ADCC
MCID: ADR054
MIFTS: 45

Adrenocortical Carcinoma, Hereditary (ADCC)

Categories: Cancer diseases, Endocrine diseases, Genetic diseases, Rare diseases
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Aliases & Classifications for Adrenocortical Carcinoma, Hereditary

MalaCards integrated aliases for Adrenocortical Carcinoma, Hereditary:

Name: Adrenocortical Carcinoma, Hereditary 57 28 5 38 71
Adrenocortical Carcinoma, Pediatric 57 28 5 71
Adrenocortical Carcinoma 73 71
Adcc 57 73
Malignant Neoplasm of Adrenal Cortex 71
Hereditary Adrenocortical Carcinoma 73
Pediatric Adrenocortical Carcinoma 73
Tumors of Adrenal Cortex 71

Characteristics:


Inheritance:

Autosomal dominant 57

Classifications:



External Ids:

OMIM® 57 202300
MeSH 43 D018268
SNOMED-CT via HPO 69 2227007 255035007 88252006
UMLS 71 C0001618 C0206686 C0346402 more

Summaries for Adrenocortical Carcinoma, Hereditary

OMIM®: 57 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) (Updated 24-Oct-2022)

MalaCards based summary: Adrenocortical Carcinoma, Hereditary, also known as adrenocortical carcinoma, pediatric, is related to adrenal carcinoma and adrenal cortical adenocarcinoma, and has symptoms including fever and flank pain. An important gene associated with Adrenocortical Carcinoma, Hereditary is TP53 (Tumor Protein P53). The drugs Streptozocin and Picropodophyllin have been mentioned in the context of this disorder. Affiliated tissues include adrenal cortex, cortex and adrenal gland, and related phenotypes are adrenocortical carcinoma and choroid plexus carcinoma

UniProtKB/Swiss-Prot: 73 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 500)
# Related Disease Score Top Affiliating Genes
1 adrenal carcinoma 11.5
2 adrenal cortical adenocarcinoma 11.4
3 li-fraumeni syndrome 11.4
4 adrenocortical carcinoma with pure aldosterone hypersecretion 11.3
5 acth-independent cushing syndrome 11.1
6 adrenal gland disease 11.1
7 rare tumor 10.7
8 adenoma 10.7
9 primary agammaglobulinemia 10.7
10 adrenal adenoma 10.6
11 factor vii deficiency 10.5
12 hypokalemia 10.5
13 adrenal cortical adenoma 10.5
14 pheochromocytoma 10.5
15 premature ovarian failure 7 10.5
16 endocrine gland cancer 10.4
17 renal cell carcinoma, nonpapillary 10.4
18 gynecomastia 10.4
19 lynch syndrome 10.4
20 bap1 tumor predisposition syndrome 10.4
21 lynch syndrome i 10.4
22 collecting duct carcinoma 10.3
23 inherited cancer-predisposing syndrome 10.3
24 hyperandrogenism 10.3
25 amenorrhea 10.3
26 hypoglycemia 10.3
27 toe syndactyly, telecanthus, and anogenital and renal malformations 10.2
28 lymphoma, hodgkin, classic 10.2
29 human immunodeficiency virus type 1 10.2
30 viral infectious disease 10.2
31 beckwith-wiedemann syndrome 10.2
32 down syndrome 10.2
33 neuroendocrine tumor 10.2
34 acne 10.2
35 burkitt lymphoma 10.2
36 lymphoma 10.2
37 thrombophilia due to thrombin defect 10.2
38 camptodactyly-arthropathy-coxa vara-pericarditis syndrome 10.2
39 47 xxx syndrome 10.2
40 breast cancer 10.2
41 hemihyperplasia, isolated 10.1
42 mismatch repair cancer syndrome 1 10.1
43 endometrial cancer 10.1
44 immunodeficiency, common variable, 10 10.1
45 familial adenomatous polyposis 10.1
46 paraganglioma 10.1
47 cardiac arrest 10.1
48 central precocious puberty 10.1
49 acute kidney failure 10.1
50 49, xxxxy syndrome 10.1

