ADCC
MCID: ADR054
MIFTS: 66

Adrenocortical Carcinoma, Hereditary (ADCC)

Categories: Cancer diseases, Endocrine diseases, Genetic diseases, Rare diseases, Reproductive diseases

Aliases & Classifications for Adrenocortical Carcinoma, Hereditary

MalaCards integrated aliases for Adrenocortical Carcinoma, Hereditary:

Name: Adrenocortical Carcinoma, Hereditary 56 29 6 39 71
Adrenocortical Carcinoma 58 73 29 6 43 71
Adrenocortical Carcinoma, Pediatric 56 29 6 71
Adcc 56 73
Malignant Neoplasm of Adrenal Cortex 71
Hereditary Adrenocortical Carcinoma 73
Pediatric Adrenocortical Carcinoma 73
Carcinoma Adrenocortical 54
Tumors of Adrenal Cortex 71
Adrenocortical Cancer 54

Characteristics:

Orphanet epidemiological data:

58
adrenocortical carcinoma
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe); Age of onset: Childhood;

OMIM:

56
Inheritance:
autosomal dominant


HPO:

31
adrenocortical carcinoma, hereditary:
Inheritance autosomal dominant inheritance autosomal recessive inheritance


Classifications:

Orphanet: 58  
Rare infertility disorders
Rare endocrine diseases


External Ids:

OMIM 56 202300
MeSH 43 D018268
MESH via Orphanet 44 D018268
ICD10 via Orphanet 33 C74.0
UMLS via Orphanet 72 C0206686
Orphanet 58 ORPHA1501
UMLS 71 C0001618 C0206686 C0346402 more

Summaries for Adrenocortical Carcinoma, Hereditary

OMIM : 56 Adrenocortical carcinoma (ADCC) is a rare but aggressive childhood tumor, representing about 0.4% of childhood tumors, with a high incidence of associated tumors. ADCC occurs with increased frequency in patients with the Beckwith-Wiedemann syndrome (130650) and is a component tumor in Li-Fraumeni syndrome (LFS; 151623). (202300)

MalaCards based summary : Adrenocortical Carcinoma, Hereditary, also known as adrenocortical carcinoma, is related to adrenal cortical carcinoma and conn's syndrome, and has symptoms including fever and flank pain. An important gene associated with Adrenocortical Carcinoma, Hereditary is TP53 (Tumor Protein P53), and among its related pathways/superpathways are Parkinson disease and Toll-like Receptor Signaling Pathway. The drugs Bromocriptine and Dopamine have been mentioned in the context of this disorder. Affiliated tissues include cortex, adrenal cortex and breast, and related phenotypes are adrenocortical carcinoma and muscle weakness

UniProtKB/Swiss-Prot : 73 Adrenocortical carcinoma: A malignant neoplasm of the adrenal cortex and a rare childhood tumor. It occurs with increased frequency in patients with Beckwith-Wiedemann syndrome and Li-Fraumeni syndrome.

Related Diseases for Adrenocortical Carcinoma, Hereditary

Diseases related to Adrenocortical Carcinoma, Hereditary via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 485)
# Related Disease Score Top Affiliating Genes
1 adrenal cortical carcinoma 32.2 TP53 PRKAR1A MIR483 MC2R CYP17A1 CYP11A1
2 conn's syndrome 31.3 PRKAR1A MC2R GNAS CYP17A1 CYP11A1
3 adrenal carcinoma 31.3 TP53 STAR PRKAR1A MIR483 CYP17A1 CYP11A1
4 adenoma 30.3 TP53 PRKAR1A PIK3CA MC2R GNAS CTNNB1
5 breast adenocarcinoma 30.2 TP53 PIK3CA H19 CHEK2
6 transitional cell carcinoma 30.2 TP53 PIK3CA CTNNB1 BRAF
7 carcinosarcoma 30.1 TP53 PIK3CA CTNNB1
8 renal cell carcinoma, nonpapillary 30.1 TP53 PIK3CA NRAS MIR214 MIR195 H19
9 ovary adenocarcinoma 30.1 TP53 PIK3CA GNAS CTNNB1
10 myeloma, multiple 30.0 TP53 NRAS MIR483 H19 CTNNB1 BRAF
11 li-fraumeni syndrome 30.0 TP53 MIR483 CTNNB1 CHEK2
12 endocrine gland cancer 30.0 MIR503 MIR483 MIR214 MIR195 GNAS
13 adenocarcinoma 30.0 TP53 PIK3CA H19 GNAS CTNNB1 BRAF
14 lymphoma, non-hodgkin, familial 29.9 TP53 PIK3CA NRAS MIR483 MIR214 BRAF
15 beckwith-wiedemann syndrome 29.9 TP53 PRKAR1A MIR483 H19 GNAS CTNNB1
16 lynch syndrome 29.9 TP53 PIK3CA CYP17A1 CTNNB1 CHEK2 BRAF
17 peutz-jeghers syndrome 29.8 TP53 PRKAR1A GNAS CTNNB1
18 acth-independent cushing syndrome 29.8 PRKAR1A GNAS
19 meningioma, familial 29.7 TP53 PRKAR1A PIK3CA CTNNB1
20 neuroblastoma 29.7 TP53 PIK3CA NRAS MIR184 H19 CTNNB1
21 sarcoma 29.6 TP53 PIK3CA NRAS CTNNB1 CHEK2 BRAF
22 thyroid tumor 29.6 TP53 PRKAR1A PIK3CA NRAS BRAF
23 carney complex variant 29.6 PRKAR1A MIR483 MC2R GNAS CTNNB1
24 acth-independent macronodular adrenal hyperplasia 29.5 PRKAR1A MIR483 MC2R GNAS
25 eye disease 29.5 TP53 MIR483 MIR214 IGFBP2 CTNNB1
26 leukemia, acute myeloid 29.4 TP53 PIK3CA NRAS MIR483 MIR195 MIR184
27 melanoma 29.4 TP53 PIK3CA NRAS MIR184 H19 CTNNB1
28 leukemia, chronic lymphocytic 29.4 TP53 PIK3CA NRAS MIR483 MIR214 MIR195
29 adrenal cortical adenoma 29.4 PRKAR1A MIR483 MC2R CYP17A1 CYP11A1 CTNNB1
30 squamous cell carcinoma 29.3 TP53 PIK3CA H19 GNAS CTNNB1 CHEK2
31 bladder cancer 29.3 TP53 PIK3CA NRAS MIR214 MIR195 H19
32 endometrial cancer 29.3 TP53 PIK3CA NRAS H19 CYP17A1 CTNNB1
33 glioblastoma multiforme 29.2 TP53 PIK3CA NRAS IGFBP2 CTNNB1 CHEK2
34 adrenal adenoma 29.1 PRKAR1A MIR483 MC2R GNAS CYP17A1 CYP11A1
35 primary pigmented nodular adrenocortical disease 28.9 PRKAR1A MIR483 MC2R IGFBP2 GNAS CTNNB1
36 lipoid congenital adrenal hyperplasia 28.8 STAR MC2R CYP17A1 CYP11A1
37 lung cancer susceptibility 3 28.8 TP53 PIK3CA NRAS MIR503 MIR483 H19
38 leydig cell tumor 28.5 STAR GNAS CYP17A1 CYP11A1
39 hypoadrenocorticism, familial 28.3 STAR MC2R CYP17A1 CYP11A1
40 hepatocellular carcinoma 28.2 TP53 PIK3CA NRAS MIR503 MIR483 MIR214
41 ovarian cancer 27.9 TP53 PIK3CA MIR483 MIR214 MIR195 MIR184
42 ovarian disease 27.6 TP53 STAR MIR483 IGFBP2 CYP17A1 CYP11A1
43 prostate cancer 27.3 TP53 PIK3CA MIR503 MIR214 MIR195 MIR184
44 adrenocortical carcinoma with pure aldosterone hypersecretion 12.5
45 adrenal cortical adenocarcinoma 12.3
46 rare tumor 10.7
47 vulvar intraepithelial neoplasia 10.6 TP53 CTNNB1
48 nevus of ota 10.5 TP53 BRAF
49 rare head and neck tumor 10.5 TP53 PIK3CA
50 melphalan allergy 10.5 NRAS BRAF

