Human Phenotype Ontology 
Grandparent Node:
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Abnormality of adrenal morphology (HP:0011732)help
Grandparent Node:
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Neoplasm of the endocrine system (HP:0100568)help
Parent Node:
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Neoplasm of the adrenal gland (HP:0100631)help
..Starting node
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Neoplasm of the adrenal cortex (HP:0100641)help
Term ID: 100641
Name: Neoplasm of the adrenal cortex
Synonym: Cortical adrenal neoplasia
Definition: The presence of a neoplasm of the adrenal cortex.
Comments:
Reference: HP:0100641
Genes and Diseases:
 
       Child Nodes:
........expandAdrenocortical carcinoma (HP:0006744) help
........expandAdrenocortical adenoma (HP:0008256) help
................... HP:0011745 Non-secretory adrenocortical adenoma
................... HP:0011746 Secretory adrenocortical adenoma

 Sister Nodes: 
..expandNeoplasm of the adrenal medulla (HP:0100642) help
InputHPO IDHPO termDistanceGeneGene id entrezHGNC IDDiseaseIdDiseaseNameFrequencyOnsetHGMD variantsClinVar variants
 
HPO disease - gene - phenotype typical associations:
 
HPO disease - gene - phenotype less frequent non-typical associations:
HP:0100641HP:0100641Neoplasm of the adrenal cortex0APC CL E G H324583OMIM:175100Adenomatous polyposis coli3179
HP:0100641HP:0100641Neoplasm of the adrenal cortex0APC CL E G H324583ORPHA:247806APC-related attenuated familial adenomatous polyposis3179
HP:0100641HP:0100641Neoplasm of the adrenal cortex0APC CL E G H324583ORPHA:79665Gardner syndrome3179
HP:0100641HP:0100641Neoplasm of the adrenal cortex0CACNA1S CL E G H7791397ORPHA:681Hypokalemic periodic paralysis247
HP:0100641HP:0100641Neoplasm of the adrenal cortex0CDKN1A CL E G H10261784ORPHA:652Multiple endocrine neoplasia type 12
HP:0100641HP:0100641Neoplasm of the adrenal cortex0CDKN1B CL E G H10271785ORPHA:652Multiple endocrine neoplasia type 1102
HP:0100641HP:0100641Neoplasm of the adrenal cortex0CDKN1B CL E G H10271785ORPHA:276152Multiple endocrine neoplasia type 4102
HP:0100641HP:0100641Neoplasm of the adrenal cortex0CDKN1C CL E G H10281786OMIM:130650Beckwith-Wiedemann syndrome114
HP:0100641HP:0100641Neoplasm of the adrenal cortex0CDKN2A CL E G H10291787ORPHA:1501Adrenocortical carcinoma289
HP:0100641HP:0100641Neoplasm of the adrenal cortex0CDKN2A CL E G H10291787ORPHA:524Li-Fraumeni syndrome289
HP:0100641HP:0100641Neoplasm of the adrenal cortex0CDKN2B CL E G H10301788ORPHA:652Multiple endocrine neoplasia type 11
HP:0100641HP:0100641Neoplasm of the adrenal cortex0CDKN2C CL E G H10311789ORPHA:652Multiple endocrine neoplasia type 1
HP:0100641HP:0100641Neoplasm of the adrenal cortex0CHEK2 CL E G H1120016627ORPHA:524Li-Fraumeni syndrome833
HP:0100641HP:0100641Neoplasm of the adrenal cortex0CLCN2 CL E G H11812020ORPHA:404Familial hyperaldosteronism type II44
HP:0100641HP:0100641Neoplasm of the adrenal cortex0CTNNB1 CL E G H14992514ORPHA:1501Adrenocortical carcinoma88
HP:0100641HP:0100641Neoplasm of the adrenal cortex0CYP11B1 CL E G H15842591ORPHA:403Familial hyperaldosteronism type I112