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:

30
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 adrenocortical carcinoma 30 Very rare (1%) HP:0006744
2 choroid plexus carcinoma 30 Very rare (1%) HP:0030392

Symptoms via clinical synopsis from OMIM®:

57 (Updated 24-Oct-2022)
Neoplasia:
adrenocortical carcinoma

Endocrine Features:
virilism

Clinical features from OMIM®:

202300 (Updated 24-Oct-2022)

UMLS symptoms related to Adrenocortical Carcinoma, Hereditary:


fever; flank pain

Drugs & Therapeutics for Adrenocortical Carcinoma, Hereditary

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

(show top 50) (show all 106)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Streptozocin Approved, Investigational Phase 3 18883-66-4, 66395-18-4 29327
2
Picropodophyllin Approved, Investigational Phase 3 518-28-5, 477-47-4 10607 72435
3
Cisplatin Approved Phase 3 15663-27-1 2767 5702198 441203
4
Etoposide Approved Phase 3 33419-42-0 36462
5
Lenograstim Approved, Investigational Phase 3 135968-09-1
6
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
7 Mitogens Phase 3
8
Insulin Phase 3
9 Insulin, Globin Zinc Phase 3
10 Tubulin Modulators Phase 3
11 Antimitotic Agents Phase 3
12 Dermatologic Agents Phase 3
13 Keratolytic Agents Phase 3
14 Liposomal doxorubicin Phase 3
15
Etoposide phosphate Phase 3 16760419
16 Antibiotics, Antitubercular Phase 3
17 Anti-Bacterial Agents Phase 3
18
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
19
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239
20
Acetic acid Approved Phase 2 64-19-7 176
21
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
22
Gefitinib Approved, Investigational Phase 2 184475-35-2 123631
23
Sunitinib Approved, Investigational Phase 2 557795-19-4 5329102
24
Lactitol Approved, Investigational Phase 2 585-86-4, 585-88-6 157355 493591
25
Docetaxel Approved, Investigational Phase 2 114977-28-5 148124
26
Oxaliplatin Approved, Investigational Phase 2 61825-94-3 43805 11947679 6857599
27
Sodium citrate Approved, Investigational Phase 2 68-04-2 23431961
28
Tamoxifen Approved Phase 2 10540-29-1, 54965-24-1 2733526
29
Axitinib Approved, Investigational Phase 2 319460-85-0 6450551
30
Ipilimumab Approved Phase 2 477202-00-9
31
Mitotane Approved Phase 2 53-19-0 4211
32
Pembrolizumab Approved Phase 2 1374853-91-4 254741536
33
Metyrapone Approved, Investigational Phase 2 54-36-4 4174
34
Ketoconazole Approved, Investigational Phase 2 65277-42-1, 142128-57-2 47576 3823 638701
35
Lenvatinib Approved, Investigational Phase 2 417716-92-8 9823820
36
Nivolumab Approved Phase 2 946414-94-4
37
Hydrocortisone Approved, Vet_approved Phase 2 50-23-7 3640 5754
38
Hydrocortisone succinate Approved Phase 2 2203-97-6 3643
39
Hydrocortisone acetate Approved, Vet_approved Phase 2 50-03-3
40
Vitamin A Approved, Nutraceutical, Vet_approved Phase 2 2052-63-3, 22737-97-9, 22737-96-8, 68-26-8 9904001 9947823 5280382 445354
41
D-Tyrosine Approved, Experimental, Investigational, Nutraceutical Phase 2 133585-56-5, 556-02-5, 60-18-4 1153 6057
42
Citric acid Approved, Nutraceutical, Vet_approved Phase 2 77-92-9 311
43
Zuretinol acetate Investigational Phase 2 127-47-9, 29584-22-3 10245972
44
Gossypol Investigational Phase 2 303-45-7, 90141-22-3 3503
45
Rivoceranib Investigational Phase 2 811803-05-1 11315474
46
Suramin Investigational Phase 2 129-46-4, 145-63-1 5361
47 Albumin-Bound Paclitaxel Phase 2
48 Cytochrome P-450 Enzyme Inhibitors Phase 2
49
Abiraterone Acetate Phase 2 154229-18-2 9821849
50 Antineoplastic Agents, Hormonal Phase 2