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



Diseases related to Adrenocortical Carcinoma, Hereditary

Symptoms & Phenotypes for Adrenocortical Carcinoma, Hereditary

Human phenotypes related to Adrenocortical Carcinoma, Hereditary:

58 31 (show all 29)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 adrenocortical carcinoma 58 31 obligate (100%) Obligate (100%) HP:0006744
2 muscle weakness 58 31 frequent (33%) Frequent (79-30%) HP:0001324
3 hypokalemia 58 31 frequent (33%) Frequent (79-30%) HP:0002900
4 hyperhidrosis 58 31 frequent (33%) Frequent (79-30%) HP:0000975
5 irritability 58 31 frequent (33%) Frequent (79-30%) HP:0000737
6 weight loss 58 31 frequent (33%) Frequent (79-30%) HP:0001824
7 abdominal pain 58 31 frequent (33%) Frequent (79-30%) HP:0002027
8 anxiety 58 31 frequent (33%) Frequent (79-30%) HP:0000739
9 hypertension 58 31 frequent (33%) Frequent (79-30%) HP:0000822
10 striae distensae 58 31 frequent (33%) Frequent (79-30%) HP:0001065
11 diabetes mellitus 58 31 frequent (33%) Frequent (79-30%) HP:0000819
12 hyperaldosteronism 58 31 frequent (33%) Frequent (79-30%) HP:0000859
13 abnormality of reproductive system physiology 58 31 frequent (33%) Frequent (79-30%) HP:0000080
14 lung adenocarcinoma 58 31 frequent (33%) Frequent (79-30%) HP:0030078
15 adrenocorticotropic hormone deficiency 58 31 frequent (33%) Frequent (79-30%) HP:0011748
16 hypertrichosis 58 31 frequent (33%) Frequent (79-30%) HP:0000998
17 increased body weight 58 31 frequent (33%) Frequent (79-30%) HP:0004324
18 palpitations 58 31 frequent (33%) Frequent (79-30%) HP:0001962
19 increased urinary cortisol level 58 31 frequent (33%) Frequent (79-30%) HP:0012030
20 paradoxical increased cortisol secretion on dexamethasone suppression test 58 31 frequent (33%) Frequent (79-30%) HP:0003466
21 increased serum estradiol 58 31 frequent (33%) Frequent (79-30%) HP:0025134
22 panic attack 58 31 frequent (33%) Frequent (79-30%) HP:0025269
23 increased serum androstenedione 58 31 frequent (33%) Frequent (79-30%) HP:0025380
24 elevated serum 11-deoxycortisol 58 31 frequent (33%) Frequent (79-30%) HP:0025436
25 abnormal serum dehydroepiandrosterone level 58 31 frequent (33%) Frequent (79-30%) HP:0500022
26 abnormality of urine homeostasis 58 31 occasional (7.5%) Occasional (29-5%) HP:0003110
27 abnormality of metabolism/homeostasis 58 Frequent (79-30%)
28 increased circulating androgen level 58 Frequent (79-30%)
29 increased circulating cortisol level 58 Frequent (79-30%)

Symptoms via clinical synopsis from OMIM:

56
Neoplasia:
adrenocortical carcinoma

Endocrine Features:
virilism

Clinical features from OMIM:

202300

UMLS symptoms related to Adrenocortical Carcinoma, Hereditary:


fever, flank pain

MGI Mouse Phenotypes related to Adrenocortical Carcinoma, Hereditary:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.11 BRAF CTNNB1 CYP11A1 CYP17A1 GNAS IGFBP2
2 endocrine/exocrine gland MP:0005379 10.06 BRAF CHEK2 CTNNB1 CYP11A1 GNAS MC2R
3 immune system MP:0005387 10 BRAF CHEK2 CTNNB1 CYP11A1 GNAS IGFBP2
4 adipose tissue MP:0005375 9.95 BRAF CYP17A1 GNAS MC2R PIK3CA PRKAR1A
5 liver/biliary system MP:0005370 9.76 BRAF CTNNB1 CYP11A1 GNAS IGFBP2 NRAS
6 neoplasm MP:0002006 9.56 BRAF CHEK2 CTNNB1 GNAS NRAS PIK3CA
7 pigmentation MP:0001186 9.02 BRAF CTNNB1 NRAS PRKAR1A TP53

