Details of Disease

General Information of Disease (ID: DISFK7RF)

• Disease Name: Adrenal gland neoplasm
• Synonyms: adrenal/paraganglial tumour; ADRENALGLAND; adrenal/paraganglial tumor; adrenal neoplasm; adrenal tumor; neoplasm of the adrenal gland; tumour of adrenal gland; tumour of the adrenal gland; adrenal gland tumor; adrenal tumour; neoplasm of adrenal gland; tumor of the adrenal gland; adrenal neoplasms; adrenal gland tumour; tumor of adrenal gland; adrenal gland neoplasm (disease)
• Definition: A neoplasm (disease) that involves the adrenal gland.
• Disease Hierarchy:
  DISTBY9Z: Tumour
  DISFS818: Adrenal gland disorder
  DISGQU9D: Endocrine gland neoplasm
  DISFK7RF: Adrenal gland neoplasm
• Disease Identifiers:
  MONDO ID: MONDO_0021227
  MESH ID: D000310
  UMLS CUI: C0001624
  MedGen ID: 1352
  HPO ID: HP:0100631
  Orphanet ID: 100091
  SNOMED CT ID: 127021009

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 2 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Erlotinib	DMCMBHA	Approved	Small molecular drug	[1]
Pertuzumab	DMHJV0X	Approved	Monoclonal antibody	[2]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 19 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
ADM2	TTM642F	Strong	Altered Expression	[3]
AGTR1	TT8DBY3	Strong	Altered Expression	[4]
AVPR2	TTK8R02	Strong	Altered Expression	[5]
CDKN2A	TTFTWQ8	Strong	Biomarker	[6]
CGB3	TTUH273	Strong	Biomarker	[7]
CYP11B1	TTIQUX7	Strong	Altered Expression	[8]
CYP11B2	TT9MNE2	Strong	Altered Expression	[9]
FSHR	TTZFDBT	Strong	Altered Expression	[10]
GNRH1	TT0ID4A	Strong	Therapeutic	[11]
GNRHR	TT8R70G	Strong	Biomarker	[12]
HSD11B1	TTN7BL9	Strong	Genetic Variation	[13]
KCNJ5	TTEO25X	Strong	Biomarker	[14]
MC2R	TTPWFDX	Strong	Biomarker	[15]
PDE11A	TTTWC79	Strong	Genetic Variation	[16]
PRKACA	TT5U49F	Strong	Biomarker	[6]
SLC33A1	TTL69WB	Strong	Genetic Variation	[17]
SSTR1	TTIND6G	Strong	Altered Expression	[18]
STAR	TTEI40H	Strong	Genetic Variation	[19]
TSHR	TT6NYJA	Strong	Biomarker	[12]

This Disease Is Related to 12 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
AIP	OTDJ3OSV	Limited	Biomarker	[20]
GNAS	OTMH8BKJ	Limited	Biomarker	[6]
ATP2B3	OT9DIEOP	Strong	Genetic Variation	[21]
CDC42	OT5QBC5M	Strong	Biomarker	[6]
CYP2W1	OTXSB1AE	Strong	Altered Expression	[22]
MEN1	OTN6U6V0	Strong	Biomarker	[23]
PCP4	OTM1XXYX	Strong	Altered Expression	[24]
PDE8B	OT4217NK	Strong	Genetic Variation	[25]
PMCH	OT3D9SA4	Strong	Altered Expression	[26]
PRKACB	OT6RMDCE	Strong	Genetic Variation	[27]
RAMP3	OTX6XLLM	Strong	Altered Expression	[3]
RB1	OT9VMY7B	Strong	Biomarker	[6]