Interventional clinical trials:

(show top 50) (show all 69)
# Name Status NCT ID Phase Drugs
1 Efficacy of Adjuvant Mitotane Treatment in Prolonging Recurrence-free Survival in Patients With Adrenocortical Carcinoma at Low-intermediate Risk of Recurrence Unknown status NCT00777244 Phase 3 MITOTANE
2 Mitotane With or Without Cisplatin and Etoposide After Surgery in Treating Participants With Stage I-III Adrenocortical Cancer With High Risk of Recurrence Unknown status NCT03723941 Phase 3 Cisplatin plus Etoposide
3 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
4 First International Randomized Trial in Locally Advanced and Metastatic Adrenocortical Carcinoma Treatment Completed NCT00094497 Phase 3 Etoposide;Doxorubicin;Cisplatin;Streptozotocin;Mitotane
5 A Randomized Registry Trial of Adjuvant Mitotane vs. Mitotane With Cisplatin/Etoposide After Primary Surgical Resection of Localized Adrenocortical Carcinoma With High Risk of Recurrence (ADIUVO-2 Trial) Recruiting NCT03583710 Phase 3 Cisplatin;Etoposide;Mitotane
6 Treatment of Adrenocortical Tumors With Surgery Plus Lymph Node Dissection and Multiagent Chemotherapy: A Groupwide Phase III Study Active, not recruiting NCT00304070 Phase 3 doxorubicin hydrochloride;cisplatin;mitotane;etoposide
7 Activity of Abiraterone Acetate in the Management of Cushing's Syndrome in Patients With Adrenocortical Carcinoma Unknown status NCT03145285 Phase 2 Abiraterone Acetate
8 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
9 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 Unknown status NCT03257891 Phase 2 Cabazitaxel
10 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
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 A Phase II Trial of ZD1839 (Iressa) in Patients With Nonresectable Adrenocortical Carcinoma (ACC) Completed NCT00215202 Phase 2 Iressa (ZD1839)
13 Sunitinib in Refractory Adrenocortical-Carcinoma Patients Progressing After Cytotoxic Chemotherapy Completed NCT00453895 Phase 2 Sunitinib
14 Phase II Clinical Trial of Dovitinib (TKI-258) in First-line Metastatic or Locally Advanced Non-resectable Adrenocortical Carcinoma Completed NCT01514526 Phase 2 Dovitinib
15 Phase II Trial of Surgical Resection and Heated Intraperitoneal Peritoneal Chemotherapy (HIPEC) for Adrenocortical Carcinoma Completed NCT01833832 Phase 2 Cisplatin;sodium thiosulfate
16 Phase II Trial With Taxotere and Cisplatin in Non-operable Adrenocortical Carcinoma Completed NCT00324012 Phase 2 cisplatin, taxotere
17 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
18 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
19 Evaluation of 123I-Iodometomidate for Adrenal Scintigraphy Completed NCT00454103 Phase 1, Phase 2 123I-Iodometomidate
20 A Phase II Study of Oxaliplatin in Children With Recurrent Solid Tumors Completed NCT00091182 Phase 2 oxaliplatin
21 Cisplatin, Doxorubicin and Tamoxifen in the Treatment of Incurable Soft Tissue and Endocrine Malignancies Completed NCT00002608 Phase 2 cisplatin;doxorubicin hydrochloride;tamoxifen citrate
22 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)
23 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
24 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
25 A Multicenter, Open-label, Phase II Study to Evaluate the Efficacy and Safety of Cabozantinib in Advanced (Unresectable or Metastatic) Adrenocortical Carcinoma Recruiting NCT03612232 Phase 2 Cabozantinib-s-malate
26 A Phase 1/2 Trial of a Novel Therapeutic Vaccine (EO2401) in Combination With Immune Check Point Blockade, for Treatment of Patients With Locally Advanced or Metastatic Adrenocortical Carcinoma, or Malignant Pheochromocytoma/Paraganglioma Recruiting NCT04187404 Phase 1, Phase 2