Drugs & Therapeutics for Adrenocortical Carcinoma, Hereditary

Drugs for Adrenocortical Carcinoma, Hereditary (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 267)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bromocriptine Approved, Investigational Phase 4 25614-03-3 31101
2
Dopamine Approved Phase 4 51-61-6, 62-31-7 681
3 Dopamine agonists Phase 4
4 Dopamine Agents Phase 4
5 Antiparkinson Agents Phase 4
6
Epinephrine Approved, Vet_approved Phase 3 51-43-4 5816
7
Racepinephrine Approved Phase 3 329-65-7 838
8
Etomidate Approved Phase 3 33125-97-2 36339 667484
9
Streptozocin Approved, Investigational Phase 3 18883-66-4 29327
10
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
11
Etoposide Approved Phase 3 33419-42-0 36462
12
Cisplatin Approved Phase 3 15663-27-1 2767 441203 84093
13
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3 1177-87-3
14
Dexamethasone Approved, Investigational, Vet_approved Phase 3 50-02-2 5743
15
Lenalidomide Approved Phase 3 191732-72-6 216326
16
rituximab Approved Phase 3 174722-31-7 10201696
17
Carboplatin Approved Phase 3 41575-94-4 10339178 38904 498142
18
Atezolizumab Approved, Investigational Phase 3 1380723-44-3
19
Daratumumab Approved Phase 3 945721-28-8
20
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
21
Docetaxel Approved, Investigational Phase 3 114977-28-5 148124
22
Paclitaxel Approved, Vet_approved Phase 3 33069-62-4 36314
23
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
24
Trastuzumab Approved, Investigational Phase 3 180288-69-1 9903
25
Aldesleukin Approved Phase 3 85898-30-2, 110942-02-4
26
Isotretinoin Approved Phase 3 4759-48-2 5282379 5538
27
Daunorubicin Approved Phase 3 20830-81-3 30323
28
Lenograstim Approved, Investigational Phase 2, Phase 3 135968-09-1
29
Vinorelbine Approved, Investigational Phase 2, Phase 3 71486-22-1 60780 44424639
30
Sargramostim Approved, Investigational Phase 2, Phase 3 83869-56-1, 123774-72-1
31
Vitamin A Approved, Nutraceutical, Vet_approved Phase 3 22737-96-8, 68-26-8, 11103-57-4 9904001 445354
32
Tretinoin Approved, Investigational, Nutraceutical Phase 3 302-79-4 444795 5538
33 Pancreatic Polypeptide Investigational Phase 3 59763-91-6
34 Molgramostim Investigational Phase 3 99283-10-0
35 Epinephryl borate Phase 3
36 Fluorodeoxyglucose F18 Phase 3
37 Cola Phase 3
38 Anesthetics Phase 3
39 Narcotics Phase 3
40 Analgesics, Opioid Phase 3
41 Anesthetics, General Phase 3
42 Anesthetics, Intravenous Phase 3
43 Antiemetics Phase 2, Phase 3
44 Gastrointestinal Agents Phase 2, Phase 3
45 Angiogenesis Inhibitors Phase 3
46 Etoposide phosphate Phase 3
47 Podophyllotoxin Phase 3 518-28-5
48 Anti-Infective Agents Phase 3
49 Retinol palmitate Phase 3
50 retinol Phase 3

Interventional clinical trials:

