Details of Disease

General Information of Disease (ID: DISZF4HX)

• Disease Name: Adrenocortical carcinoma
• Synonyms: adrenal cortical tumours; adrenal cortex cancer; adrenocortical cancer; cancer of the adrenal cortex; malignant tumour of adrenal cortex; adrenal cortical tumors; malignant tumor of adrenal cortex; malignant adrenocortical tumour; malignant adrenocortical tumor; malignant neoplasm of adrenal cortex; adrenal cortex carcinoma; adrenocortical carcinoma (disease); adrenal cortex adenocarcinoma; adrenal cortical carcinoma; Adenoid Cystic Carcinoma; carcinoma of adrenal cortex; cortical cell carcinoma; adenocarcinoma, adrenocortical, malignant; ACC; adrenocortical carcinoma; adrenal cortical carcinoma (morphologic abnormality); carcinoma, adrenocortical, malignant; carcinoma of the adrenal cortex; adrenal cortical adenocarcinoma
• Disease Class: 2D11: Adrenal cancer
• Definition: A rare, usually large (greater than 5cm), malignant epithelial tumor arising from the adrenal cortical cells. Symptoms are usually related to the excessive production of hormones, and include Cushing's syndrome and virilism in women. Common sites of metastasis include liver, lung, bone, and retroperitoneal lymph nodes. Advanced radiologic procedures have enabled the detection of small tumors, resulting in the improvement of the 5-year survival.|NCIT treats carcinoma and adenocarcinoma as synonyms.
• Disease Hierarchy:
  DISNSLVG: Vascular cancer
  DISQZ2X8: Renal cell carcinoma
  DIS53IXW: Adrenal carcinoma
  DIS7E0I8: Malignant tumor of adrenal cortex
  DISH9F1N: Carcinoma
  DISZF4HX: Adrenocortical carcinoma
• ICD Code:
  ICD-11: ICD-11: 2D11.Z
  ICD-9: ICD-9: 194
  Expand ICD-11: '2D11.Z
  Expand ICD-9: 194
• Disease Identifiers:
  MONDO ID: MONDO_0006639
  MESH ID: D018268
  UMLS CUI: C0206686
  MedGen ID: 104917
  HPO ID: HP:0006744
  Orphanet ID: 1501
  SNOMED CT ID: 2227007

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 1 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Mitotane	DMU1GX0	Approved	Small molecular drug	[1]

This Disease is Treated as An Indication in 3 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
IPI-549	DMG5Q7F	Phase 2	NA	[2]
EO2401	DMCN3UU	Phase 1/2	Vaccine	[3]
ATR-01	DMU67HD	Phase 1	NA	[4]

This Disease is Treated as An Indication in 1 Discontinued Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Tariquidar	DMJG7VP	Discontinued in Phase 2	Small molecular drug	[5]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 38 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
ATP1A1	TTWK8D0	Limited	Genetic Variation	[6]
CD22	TTM6QSK	Limited	Biomarker	[7]
CDKN1C	TTBSUAR	Limited	Genetic Variation	[8]
ENPP3	TTD4TKP	Limited	Biomarker	[7]
FSCN1	TTTRS9B	Limited	Biomarker	[9]
NNT	TTKIH76	Limited	Biomarker	[10]
PRKACA	TT5U49F	Limited	Genetic Variation	[6]
SPARC	TTBQFM7	Limited	Biomarker	[11]
CTNNB1	TTRPKQG	Disputed	Biomarker	[12]
BAP1	TT47RXJ	moderate	Biomarker	[13]
BRAF	TT0EOB8	moderate	Genetic Variation	[14]
TOP2A	TTCGY2K	moderate	Biomarker	[15]
ABCB1	TT3OT40	Strong	Biomarker	[16]
CACNA1H	TTZPWGN	Strong	Genetic Variation	[17]
CDKN2A	TTFTWQ8	Strong	Biomarker	[18]
CPA1	TT3LJ6G	Strong	Genetic Variation	[19]
CYP11B2	TT9MNE2	Strong	Altered Expression	[20]
DLK1	TTF4AVB	Strong	Biomarker	[21]
ENPEP	TT9PBIL	Strong	Biomarker	[22]
FOLH1	TT9G4N0	Strong	Biomarker	[23]
GHRHR	TTG4R8V	Strong	Biomarker	[24]
GIPR	TTYMKBE	Strong	Altered Expression	[25]
HTR7	TTO9X1H	Strong	Biomarker	[26]
IGF1R	TTQFBMY	Strong	Biomarker	[27]
IGFBP2	TTU4QSN	Strong	Altered Expression	[28]
KCNJ5	TTEO25X	Strong	Genetic Variation	[29]
LHCGR	TT2O4W9	Strong	Altered Expression	[30]
MAP3K1	TTW8TJI	Strong	Biomarker	[31]
MC2R	TTPWFDX	Strong	Altered Expression	[32]
NAGLU	TTDM6HZ	Strong	Altered Expression	[33]
NR0B1	TTTK36V	Strong	Altered Expression	[34]
NRAS	TTW2R9X	Strong	Genetic Variation	[14]
PDE11A	TTTWC79	Strong	Biomarker	[29]
PRKAR1A	TTNAHEX	Strong	Genetic Variation	[35]
RRM1	TTWP0NS	Strong	Biomarker	[36]
SLC25A1	TTTD730	Strong	Genetic Variation	[37]
SLC12A7	TTU2PCD	Definitive	Genetic Variation	[38]
TERT	TTQY2EJ	Definitive	Biomarker	[18]