27 Phase II Trial of Surgical Resection and Heated Intraperitoneal Peritoneal Chemotherapy (HIPEC) for Adrenocortical Carcinoma Recruiting NCT03127774 Phase 2 Cisplatin;Sodium thiosulfate
28 A Phase II Study of Nivolumab Combined With Ipilimumab for Patients With Advanced Rare Genitourinary Tumors Recruiting NCT03333616 Phase 2 Ipilimumab;Nivolumab
29 DART: Dual Anti-CTLA-4 and Anti-PD-1 Blockade in Rare Tumors Recruiting NCT02834013 Phase 2
30 A Phase II Clinical Trial of Single Agent Pembrolizumab in Subjects With Advanced Adrenocortical Carcinoma Active, not recruiting NCT02673333 Phase 2 Pembrolizumab
31 A Phase II Study to Evaluate the Effects of Cabozantinib in Patients With Unresectable/Metastatic Adrenocortical Carcinoma Active, not recruiting NCT03370718 Phase 2 Cabozantinib
32 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 Active, not recruiting NCT02867592 Phase 2 Cabozantinib;Cabozantinib S-malate
33 Phase II Study for the Evaluation of Efficacy of Pembrolizumab (MK-3475) in Patients With Rare Tumors Active, not recruiting NCT02721732 Phase 2
34 Phase II Study for Combination of Camrelizumab and Apatinib in the Second-line Treatment of Recurrent or Metastatic Adrenocortical Carcinoma Not yet recruiting NCT04318730 Phase 2 Camrelizumab
35 A Multicentre, Open-label Phase 2 Study to Evaluate the Efficacy and Safety of Pembrolizumab in the Treatment of Advanced, Progressive Adrenocortical Carcinoma. Not yet recruiting NCT05563467 Phase 2 Pembrolizumab 25 MG/ML [Keytruda]
36 A Prospective Phase II Trial of Pembrolizumab Plus Lenvatinib in Advanced Adrenal Cortical Carcinoma After Failure of Platinum- and Mitotane-Based Chemotherapy Not yet recruiting NCT05036434 Phase 2 Pembrolizumab / Lenvatinib
37 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
38 Phase II Study of Nivolumab (Anti-PD-1 Antibody) for Treatment of Metastatic Adrenocortical Carcinoma Terminated NCT02720484 Phase 2 Nivolumab
39 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)
40 Evaluation of Suramin in Advanced Adrenal Cortical Carcinoma, Phase II Terminated NCT00002921 Phase 2 suramin;therapeutic hydrocortisone
41 A Phase II Study of Bevacizumab in Patients With Nonresectable Cancer of the Adrenal Cortex. Withdrawn NCT00469469 Phase 2 Bevacizumab
42 A Phase I Trial of Herceptin and Interleukin-12 Completed NCT00004074 Phase 1
43 Phase 1 Study of the Safety and Tolerability of ATR-101 in Adrenocortical Carcinoma Completed NCT01898715 Phase 1 ATR-101
44 A Phase I Trial of Suramin With Sequential Doxorubicin in Patients With Advanced Solid Tumors Completed NCT00003038 Phase 1 doxorubicin hydrochloride;suramin
45 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
46 A Phase I Study of Continuous Hyperthermic Peritoneal Perfusion (CHPP) With Escalating Doses of Cisplatin for Children With Peritoneal Carcinomatosis or Advanced Peritoneal and Retroperitoneal Disease Completed NCT00436657 Phase 1 CHPP of Cisplatin
47 A Phase 1b, Open-Label Study of Relacorilant in Combination With Pembrolizumab for Patients With Adrenocortical Carcinoma With Excess Glucocorticoid Production Recruiting NCT04373265 Phase 1 Relacorilant;Pembrolizumab
48 B7-H3-Specific Chimeric Antigen Receptor Autologous T-Cell Therapy for Pediatric Patients With Solid Tumors (3CAR) Recruiting NCT04897321 Phase 1 Fludarabine;Cyclophosphamide;MESNA;B7-H3 CAR T cells
49 A Phase 1/1b First-In-Human, Dose-Escalation Study to Evaluate the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of IPI-549 Monotherapy and in Combination With Nivolumab in Subjects With Advanced Solid Tumors Active, not recruiting NCT02637531 Phase 1 IPI-549 (eganelisib);Nivolumab
50 A Phase I/II Trial of IL-13-PE in Patients With Treatment Refractory Malignancies With a Focus on Metastatic and Locally Advanced Adrenocortical Carcinoma Terminated NCT01832974 Phase 1 IL-13-PE