(show top 50) (show all 194)
# 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 Multi-center, OPen, InvEstigator Initiated Phase IV Clinical TRial to Evaluate the Efficacy and SaFety of Ingenol Mebutate Gel 0.015% on Face and Scalp & 0.05% on Trunk and Extremities in KorEan Patient With ACtinic KeraTosis (PERFECT) Completed NCT02716714 Phase 4 ingenol mebutate gel 0.015%;ingenol mebutate gel 0.05%
3 Treatment of Adrenocortical Tumors With Surgery Plus Lymph Node Dissection and Multiagent Chemotherapy: A Groupwide Phase III Study Unknown status NCT00304070 Phase 3 doxorubicin hydrochloride;cisplatin;mitotane;etoposide
4 Combined 18F-Fluorodeoxyglucose (FDG) Positron Emission Tomography (PET) and 123I-Iodometomidate (123I-IMTO) Imaging for Adrenal Neoplasia Unknown status NCT02010957 Phase 3
5 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
6 First International Randomized Trial in Locally Advanced and Metastatic Adrenocortical Carcinoma Treatment Completed NCT00094497 Phase 3 Etoposide;Doxorubicin;Cisplatin;Streptozotocin;Mitotane
7 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
8 "Monoclonal Antibodies for Treatment of Multiple Myeloma. Present Status and Aspects of Effector Mechanisms With Emphasis on the CD38 Antibody Daratumumab " Completed NCT02419118 Phase 2, Phase 3 Daratumumab;Lenalidomide;Dexamethasone
9 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
10 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
11 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
12 A Phase III Double Blind Randomized Placebo Controlled Study of Trastuzumab as Short Duration Preoperative Therapy in Patients With HER2-neu Positive Operable Breast Cancer Recruiting NCT01785420 Phase 3 Trastuzumab;Placebo
13 A Randomized Phase III Multicenter Trial Assessing Efficacy and Toxicity of a Combination of Rituximab and Lenalidomide (R2) vs Rituximab Alone as Maintenance After Chemoimmunotherapy With Rituximab-chemotherapy (R-CHT) for Relapsed/Refractory FL Patients Not Eligible for Autologous Transplantation (ASCT). Recruiting NCT02390869 Phase 3 R2-MANT;R-MANT
14 Atezolizumab, Pertuzumab and Trastuzumab With Chemotherapy as Neoadjuvant Treatment of HER2 Positive Early High-risk and Locally Advanced Breast Cancer (APTneo) Recruiting NCT03595592 Phase 3 Trastuzumab;Pertuzumab;Carboplatin;Paclitaxel;Doxorubicin;Cyclophosphamide;Atezolizumab
15 A Conceptual Study of Daratumumab Intensified Treatment to Eligible Multiple Myeloma New Patients- Cyclophosphamide, Thalidomide, Dexamethasone and Daratumumab Induction, Follow by Daratumumab Consolidation and Maintenance Recruiting NCT03792620 Phase 3
16 A Randomized Phase III Trial of Adjuvant Therapy Comparing Chemotherapy Alone (Six Cycles of Docetaxel Plus Cyclophosphamide or Four Cycles of Doxorubicin Plus Cyclophosphamide Followed by Weekly Paclitaxel) to Chemotherapy Plus Trastuzumab in Women With Node-Positive or High-Risk Node-Negative HER2-Low Invasive Breast Cancer Active, not recruiting NCT01275677 Phase 3 Cyclophosphamide;Docetaxel;Doxorubicin;Doxorubicin Hydrochloride;Paclitaxel
17 Phase III Randomized Study of Chimeric Antibody 14.18 (Ch14.18) in High Risk Neuroblastoma Following Myeloablative Therapy and Autologous Stem Cell Rescue Active, not recruiting NCT00026312 Phase 3 Isotretinoin
18 A Phase II Trial of Trastuzumab, Neupogen, and Vinorelbine Investigating the Effects on Immune Function and Clinical Outcomes in Patients With Metastatic Breast Cancer Overexpressing Her-2/Neu Terminated NCT00169104 Phase 2, Phase 3 G-CSF;trastuzumab;vinorelbine;G-CSF;saline placebo
19 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
20 Phase II Clinical Trial of Immunotherapy With Rituximab and Autologous Effector Lymphocytes in Patients With Non-Hodgkin Follicular Lymphoma in Response to First Line Chemotherapy Unknown status NCT01329354 Phase 2
21 A Basic-clinical Translational Research in Hepatitis B Virus (HBV)-Specific Antigen Peptides and HepG2 Cell Lysate Co-activated Dendritic Cells Combined With Transarterial Chemoembolization (TACE) in HBV-related HCC Treatment Unknown status NCT03086564 Phase 1, Phase 2
22 Combination Trastuzumab With Expanded NK Cells for Treating HER2-positive Gastric Cancer Unknown status NCT02805829 Phase 1, Phase 2 Trastuzumab + NK cells
23 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
24 Phase II Multicentric Randomized Trial, Evaluating the Best Protocol of Chemotherapy, Associated With Targeted Therapy According to the Tumor KRAS Status, in Metastatic Colorectal Cancer (CCRM) Patients With Initially Non-resectable Hepatic Metastases Unknown status NCT01442935 Phase 2 Oxaliplatin;Folinic Acid;5-FU;Irinotecan;Irinotecan;Bevacizumab;Cetuximab
25 Phase I/II Study of Expanded, Activated Autologous Natural Killer Cell Infusions With Trastuzumab for Patients With HER2+ Breast and Gastric Cancer Unknown status NCT02030561 Phase 1, Phase 2 Trastuzumab + NK cells
26 Phase 1/2 Study of Lenalidomide and Cetuximab in Patients With Advanced Solid Tumors Unknown status NCT01166035 Phase 1, Phase 2 Lenalidomide;Cetuximab
27 Optimal Control of Liver Metastases With Intravenous Cetuximab and Hepatic Artery Infusion of Three-drug Chemotherapy in Patients With Liver-only Metastases From Colorectal Cancer. A European Multicenter Phase II Trial Unknown status NCT00852228 Phase 2 IV cetuximab;HAI chronomodulated chemotherapy;HAI conventional chemotherapy
28 A Phase II Trial of ZD1839 (Iressa) in Patients With Nonresectable Adrenocortical Carcinoma (ACC) Completed NCT00215202 Phase 2 Iressa (ZD1839)
29 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
30 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
31 Phase II Trial With Taxotere and Cisplatin in Non-operable Adrenocortical Carcinoma Completed NCT00324012 Phase 2 cisplatin, taxotere
32 Phase II Clinical Trial of Dovitinib (TKI-258) in First-line Metastatic or Locally Advanced Non-resectable Adrenocortical Carcinoma Completed NCT01514526 Phase 2 Dovitinib
33 Evaluation of 123I-Iodometomidate for Adrenal Scintigraphy Completed NCT00454103 Phase 1, Phase 2 123I-Iodometomidate
34 Sunitinib in Refractory Adrenocortical-Carcinoma Patients Progressing After Cytotoxic Chemotherapy Completed NCT00453895 Phase 2 Sunitinib
35 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™)
36 Phase II Trial of Surgical Resection and Heated Intraperitoneal Peritoneal Chemotherapy (HIPEC) for Adrenocortical Carcinoma Completed NCT01833832 Phase 2 Cisplatin;sodium thiosulfate
37 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
38 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)
39 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
40 Cisplatin, Doxorubicin and Tamoxifen in the Treatment of Incurable Soft Tissue and Endocrine Malignancies Completed NCT00002608 Phase 2 cisplatin;doxorubicin hydrochloride;tamoxifen citrate
41 Phase II Study of Cetuximab and Lenalidomide in Recurrent/Metastatic Squamous Cell Carcinoma of the Head and Neck Completed NCT01133665 Phase 2 Cetuximab and Lenalidomide
42 A Phase II Study of Oxaliplatin in Children With Recurrent Solid Tumors Completed NCT00091182 Phase 2 oxaliplatin
43 Phase 2 Study of the Safety and Efficacy of CORT125134 in the Treatment of Endogenous Cushing's Syndrome Completed NCT02804750 Phase 2 CORT125134
44 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
45 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
46 A Phase I/II, a Single Arm, Open-label Study of Ofatumumab (GSK1841157) in Patients With Previously Treated Chronic Lymphocytic Leukemia Completed NCT01077622 Phase 2 ofatumumab 100 mg, 1000 mg / vial
47 A Phase II Study of Rituxan and IL-2 in Patients With Low Grade or Follicular B-Cell Lymphoma Completed NCT00003356 Phase 1, Phase 2
48 Phase II Study of Cetuximab Combined With Cisplatin or Carboplatin/Pemetrexed as First Line Treatment in Patients With Malignant Pleural Mesothelioma. Completed NCT00996567 Phase 2 Cetuximab (Erbitux)
49 Open-Label, Multi-Center, Phase 2 Study of Anti-CCR4 Monoclonal Antibody KW-0761 (Mogamulizumab) in Subjects With Previously Treated Peripheral T-cell Lymphoma (PTCL) Completed NCT01611142 Phase 2
50 Phase II Study of the Anti-Ganglioside GD3 Mouse/Human Chimeric Antibody KW2871 Combined With High Dose Interferon-α2b in Patients With Metastatic Melanoma Completed NCT00679289 Phase 2 HDI;KW2871;KW2871;KW2871

