carcinoma: A single institution experience. 38
31367073 2019
25
Value of Molecular Classification for Prognostic Assessment of Adrenocortical Carcinoma. 38
31294750 2019
26
Adrenocortical carcinoma: presentation and outcome of a contemporary patient series. 38
30980285 2019
27
Features of synchronous versus metachronous metastasectomy in adrenal cortical carcinoma: Analysis from the US adrenocortical carcinoma database. 38
31272813 2019
28
Volume-outcome relationship in adrenal surgery: A review of existing literature. 38
31331729 2019
29
5-oxo-ETE activates migration of H295R adrenocortical cells via MAPK and PKC pathways. 38
31301403 2019
30
H-score of 11β-hydroxylase and aldosterone synthase in the histopathological diagnosis of adrenocortical tumors. 38
31332713 2019
31
Targeting PDZ binding kinase is anti-tumorigenic in novel preclinical models of ACC. 38
31325906 2019
32
Radiological differentiation of phaeochromocytoma from other malignant adrenal masses: importance of wash-in characteristics on multiphase CECT. 38
31252396 2019
33
Adrenal natural orifice transluminal endoscopic surgery (NOTES): a step too far? 38
31404193 2019
34
Lynch syndrome and urologic malignancies: a contemporary review. 38
31045926 2019
35
Interpretation of core biopsy of liver mass lesions: A comparison study between cytopathologist and gastrointestinal pathologist. 38
31100645 2019
36
microRNA-431 as a Chemosensitizer and Potentiator of Drug Activity in Adrenocortical Carcinoma. 38
30918109 2019
37
Resection of an Adrenocortical Carcinoma Invading the Inferior Vena Cava Extending into the Right Ventricle. 38
31246598 2019
38
Epidemiological risk factors for adrenocortical carcinoma: A hospital-based case-control study. 38
31241762 2019
39
Changes in total and acylated ghrelin in patients with adrenocortical carcinoma during mitotane treatment. 38
31169262 2019
40
Targeted Assessment of G0S2 Methylation Identifies a Rapidly Recurrent, Routinely Fatal Molecular Subtype of Adrenocortical Carcinoma. 38
30770352 2019
41
Adjuvant mitotane therapy is beneficial in non-metastatic adrenocortical carcinoma at high risk of recurrence. 38
30991359 2019
42
Potentiation of mitotane action by rosuvastatin: New insights for adrenocortical carcinoma management. 38
30942448 2019
43
Clinical spectrum of Li-Fraumeni syndrome/Li-Fraumeni-like syndrome in Brazilian individuals with the TP53 p.R337H mutation. 38
30974190 2019
44
Management of orbital rhabdomyosarcoma in a child with Li-Fraumeni syndrome. 38
30974170 2019
45
Adrenocortical carcinoma: current knowledge. 38
29442479 2019
46
IGF and mTOR pathway expression and in vitro effects of linsitinib and mTOR inhibitors in adrenocortical cancer. 38
30838516 2019
47
DLK1/PREF1 marks a novel cell population in the human adrenal cortex. 38
31265901 2019
48
Endoscopic Ultrasonography-guided Fine-needle Aspiration Revealed Metastasis-induced Acute Pancreatitis in a Patient with Adrenocortical Carcinoma: A Case Report. 38
31178487 2019
49
Massive Migration of an Adrenocortical Carcinoma Thrombus. 38
31030537 2019
50
Screening in adrenal tumors. 38
30844886 2019

Variations for Adrenal Cortical Carcinoma

Cosmic variations for Adrenal Cortical Carcinoma:

9
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM4167506 PRKACA adrenal gland,adrenal gland,adrenal cortical adenoma,cortisol producing c.617T>G p.L206R 19:14097604-14097604 8

Expression for Adrenal Cortical Carcinoma

Search GEO for disease gene expression data for Adrenal Cortical Carcinoma.

Pathways for Adrenal Cortical Carcinoma

GO Terms for Adrenal Cortical Carcinoma

Biological processes related to Adrenal Cortical Carcinoma according to GeneCards Suite gene sharing:

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 cytokine-mediated signaling pathway GO:0019221 9.84 VIM TP53 POMC MUC1
2 positive regulation of gene expression GO:0010628 9.83 VIM TP53 NR5A1 CTNNB1 ACTC1
3 steroid metabolic process GO:0008202 9.72 CYP11B2 CYP11B1 CYP11A1
4 cholesterol metabolic process GO:0008203 9.69 CYP11B2 CYP11B1 CYP11A1
5 regulation of blood pressure GO:0008217 9.65 POMC CYP11B1 CHGA
6 steroid biosynthetic process GO:0006694 9.63 CYP11B2 CYP11B1 CYP11A1
7 DNA damage response, signal transduction by p53 class mediator resulting in transcription of p21 class mediator GO:0006978 9.52 TP53 MUC1
8 cellular response to potassium ion GO:0035865 9.49 CYP11B2 CYP11B1
9 sterol metabolic process GO:0016125 9.43 CYP11B2 CYP11B1 CYP11A1
10 cortisol biosynthetic process GO:0034651 9.4 CYP11B2 CYP11B1
11 aldosterone biosynthetic process GO:0032342 9.37 CYP11B2 CYP11B1
12 cellular response to peptide hormone stimulus GO:0071375 9.33 CYP11B2 CYP11B1 CYP11A1
13 regulation of blood volume by renal aldosterone GO:0002017 9.32 HSD11B2 CYP11B2
14 C21-steroid hormone biosynthetic process GO:0006700 9.13 CYP11B2 CYP11B1 CYP11A1
15 glucocorticoid biosynthetic process GO:0006704 8.92 HSD11B2 CYP11B2 CYP11B1 CYP11A1

Molecular functions related to Adrenal Cortical Carcinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen GO:0016705 9.33 CYP11B2 CYP11B1 CYP11A1
2 steroid 11-beta-monooxygenase activity GO:0004507 8.96 CYP11B2 CYP11B1
3 corticosterone 18-monooxygenase activity GO:0047783 8.62 CYP11B2 CYP11B1

Sources for Adrenal Cortical Carcinoma

3 CDC
7 CNVD
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10 dbSNP
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17 EFO
18 ExPASy
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