Search NIH Clinical Center for Adrenocortical Carcinoma, Hereditary

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


Mitotane

Genetic Tests for Adrenocortical Carcinoma, Hereditary

Genetic tests related to Adrenocortical Carcinoma, Hereditary:

# Genetic test Affiliating Genes
1 Adrenocortical Carcinoma, Hereditary 28 TP53
2 Adrenocortical Carcinoma, Pediatric 28

Anatomical Context for Adrenocortical Carcinoma, Hereditary

Organs/tissues related to Adrenocortical Carcinoma, Hereditary:

MalaCards : Adrenal Cortex, Cortex, Adrenal Gland, T Cells, Nk Cells, Lymph Node, Salivary Gland

Publications for Adrenocortical Carcinoma, Hereditary

Articles related to Adrenocortical Carcinoma, Hereditary:

(show top 50) (show all 7890)
# 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. 62 57 5
18762572 2008
2
Inhibin alpha-subunit (INHA) gene and locus changes in paediatric adrenocortical tumours from TP53 R337H mutation heterozygote carriers. 57 5
15121773 2004
3
Constitutive beta-catenin activation induces adrenal hyperplasia and promotes adrenal cancer development. 62 57
20106872 2010
4
Penetrance of adrenocortical tumours associated with the germline TP53 R337H mutation. 62 5
16033918 2006
5
A novel mechanism of tumorigenesis involving pH-dependent destabilization of a mutant p53 tetramer. 62 5
11753428 2002
6
Molecular definition of the 11p15.5 region involved in Beckwith-Wiedemann syndrome and probably in predisposition to adrenocortical carcinoma. 62 57
2921038 1989
7
Deletion mapping of chromosome 17 in benign and malignant adrenocortical tumors associated with the Arg337His mutation of the p53 tumor suppressor protein. 5
15741269 2005
8
Germline TP53 mutations and Li-Fraumeni syndrome. 57
12619118 2003
9
An inherited mutation outside the highly conserved DNA-binding domain of the p53 tumor suppressor protein in children and adults with sporadic adrenocortical tumors. 5
11600572 2001
10
An inherited p53 mutation that contributes in a tissue-specific manner to pediatric adrenal cortical carcinoma. 5
11481490 2001
11
Comparative genomic hybridization analysis of adrenocortical tumors of childhood. 57
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. 57
9253334 1997
13
Virilizing adrenal carcinoma in two sibs. 57
5096538 1971
14
Adrenocortical neoplasms with hemihypertrophy, brain tumors, and other disorders. 57
6016796 1967
15
Vitamin D receptor activation is a feasible therapeutic target to impair adrenocortical tumorigenesis. 62
36049598 2022
16
Treatment Differences for Adrenocortical Carcinoma by Race and Insurance Status. 62
35987166 2022
17
Strategies to evaluate potential effector function of glycan variants: a case study of ordesekimab (AMG 714 or PRV-015). 62
36083248 2022
18
Minimally Invasive Surgery for Resectable Adrenocortical Carcinoma: A Nationwide Analysis. 62
35780533 2022
19
Altered expression of the IGF2‑H19 locus and mitochondrial respiratory complexes in adrenocortical carcinoma. 62
36169175 2022
20
ELANE neutropenia and solid tumors: Four cases from the French severe chronic neutropenia registry. 62
35969146 2022
21
Adrenal functional imaging - which marker for which indication? 62
36081395 2022
22
Adenoid Cystic Carcinoma of the Larynx: A SEER Database Review. 62
33170034 2022
23
Tafasitamab mediates killing of B-cell non-Hodgkin's lymphoma in combination with γδ T cell or allogeneic NK cell therapy. 62
35348812 2022
24
FcγRIIIA affinity chromatography complements conventional functional characterization of rituximab. 62
36181372 2022
25