Search NIH Clinical Center for Adrenocortical Carcinoma, Hereditary

Inferred drug relations via UMLS 71 / NDF-RT 50 :


Mitotane

Cochrane evidence based reviews: adrenocortical carcinoma

Genetic Tests for Adrenocortical Carcinoma, Hereditary

Genetic tests related to Adrenocortical Carcinoma, Hereditary:

# Genetic test Affiliating Genes
1 Adrenocortical Carcinoma, Hereditary 29 TP53
2 Adrenocortical Carcinoma 29
3 Adrenocortical Carcinoma, Pediatric 29

Anatomical Context for Adrenocortical Carcinoma, Hereditary

MalaCards organs/tissues related to Adrenocortical Carcinoma, Hereditary:

40
Cortex, Adrenal Cortex, Breast, Adrenal Gland, Liver, B Cells, Thyroid

Publications for Adrenocortical Carcinoma, Hereditary

Articles related to Adrenocortical Carcinoma, Hereditary:

(show top 50) (show all 2593)
# Title Authors PMID Year
1
Identification of a novel TP53 germline mutation E285V in a rare case of paediatric adrenocortical carcinoma and choroid plexus carcinoma. 61 54 6 56
18762572 2008
2
Inhibin alpha-subunit (INHA) gene and locus changes in paediatric adrenocortical tumours from TP53 R337H mutation heterozygote carriers. 6 56
15121773 2004
3
Penetrance of adrenocortical tumours associated with the germline TP53 R337H mutation. 61 6 54
16033918 2006
4
A novel mechanism of tumorigenesis involving pH-dependent destabilization of a mutant p53 tetramer. 61 54 6
11753428 2002
5
Constitutive beta-catenin activation induces adrenal hyperplasia and promotes adrenal cancer development. 61 56
20106872 2010
6
An inherited p53 mutation that contributes in a tissue-specific manner to pediatric adrenal cortical carcinoma. 6 54
11481490 2001
7
Molecular definition of the 11p15.5 region involved in Beckwith-Wiedemann syndrome and probably in predisposition to adrenocortical carcinoma. 56 61
2921038 1989
8
Deletion mapping of chromosome 17 in benign and malignant adrenocortical tumors associated with the Arg337His mutation of the p53 tumor suppressor protein. 6
15741269 2005
9
Germline TP53 mutations and Li-Fraumeni syndrome. 56
12619118 2003
10
An inherited mutation outside the highly conserved DNA-binding domain of the p53 tumor suppressor protein in children and adults with sporadic adrenocortical tumors. 6
11600572 2001
11
Comparative genomic hybridization analysis of adrenocortical tumors of childhood. 56
10084604 1999
12
Structural and functional abnormalities at 11p15 are associated with the malignant phenotype in sporadic adrenocortical tumors: study on a series of 82 tumors. 56
9253334 1997
13
Virilizing adrenal carcinoma in two sibs. 56
5096538 1971
14
Adrenocortical neoplasms with hemihypertrophy, brain tumors, and other disorders. 56
6016796 1967
15
Li-Fraumeni syndrome in a Turkish family. 61 54
20426520 2010
16
Safety, tolerability, and pharmacokinetics of the anti-IGF-1R monoclonal antibody figitumumab in patients with refractory adrenocortical carcinoma. 61 54
19649631 2010
17
Identification of a novel germ-line mutation in the TP53 gene in a Mexican family with Li-Fraumeni syndrome. 61 54
20017945 2009
18
miR-195 and miR-483-5p Identified as Predictors of Poor Prognosis in Adrenocortical Cancer. 46
19996210 2009
19
Integrative molecular bioinformatics study of human adrenocortical tumors: microRNA, tissue-specific target prediction, and pathway analysis. 46
19546168 2009
20
Inhibition of adrenocortical carcinoma cell proliferation by steroidogenic factor-1 inverse agonists. 61 54
19318454 2009
21
[Identification of a novel TP53 germline mutation in one child with adrenocortical carcinoma]. 54 61
19671334 2009
22
Nestin as a marker in the classification of adrenocortical tumors. 61 54
19294612 2009
23
Beyond Li Fraumeni Syndrome: clinical characteristics of families with p53 germline mutations. 54 61
19204208 2009
24
Estrogen biosynthesis in human H295 adrenocortical carcinoma cells. 61 54
19026713 2009
25
Preclinical targeting of the type I insulin-like growth factor receptor in adrenocortical carcinoma. 54 61
18854392 2009
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. 61 54
19714490 2009
27
Germline p53 mutation in a Micronesian child with adrenocortical carcinoma and subsequent osteosarcoma. 54 61
18989156 2008
28
Development of an adrenocorticotropin-responsive human adrenocortical carcinoma cell line. 61 54
18713819 2008
29
Expression of insulin-like growth factor-II and its receptor in pediatric and adult adrenocortical tumors. 54 61
18611974 2008
30
Effects of lactone derivatives on aromatase (CYP19) activity in H295R human adrenocortical and (anti)androgenicity in transfected LNCaP human prostate cancer cells. 54 61
18639541 2008
31
Effects of fifteen PBDE metabolites, DE71, DE79 and TBBPA on steroidogenesis in the H295R cell line. 61 54
18313098 2008
32
The role of immunohistochemistry in histopathological diagnostics of clinically "silent" incidentally detected adrenal masses. 61 54
18393131 2008
33
Expression of aromatase and estrogen receptors in human adrenocortical tumors. 61 54
18157729 2008
34
Inhibition of human placental aromatase activity by hydroxylated polybrominated diphenyl ethers (OH-PBDEs). 54 61
18022659 2008
35
Usefulness of transcription factors Ad4BP/SF-1 and DAX-1 as immunohistologic markers for diagnosis of advanced adrenocortical carcinoma. 61 54
18824868 2008
36
Nephroblastoma overexpressed/cysteine-rich protein 61/connective tissue growth factor/nephroblastoma overexpressed gene-3 (NOV/CCN3), a selective adrenocortical cell proapoptotic factor, is down-regulated in childhood adrenocortical tumors. 61 54
17566092 2007
37
Mono-(2-ethylhexyl) phthalate (MEHP) induces nuclear receptor 4A subfamily in NCI-H295R cells: a possible mechanism of aromatase suppression by MEHP. 61 54
17574328 2007
38
[Effect of insulin-like growth factor and its receptor-I antibody on growth of human adrenocortical carcinoma SW-13 cell lines in vitro]. 54 61
17259156 2007
39
In vitro effects of brominated flame retardants and metabolites on CYP17 catalytic activity: a novel mechanism of action? 54 61
16828825 2006
40
Effect of epidermal growth factor and prostaglandin on the expression of aromatase (CYP19) in human adrenocortical carcinoma cell line NCI-H295R cells. 61 54
16394175 2006
41
Inhibition and induction of aromatase (CYP19) activity by brominated flame retardants in H295R human adrenocortical carcinoma cells. 54 61
16177243 2005
42
Effects of bisphenol A-related diphenylalkanes on vitellogenin production in male carp (Cyprinus carpio) hepatocytes and aromatase (CYP19) activity in human H295R adrenocortical carcinoma cells. 54 61
15907334 2005
43
Disruption of an intermonomer salt bridge in the p53 tetramerization domain results in an increased propensity to form amyloid fibrils. 61 54
16260757 2005
44
Clinical and molecular evidence for DAX-1 inhibition of steroidogenic factor-1-dependent ACTH receptor gene expression. 61 54
15879363 2005
45
TP53 mutations as biomarkers for cancer epidemiology in Latin America: current knowledge and perspectives. 61 54
15878142 2005
46
Inhibition of aromatase activity by methyl sulfonyl PCB metabolites in primary culture of human mammary fibroblasts. 54 61
15589976 2005
47
Induction and inhibition of aromatase (CYP19) activity by natural and synthetic flavonoid compounds in H295R human adrenocortical carcinoma cells. 54 61
15319488 2004
48
Rhabdomyosarcoma, osteosarcoma, and adrenocortical carcinoma in a child with a germline p53 mutation. 61 54
15390294 2004
49
Nur-related factor 1 and nerve growth factor-induced clone B in human adrenal cortex and its disorders. 61 54
15292355 2004
50
Role of the insulin-like growth factor system in adrenocortical growth control and carcinogenesis. 54 61
15241731 2004