References

• [1] Erlotinib FDA Label
• [2] A standard database for drug repositioning. Sci Data. 2017 Mar 14;4:170029.
• [3] Expression of adrenomedullin 2/intermedin in human adrenal tumors and attached non-neoplastic adrenal tissues.J Endocrinol. 2008 Jul;198(1):175-83. doi: 10.1677/JOE-08-0103. Epub 2008 May 6.
• [4] Immunohistochemical detection of angiotensin receptors AT1 and AT2 in adrenal tumors.Folia Histochem Cytobiol. 2008;46(1):51-5. doi: 10.2478/v10042-008-0006-7.
• [5] Familial vasopressin-sensitive ACTH-independent macronodular adrenal hyperplasia (VPs-AIMAH): clinical studies of three kindreds.Clin Endocrinol (Oxf). 2009 Jun;70(6):883-91. doi: 10.1111/j.1365-2265.2008.03471.x. Epub 2008 Nov 5.
• [6] Recurrent activating mutation in PRKACA in cortisol-producing adrenal tumors.Nat Genet. 2014 Jun;46(6):613-7. doi: 10.1038/ng.2956. Epub 2014 Apr 20.
• [7] Adrenocorticotropin-independent Cushing's syndrome in pregnancy related to overexpression of adrenal luteinizing hormone/human chorionic gonadotropin receptors.J Endocrinol Invest. 2009 Apr;32(4):313-6. doi: 10.1007/BF03345718.
• [8] The product of the CYP11B2 gene is required for aldosterone biosynthesis in the human adrenal cortex.Mol Endocrinol. 1991 Oct;5(10):1513-22. doi: 10.1210/mend-5-10-1513.
• [9] Clinicopathological features of primary aldosteronism associated with subclinical Cushing's syndrome.Endocr J. 2011;58(7):543-51. doi: 10.1507/endocrj.k10e-402. Epub 2011 Apr 27.
• [10] Direct luteinizing hormone action triggers adrenocortical tumorigenesis in castrated mice transgenic for the murine inhibin alpha-subunit promoter/simian virus 40 T-antigen fusion gene.Mol Endocrinol. 1998 Jun;12(6):801-9. doi: 10.1210/mend.12.6.0117.
• [11] Hecate-CGbeta conjugate and gonadotropin suppression shows two distinct mechanisms of action in the treatment of adrenocortical tumors in transgenic mice expressing Simian Virus 40 T antigen under inhibin-alpha promoter. Endocr Relat Cancer. 2009 Jun;16(2):549-64. doi: 10.1677/ERC-08-0232. Epub 2009 Mar 4.
• [12] Screening for membrane hormone receptor expression in primary aldosteronism.Eur J Endocrinol. 2009 Mar;160(3):443-51. doi: 10.1530/EJE-08-0711. Epub 2009 Jan 8.
• [13] The 83,557insA variant of the gene coding 11-hydroxysteroid dehydrogenase type 1 enzyme associates with serum osteocalcin in patients with endogenous Cushing's syndrome.J Steroid Biochem Mol Biol. 2011 Jan;123(1-2):79-84. doi: 10.1016/j.jsbmb.2010.11.009. Epub 2010 Nov 24.
• [14] Prevalence, clinical, and molecular correlates of KCNJ5 mutations in primary aldosteronism.Hypertension. 2012 Mar;59(3):592-8. doi: 10.1161/HYPERTENSIONAHA.111.186478. Epub 2012 Jan 23.
• [15] Preliminary studies on the differential expression of adrenocorticotropic hormone receptor in adrenal adenomas.Endokrynol Pol. 2017;68(5):512-518. doi: 10.5603/EP.a2017.0047. Epub 2017 Sep 7.
• [16] Phosphodiesterase 11A (PDE11A) gene defects in patients with acth-independent macronodular adrenal hyperplasia (AIMAH): functional variants may contribute to genetic susceptibility of bilateral adrenal tumors.J Clin Endocrinol Metab. 2012 Nov;97(11):E2063-9. doi: 10.1210/jc.2012-2275. Epub 2012 Sep 20.
• [17] Absence of angiotensin II type 1 receptor gene mutations in human adrenal tumors.Eur J Endocrinol. 1997 Sep;137(3):262-6. doi: 10.1530/eje.0.1370262.
• [18] Somatostatin receptor expression in adrenocortical tumors and effect of a new somatostatin analog SOM230 on hormone secretion in vitro and in ex vivo adrenal cells.J Endocrinol Invest. 2011 Jun;34(6):e131-8. doi: 10.1007/BF03346721. Epub 2010 Oct 27.
• [19] Mutational analysis of StAR gene in adrenal tumors.Int J Cancer. 2002 Jan 20;97(3):357-60. doi: 10.1002/ijc.1604.
• [20] PURE ANDROGEN-PRODUCING ADRENAL TUMOR: CLINICAL FEATURES AND PATHOGENESIS.Endocr Pract. 2017 Apr 2;23(4):399-407. doi: 10.4158/EP161580.OR. Epub 2017 Jan 17.
• [21] A Novel Somatic Deletion Mutation of ATP2B3 in Aldosterone-Producing Adenoma.Endocr Pathol. 2015 Dec;26(4):328-33. doi: 10.1007/s12022-015-9400-9.
• [22] Tumor-specific expression of the novel cytochrome P450 enzyme, CYP2W1.Biochem Biophys Res Commun. 2006 Mar 10;341(2):451-8. doi: 10.1016/j.bbrc.2005.12.200. Epub 2006 Jan 11.
• [23] Differential expression of menin in various adrenal tumors. The role of menin in adrenal tumors.Cancer. 2001 Sep 15;92(6):1393-401. doi: 10.1002/1097-0142(20010915)92:6<1393::aid-cncr1462>3.0.co;2-4.
• [24] Purkinje Cell Protein 4 Expression Is Associated With DNA Methylation Status in Aldosterone-Producing Adenoma.J Clin Endocrinol Metab. 2018 Mar 1;103(3):965-971. doi: 10.1210/jc.2017-01996.
• [25] Identification of novel genetic variants in phosphodiesterase 8B (PDE8B), a cAMP-specific phosphodiesterase highly expressed in the adrenal cortex, in a cohort of patients with adrenal tumours.Clin Endocrinol (Oxf). 2012 Aug;77(2):195-9. doi: 10.1111/j.1365-2265.2012.04366.x.
• [26] Expression of melanin-concentrating hormone receptor messenger ribonucleic acid in tumor tissues of pheochromocytoma, ganglioneuroblastoma, and neuroblastoma.J Clin Endocrinol Metab. 2001 Jan;86(1):369-74. doi: 10.1210/jcem.86.1.7158.
• [27] Activating PRKACB somatic mutation in cortisol-producing adenomas. JCI Insight. 2018 Apr 19;3(8):e98296. doi: 10.1172/jci.insight.98296. eCollection 2018 Apr 19.