AML-327 Novel Anti-B7-H3 Blocking Antibody Enhances NK Cell-Mediated Cytotoxicity and Improves Outcomes in AML-Bearing Mice. 62
36163810 2022
26
Enhanced neutralization potency of an identical HIV neutralizing antibody expressed as different isotypes is achieved through genetically distinct mechanisms. 62
36182959 2022
27
Elotuzumab, a potential therapeutic humanized anti-SLAMF7 monoclonal antibody, enhances natural killer cell-mediated killing of primary effusion lymphoma cells. 62
35262781 2022
28
The Immunocytokine FAP-IL-2v Enhances Anti-Neuroblastoma Efficacy of the Anti-GD2 Antibody Dinutuximab Beta. 62
36230765 2022
29
Inhibitory receptors for HLA class I as immune checkpoints for natural killer cell-mediated antibody-dependent cellular cytotoxicity in cancer immunotherapy. 62
36261539 2022
30
Enhancement of effector functions of anti-CD20 monoclonal antibody by increased afucosylation in CHO cell line through cell culture medium optimization. 62
36194313 2022
31
Improved control of SARS-CoV-2 by treatment with nucleocapsid-specific monoclonal antibody. 62
36219482 2022
32
Generation of CD16A gene knockout human embryonic stem cell line using CRISPR/Cas9. 62
36219983 2022
33
Repurposing the Pentameric B-Subunit of Shiga Toxin for Gb3-Targeted Immunotherapy of Colorectal Cancer by Rhamnose Conjugation. 62
35905973 2022
34
Connectedness and spillover effects of US climate policy uncertainty on energy stock, alternative energy stock, and carbon future. 62
36223011 2022
35
Laparoscopic approach of pediatric adrenal tumors. 62
35876904 2022
36
Imaging of pediatric adrenal tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper. 62
36193741 2022
37
Tumour size in adrenal tumours: its importance in the indication of adrenalectomy and in surgical outcomes-a single-centre experience. 62
35748977 2022
38
American Association of Endocrine Surgeons Guidelines for Adrenalectomy: Executive Summary. 62
35976622 2022
39
Simultaneous Detection of 2 Types of Malignancies in a Pediatric Patient on FDG PET/CT Led to Diagnosis of Li-Fraumeni Syndrome. 62
35543639 2022
40
IRF8 regulates efficacy of therapeutic anti-CD20 monoclonal antibodies. 62
36030374 2022
41
Cartilage oligomeric matrix protein acts as a molecular biomarker in multiple cancer types. 62
36255654 2022
42
Surgical outcomes and survival of adrenocortical carcinoma between children and adults. 62
35688671 2022
43
Sexually dimorphic activation of innate antitumor immunity prevents adrenocortical carcinoma development. 62
36240276 2022
44
Association of tumor size and surgical approach with oncological outcomes and overall survival in patients with adrenocortical carcinoma. 62
35725937 2022
45
The role of molecular profiling in adrenocortical carcinoma. 62
34750847 2022
46
Differentiating between adrenocortical carcinoma and lipid-poor cortical adenoma: A novel cross-sectional imaging-based score. 62
36244817 2022
47
Metastatic oncocytic adrenocortical carcinoma of mandible: an extraordinarily rare occurrence. 62
35989231 2022
48
CDK1 serves as a therapeutic target of adrenocortical carcinoma via regulating epithelial-mesenchymal transition, G2/M phase transition, and PANoptosis. 62
36184616 2022
49
Multi-genomic analysis of 260 adrenocortical cancer patient tumors identifies novel network BIRC5-hsa-miR-335-5p-PAX8-AS1 strongly associated with poor survival. 62
36202651 2022
50
Association of Surgical Approach With Treatment Burden, Oncological Effectiveness, and Perioperative Morbidity in Adrenocortical Carcinoma. 62
35618598 2022