Variations for Adrenocortical Carcinoma, Hereditary

ClinVar genetic disease variations for Adrenocortical Carcinoma, Hereditary:

6 (show top 50) (show all 78) ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎ ‎‎
# Gene Name Type Significance ClinVarId dbSNP ID GRCh37 Pos GRCh38 Pos
1 TP53 NM_000546.6(TP53):c.1010G>A (p.Arg337His)SNV Pathogenic 12379 rs121912664 17:7574017-7574017 17:7670699-7670699
2 TP53 NM_000546.6(TP53):c.743G>A (p.Arg248Gln)SNV Pathogenic 12356 rs11540652 17:7577538-7577538 17:7674220-7674220
3 TP53 NM_000546.6(TP53):c.818G>A (p.Arg273His)SNV Pathogenic 12366 rs28934576 17:7577120-7577120 17:7673802-7673802
4 TP53 NM_000546.6(TP53):c.524G>A (p.Arg175His)SNV Pathogenic 12374 rs28934578 17:7578406-7578406 17:7675088-7675088
5 TP53 NM_001126112.2(TP53):c.854A>T (p.Glu285Val)SNV Pathogenic 12384 rs121912667 17:7577084-7577084 17:7673766-7673766
6 PIK3CA NM_006218.4(PIK3CA):c.3140A>G (p.His1047Arg)SNV Pathogenic 13652 rs121913279 3:178952085-178952085 3:179234297-179234297
7 PIK3CA NM_006218.4(PIK3CA):c.3140A>T (p.His1047Leu)SNV Pathogenic 13653 rs121913279 3:178952085-178952085 3:179234297-179234297
8 NRAS NM_002524.5(NRAS):c.182A>G (p.Gln61Arg)SNV Pathogenic 13900 rs11554290 1:115256529-115256529 1:114713908-114713908
9 BRAF NM_001374258.1(BRAF):c.1901A>G (p.Asp634Gly)SNV Pathogenic 13972 rs121913338 7:140453154-140453154 7:140753354-140753354
10 PIK3CA NM_006218.4(PIK3CA):c.3139C>T (p.His1047Tyr)SNV Pathogenic 39705 rs121913281 3:178952084-178952084 3:179234296-179234296
11 TP53 NM_000546.6(TP53):c.638G>A (p.Arg213Gln)SNV Pathogenic 135359 rs587778720 17:7578211-7578211 17:7674893-7674893
12 TP53 NM_001126112.2(TP53):c.517G>A (p.Val173Met)SNV Pathogenic 233951 rs876660754 17:7578413-7578413 17:7675095-7675095
13 NRAS NM_002524.5(NRAS):c.182A>C (p.Gln61Pro)SNV Pathogenic 280409 rs11554290 1:115256529-115256529 1:114713908-114713908
14 NRAS NM_002524.5(NRAS):c.183A>T (p.Gln61His)SNV Pathogenic 373003 rs121913255 1:115256528-115256528 1:114713907-114713907
15 TP53 NM_001126112.2(TP53):c.374C>A (p.Thr125Lys)SNV Pathogenic 216465 rs786201057 17:7579313-7579313 17:7675995-7675995
16 TP53 NM_001126112.2(TP53):c.818G>T (p.Arg273Leu)SNV Pathogenic 376655 rs28934576 17:7577120-7577120 17:7673802-7673802
17 TP53 NM_001126112.2(TP53):c.517G>T (p.Val173Leu)SNV Pathogenic 376668 rs876660754 17:7578413-7578413 17:7675095-7675095
18 TP53 NM_001126112.2(TP53):c.817C>A (p.Arg273Ser)SNV Pathogenic/Likely pathogenic 376656 rs121913343 17:7577121-7577121 17:7673803-7673803
19 TP53 NM_001126112.2(TP53):c.700T>C (p.Tyr234His)SNV Pathogenic/Likely pathogenic 376691 rs864622237 17:7577581-7577581 17:7674263-7674263
20 TP53 NM_001126112.2(TP53):c.374C>G (p.Thr125Arg)SNV Pathogenic/Likely pathogenic 376667 rs786201057 17:7579313-7579313 17:7675995-7675995
21 NRAS NM_002524.5(NRAS):c.182A>T (p.Gln61Leu)SNV Pathogenic/Likely pathogenic 375874 rs11554290 1:115256529-115256529 1:114713908-114713908
22 CTNNB1 NM_001904.4(CTNNB1):c.134C>A (p.Ser45Tyr)SNV Pathogenic/Likely pathogenic 375895 rs121913409 3:41266137-41266137 3:41224646-41224646
23 BRAF NM_004333.6(BRAF):c.1780G>C (p.Asp594His)SNV Pathogenic/Likely pathogenic 375947 rs397516896 7:140453155-140453155 7:140753355-140753355
24 TP53 NM_001126112.2(TP53):c.818G>C (p.Arg273Pro)SNV Pathogenic/Likely pathogenic 231060 rs28934576 17:7577120-7577120 17:7673802-7673802
25 TP53 NM_001126112.2(TP53):c.638G>C (p.Arg213Pro)SNV Pathogenic/Likely pathogenic 231214 rs587778720 17:7578211-7578211 17:7674893-7674893