This Disease Is Related to 5 DME Molecule(s)

Gene Name	DME ID	Evidence Level	Mode of Inheritance	REF
CYP4B1	DEMF740	Limited	Altered Expression	[39]
CHST3	DEQIZP2	Strong	Altered Expression	[40]
CYP2S1	DE6NMGO	Strong	Biomarker	[41]
CYP4F2	DE3GT9C	Strong	Biomarker	[41]
SULT2A1	DE0P6LK	Strong	Biomarker	[42]

This Disease Is Related to 48 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
ATP2B3	OT9DIEOP	Limited	Genetic Variation	[6]
BCL9	OTRBIPR4	Limited	Biomarker	[43]
CORO7	OTG7MEAJ	Limited	Biomarker	[44]
DCUN1D1	OT8UJLZU	Limited	Altered Expression	[45]
FATE1	OTPYXV64	Limited	Altered Expression	[46]
GEMIN2	OT4L6TLL	Limited	Biomarker	[47]
MCM3	OTOOHQPM	Limited	Biomarker	[48]
MED27	OT40PZ9R	Limited	Altered Expression	[49]
MRAP2	OTCQS7DB	Limited	Altered Expression	[50]
PMPCA	OT5X1G9Q	Limited	Altered Expression	[51]
RARRES2	OT1BJE8K	Limited	Biomarker	[52]
SCAF11	OTX59D0X	Limited	Biomarker	[47]
ACACA	OT5CQPZY	moderate	Biomarker	[53]
BMS1	OTEGQ8ZO	moderate	Biomarker	[53]
KRT5	OTVGI9HT	moderate	Biomarker	[54]
MEN1	OTN6U6V0	moderate	Biomarker	[18]
TCF21	OT393IMA	moderate	Altered Expression	[55]
AIP	OTDJ3OSV	Strong	Biomarker	[56]
ARMC5	OTO7IV74	Strong	Biomarker	[57]
BMP5	OTC0Y6E0	Strong	Altered Expression	[58]
CITED2	OT812TV7	Strong	Altered Expression	[59]
CYP2W1	OTXSB1AE	Strong	Altered Expression	[60]
GATA6	OTO2BC0F	Strong	Biomarker	[61]
GNAS	OTMH8BKJ	Strong	Genetic Variation	[14]
HOXC4	OT52VJ6V	Strong	Biomarker	[62]
JDP2	OTW35WKX	Strong	Altered Expression	[63]
MAP2K6	OTK13JKC	Strong	Biomarker	[31]
MATR3	OTESJ5S7	Strong	Altered Expression	[64]
MDH2	OT7364GY	Strong	Biomarker	[22]
MT3	OTVCZ7HI	Strong	Biomarker	[19]
NBAS	OTW9IBRI	Strong	Altered Expression	[33]
NDRG4	OTJBOTD8	Strong	Altered Expression	[65]
NPW	OTO7MT28	Strong	Biomarker	[66]
NR5A1	OTOULYR4	Strong	Altered Expression	[67]
PELP1	OTVXQNOT	Strong	Biomarker	[68]
PTTG1	OTIMYS4W	Strong	Biomarker	[69]
RASSF7	OT0V4EIZ	Strong	Genetic Variation	[70]
RNF31	OT4BZONL	Strong	Biomarker	[71]
SCN7A	OTK05PXY	Strong	Altered Expression	[33]
SCP2	OTPAFCPQ	Strong	Biomarker	[72]
SF1	OTLEDM2S	Strong	Biomarker	[71]
SH3KBP1	OTIUA60B	Strong	Biomarker	[73]
TARBP2	OT1QQ8H3	Strong	Altered Expression	[74]
TBX2	OTTOT7A9	Strong	Altered Expression	[75]
TFE3	OTM99ZWH	Strong	Biomarker	[76]
DAXX	OTX6O7PL	Definitive	Biomarker	[18]
MED12	OTQZ4D2X	Definitive	Biomarker	[18]
RB1	OT9VMY7B	Definitive	Biomarker	[18]

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