Variations for Adrenocortical Carcinoma, Hereditary

ClinVar genetic disease variations for Adrenocortical Carcinoma, Hereditary:

5 (show all 13)
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 TP53 NM_000546.6(TP53):c.72dup (p.Leu25fs) DUP Pathogenic
1341341 GRCh37: 17:7579840-7579841
GRCh38: 17:7676522-7676523
2 TP53 NM_000546.6(TP53):c.854A>T (p.Glu285Val) SNV Pathogenic
12384 rs121912667 GRCh37: 17:7577084-7577084
GRCh38: 17:7673766-7673766
3 TP53 NM_000546.6(TP53):c.559+1G>A SNV Pathogenic
428908 rs1131691042 GRCh37: 17:7578370-7578370
GRCh38: 17:7675052-7675052
4 TP53 NM_000546.6(TP53):c.96+1G>T SNV Pathogenic
482212 rs1131691003 GRCh37: 17:7579699-7579699
GRCh38: 17:7676381-7676381
5 TP53 NM_000546.6(TP53):c.524G>A (p.Arg175His) SNV Pathogenic
12374 rs28934578 GRCh37: 17:7578406-7578406
GRCh38: 17:7675088-7675088
6 TP53 NM_000546.6(TP53):c.1010G>A (p.Arg337His) SNV Pathogenic
Pathogenic
12379 rs121912664 GRCh37: 17:7574017-7574017
GRCh38: 17:7670699-7670699
7 TP53 NM_000546.6(TP53):c.818G>A (p.Arg273His) SNV Pathogenic
12366 rs28934576 GRCh37: 17:7577120-7577120
GRCh38: 17:7673802-7673802
8 TP53 NM_000546.6(TP53):c.743G>A (p.Arg248Gln) SNV Pathogenic
12356 rs11540652 GRCh37: 17:7577538-7577538
GRCh38: 17:7674220-7674220
9 TP53 NM_000546.6(TP53):c.799C>T (p.Arg267Trp) SNV Likely Pathogenic
141764 rs55832599 GRCh37: 17:7577139-7577139
GRCh38: 17:7673821-7673821
10 TP53 NM_000546.6(TP53):c.466C>T (p.Arg156Cys) SNV Uncertain Significance
127810 rs563378859 GRCh37: 17:7578464-7578464
GRCh38: 17:7675146-7675146
11 TP53 NM_000546.6(TP53):c.461G>A (p.Gly154Asp) SNV Uncertain Significance
237950 rs762846821 GRCh37: 17:7578469-7578469
GRCh38: 17:7675151-7675151
12 TP53 NM_000546.6(TP53):c.847C>T (p.Arg283Cys) SNV Uncertain Significance
127824 rs149633775 GRCh37: 17:7577091-7577091
GRCh38: 17:7673773-7673773
13 TP53 NM_000546.6(TP53):c.760A>G (p.Ile254Val) SNV Uncertain Significance
406605 rs746601313 GRCh37: 17:7577521-7577521
GRCh38: 17:7674203-7674203

Expression for Adrenocortical Carcinoma, Hereditary

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

Pathways for Adrenocortical Carcinoma, Hereditary

GO Terms for Adrenocortical Carcinoma, Hereditary

Sources for Adrenocortical Carcinoma, Hereditary

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
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36 LifeMap
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50 NDF-RT
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56 OMIM via Orphanet
57 OMIM® (Updated 24-Oct-2022)
61 PubChem
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64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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