26 TP53 NM_001126112.2(TP53):c.799C>T (p.Arg267Trp)SNV Pathogenic/Likely pathogenic 141764 rs55832599 17:7577139-7577139 17:7673821-7673821
27 GNAS NM_001077488.4(GNAS):c.605G>T (p.Arg202Leu)SNV Pathogenic/Likely pathogenic 210045 rs121913495 20:57484421-57484421 20:58909366-58909366
28 TP53 NM_001126112.2(TP53):c.824G>A (p.Cys275Tyr)SNV Pathogenic/Likely pathogenic 215997 rs863224451 17:7577114-7577114 17:7673796-7673796
29 TP53 NM_001126112.2(TP53):c.701A>G (p.Tyr234Cys)SNV Pathogenic/Likely pathogenic 127820 rs587780073 17:7577580-7577580 17:7674262-7674262
30 GNAS NM_001077488.4(GNAS):c.604C>T (p.Arg202Cys)SNV Pathogenic/Likely pathogenic 15933 rs11554273 20:57484420-57484420 20:58909365-58909365
31 GNAS NM_001077488.4(GNAS):c.605G>A (p.Arg202His)SNV Pathogenic/Likely pathogenic 15934 rs121913495 20:57484421-57484421 20:58909366-58909366
32 TP53 NM_001126112.2(TP53):c.559+1G>ASNV Pathogenic/Likely pathogenic 428908 rs1131691042 17:7578370-7578370 17:7675052-7675052
33 TP53 NM_001126112.2(TP53):c.96+1G>TSNV Pathogenic/Likely pathogenic 482212 rs1131691003 17:7579699-7579699 17:7676381-7676381
34 CTNNB1 NM_001904.4(CTNNB1):c.101G>A (p.Gly34Glu)SNV Pathogenic/Likely pathogenic 17584 rs28931589 3:41266104-41266104 3:41224613-41224613
35 CTNNB1 NM_001904.4(CTNNB1):c.122C>T (p.Thr41Ile)SNV Pathogenic/Likely pathogenic 17587 rs121913413 3:41266125-41266125 3:41224634-41224634
36 CTNNB1 NM_001904.4(CTNNB1):c.134C>T (p.Ser45Phe)SNV Pathogenic/Likely pathogenic 17588 rs121913409 3:41266137-41266137 3:41224646-41224646
37 CTNNB1 NM_001904.4(CTNNB1):c.133T>C (p.Ser45Pro)SNV Pathogenic/Likely pathogenic 17589 rs121913407 3:41266136-41266136 3:41224645-41224645
38 CHEK2 NM_007194.4(CHEK2):c.470T>C (p.Ile157Thr)SNV risk factor 5591 rs17879961 22:29121087-29121087 22:28725099-28725099
39 TP53 NM_001126112.2(TP53):c.470T>C (p.Val157Ala)SNV Likely pathogenic 428887 rs1131691023 17:7578460-7578460 17:7675142-7675142
40 BRAF NM_001374258.1(BRAF):c.1900G>A (p.Asp634Asn)SNV Likely pathogenic 44813 rs397516896 7:140453155-140453155 7:140753355-140753355
41 TP53 NM_001126112.2(TP53):c.403T>G (p.Cys135Gly)SNV Likely pathogenic 376563 rs1057519975 17:7578527-7578527 17:7675209-7675209
42 TP53 NM_001126112.2(TP53):c.824G>T (p.Cys275Phe)SNV Likely pathogenic 376582 rs863224451 17:7577114-7577114 17:7673796-7673796
43 TP53 NM_001126112.2(TP53):c.518T>C (p.Val173Ala)SNV Likely pathogenic 376017 rs1057519747 17:7578412-7578412 17:7675094-7675094
44 CTNNB1 NM_001904.4(CTNNB1):c.100G>C (p.Gly34Arg)SNV Likely pathogenic 376233 rs121913399 3:41266103-41266103 3:41224612-41224612
45 CTNNB1 NM_001904.4(CTNNB1):c.134C>G (p.Ser45Cys)SNV Likely pathogenic 376242 rs121913409 3:41266137-41266137 3:41224646-41224646
46 CTNNB1 NM_001904.4(CTNNB1):c.101G>C (p.Gly34Ala)SNV Likely pathogenic 376390 rs28931589 3:41266104-41266104 3:41224613-41224613
47 CTNNB1 NM_001904.4(CTNNB1):c.122C>A (p.Thr41Asn)SNV Likely pathogenic 376394 rs121913413 3:41266125-41266125 3:41224634-41224634
48 H3-3A NM_002107.6(H3-3A):c.100G>C (p.Gly34Arg)SNV Likely pathogenic 376434 rs1057519902 1:226252152-226252152 1:226064451-226064451
49 PIK3CA NM_006218.4(PIK3CA):c.3141T>G (p.His1047Gln)SNV Likely pathogenic 376481 rs1057519932 3:178952086-178952086 3:179234298-179234298
50 TP53 NM_001126112.2(TP53):c.404G>T (p.Cys135Phe)SNV Likely pathogenic 376559 rs587781991 17:7578526-7578526 17:7675208-7675208

Copy number variations for Adrenocortical Carcinoma, Hereditary from CNVD:

7 (show all 23)
# CNVD ID Chromosome Start End Type Gene Symbol CNVD Disease
1 39293 10 11285212 16864223 Deletion Adrenocortical carcinoma
2 41923 10 26030462 27568983 Deletion Adrenocortical carcinoma
3 69267 12 54496959 54887816 Amplification Adrenocortical carcinoma
4 105388 16 79195214 80590034 Amplification Adrenocortical carcinoma
5 105796 16 82774218 84680399 Amplification Adrenocortical carcinoma
6 106027 16 85171711 86599633 Amplification Adrenocortical carcinoma
7 106330 16 87455812 88254372 Amplification Adrenocortical carcinoma
8 110643 17 33805634 34663603 Deletion Adrenocortical carcinoma
9 125811 19 15352798 16118051 Deletion Adrenocortical carcinoma
10 129302 19 45108448 45718366 Deletion Adrenocortical carcinoma
11 130528 19 49537470 50323207 Deletion Adrenocortical carcinoma
12 130657 19 50358143 53057498 Deletion Adrenocortical carcinoma
13 131131 19 53210832 53917176 Deletion Adrenocortical carcinoma
14 169573 3 142987765 144031265 Deletion Adrenocortical carcinoma
15 176088 3 46534671 47516603 Deletion Adrenocortical carcinoma
16 176572 3 49687490 50219610 Deletion Adrenocortical carcinoma
17 177057 3 52432656 53876467 Deletion Adrenocortical carcinoma
18 216378 6 91152410 95641352 Deletion Adrenocortical carcinoma
19 222196 7 150275058 151470304 Amplification Adrenocortical carcinoma
20 235763 8 144753570 146201712 Deletion Adrenocortical carcinoma
21 237149 8 21408650 22539377 Deletion Adrenocortical carcinoma
22 238033 8 29147511 31607545 Deletion Adrenocortical carcinoma
23 239018 8 36481039 39128088 Deletion Adrenocortical carcinoma

Expression for Adrenocortical Carcinoma, Hereditary

Search GEO for disease gene expression data for Adrenocortical Carcinoma, Hereditary.

Pathways for Adrenocortical Carcinoma, Hereditary

Pathways related to Adrenocortical Carcinoma, Hereditary according to GeneCards Suite gene sharing:

(show all 37)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.11 TP53 PIK3CA NRAS GNAS CTNNB1 BRAF
2
Show member pathways
12.98 TP53 PIK3CA NRAS GNAS CTNNB1 BRAF
3
Show member pathways
12.82 TP53 PIK3CA NRAS CTNNB1 BRAF
4
Show member pathways
12.75 PRKAR1A NRAS GNAS CTNNB1 BRAF
5
Show member pathways
12.71 TP53 PIK3CA NRAS CTNNB1 BRAF
6 12.71 TP53 PIK3CA NRAS GNAS CTNNB1 BRAF
7
Show member pathways
12.65 PIK3CA NRAS GNAS CTNNB1 BRAF
8
Show member pathways
12.63 TP53 PIK3CA NRAS CTNNB1 BRAF
9
Show member pathways
12.59 TP53 PIK3CA NRAS GNAS BRAF
10 12.29 TP53 PIK3CA NRAS MIR483 MIR214 MIR195
11 12.28 TP53 PIK3CA NRAS CHEK2
12
Show member pathways
12.28 TP53 PIK3CA NRAS CTNNB1 CHEK2
13 12.27 PIK3CA MC2R GNAS BRAF
14
Show member pathways
12.21 PIK3CA NRAS CTNNB1 BRAF
15 12.11 TP53 PIK3CA NRAS CTNNB1 BRAF
16
Show member pathways
12.08 TP53 PIK3CA NRAS BRAF
17 12.06 TP53 PIK3CA NRAS CHEK2
18
Show member pathways
12.05 PIK3CA NRAS BRAF
19 12.04 TP53 CTNNB1 CHEK2 BRAF
20
Show member pathways
12.02 PRKAR1A PIK3CA NRAS GNAS
21
Show member pathways
12 STAR NRAS MC2R GNAS CYP17A1 CYP11A1
22 11.98 TP53 PRKAR1A GNAS CTNNB1 BRAF
23 11.91 TP53 PIK3CA NRAS CTNNB1
24 11.86 TP53 PIK3CA NRAS CTNNB1
25 11.82 TP53 NRAS CTNNB1 BRAF
26
Show member pathways
11.79 STAR CYP17A1 CYP11A1
27
Show member pathways
11.76 PIK3CA NRAS BRAF
28 11.69 TP53 NRAS BRAF
29 11.66 TP53 PIK3CA NRAS
30
Show member pathways
11.65 PRKAR1A PIK3CA GNAS CTNNB1 BRAF
31 11.64 PIK3CA GNAS BRAF
32 11.62 TP53 PIK3CA CHEK2
33 11.55 NRAS GNAS BRAF
34 11.32 STAR GNAS CYP11A1 CTNNB1 BRAF
35 11.27 STAR GNAS CYP17A1 CYP11A1
36 10.97 PIK3CA GNAS BRAF
37 10.28 TP53 CHEK2

GO Terms for Adrenocortical Carcinoma, Hereditary

Biological processes related to Adrenocortical Carcinoma, Hereditary according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.8 TP53 STAR MIR511 CTNNB1 BRAF
2 cellular response to drug GO:0035690 9.5 TP53 CHEK2 BRAF
3 positive regulation of connective tissue replacement GO:1905205 9.49 MIR214 MIR195
4 DNA damage response, signal transduction by p53 class mediator resulting in transcription of p21 class mediator GO:0006978 9.48 TP53 CHEK2
5 replicative senescence GO:0090399 9.46 TP53 CHEK2
6 C21-steroid hormone biosynthetic process GO:0006700 9.43 STAR CYP11A1
7 steroid biosynthetic process GO:0006694 9.43 STAR CYP17A1 CYP11A1
8 glucocorticoid biosynthetic process GO:0006704 9.37 CYP17A1 CYP11A1
9 response to drug GO:0042493 9.35 TP53 STAR IGFBP2 GNAS CTNNB1
10 gene silencing by miRNA GO:0035195 9.17 MIR511 MIR503 MIR483 MIR214 MIR195 MIR184
11 hair follicle placode formation GO:0060789 9.16 GNAS CTNNB1

Molecular functions related to Adrenocortical Carcinoma, Hereditary according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 mRNA binding involved in posttranscriptional gene silencing GO:1903231 9.02 MIR511 MIR503 MIR483 MIR214 MIR195

Sources for Adrenocortical Carcinoma, Hereditary

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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