HP:0100641HP:0100641Neoplasm of the adrenal cortex0CYP11B2 CL E G H15852592ORPHA:403Familial hyperaldosteronism type I73
HP:0100641HP:0100641Neoplasm of the adrenal cortex0H19-ICR CL E G H105259599OMIM:130650Beckwith-Wiedemann syndrome
HP:0100641HP:0100641Neoplasm of the adrenal cortex0IDH1 CL E G H34175382ORPHA:163634Maffucci syndromeHP:0040283 - Occasional15
HP:0100641HP:0100641Neoplasm of the adrenal cortex0IDH2 CL E G H34185383ORPHA:163634Maffucci syndromeHP:0040283 - Occasional29
HP:0100641HP:0100641Neoplasm of the adrenal cortex0IGF2 CL E G H34815466OMIM:130650Beckwith-Wiedemann syndrome9
HP:0100641HP:0100641Neoplasm of the adrenal cortex0KCNE3 CL E G H100086243ORPHA:681Hypokalemic periodic paralysis73
HP:0100641HP:0100641Neoplasm of the adrenal cortex0KCNQ1 CL E G H37846294OMIM:130650Beckwith-Wiedemann syndrome730
HP:0100641HP:0100641Neoplasm of the adrenal cortex0KCNQ1OT1 CL E G H109846295OMIM:130650Beckwith-Wiedemann syndrome1
HP:0100641HP:0100641Neoplasm of the adrenal cortex0MDM2 CL E G H41936973ORPHA:524Li-Fraumeni syndrome1
HP:0100641HP:0100641Neoplasm of the adrenal cortex0MEN1 CL E G H42217010OMIM:131100Multiple endocrine neoplasia 1462
HP:0100641HP:0100641Neoplasm of the adrenal cortex0MEN1 CL E G H42217010ORPHA:652Multiple endocrine neoplasia type 1462
HP:0100641HP:0100641Neoplasm of the adrenal cortex0PRKAR1A CL E G H55739388ORPHA:1501Adrenocortical carcinoma134
HP:0100641HP:0100641Neoplasm of the adrenal cortex0PTEN CL E G H57289588ORPHA:109Bannayan-Riley-Ruvalcaba syndromeHP:0040283 - Occasional948
HP:0100641HP:0100641Neoplasm of the adrenal cortex0SCN4A CL E G H632910591ORPHA:681Hypokalemic periodic paralysis263
HP:0100641HP:0100641Neoplasm of the adrenal cortex0TERT CL E G H701511730ORPHA:1501Adrenocortical carcinoma238
HP:0100641HP:0100641Neoplasm of the adrenal cortex0TP53 CL E G H715711998ORPHA:1501Adrenocortical carcinoma911
HP:0100641HP:0100641Neoplasm of the adrenal cortex0TP53 CL E G H715711998OMIM:202300Adrenocortical carcinoma, pediatric911
HP:0100641HP:0100641Neoplasm of the adrenal cortex0TP53 CL E G H715711998OMIM:151623Li-Fraumeni syndrome911
HP:0100641HP:0100641Neoplasm of the adrenal cortex0TP53 CL E G H715711998ORPHA:524Li-Fraumeni syndrome911
HP:0100641HP:0100641Neoplasm of the adrenal cortex0ZNRF3 CL E G H8413318126ORPHA:1501Adrenocortical carcinoma
HP:0100641HP:0008256Adrenocortical adenoma1APC CL E G H324583OMIM:175100Adenomatous polyposis coli3179
HP:0100641HP:0006744Adrenocortical carcinoma1APC CL E G H324583OMIM:175100Adenomatous polyposis coli.3179
HP:0100641HP:0008256Adrenocortical adenoma1APC CL E G H324583ORPHA:247806APC-related attenuated familial adenomatous polyposisHP:0040284 - Very rare3179
HP:0100641HP:0006744Adrenocortical carcinoma1APC CL E G H324583ORPHA:247806APC-related attenuated familial adenomatous polyposisHP:0040284 - Very rare3179
HP:0100641HP:0006744Adrenocortical carcinoma1APC CL E G H324583ORPHA:79665Gardner syndromeHP:0040284 - Very rare3179
HP:0100641HP:0008256Adrenocortical adenoma1APC CL E G H324583ORPHA:79665Gardner syndromeHP:0040283 - Occasional3179
HP:0100641HP:0008256Adrenocortical adenoma1CACNA1S CL E G H7791397ORPHA:681Hypokalemic periodic paralysisHP:0040284 - Very rare247
HP:0100641HP:0006744Adrenocortical carcinoma1CDKN1A CL E G H10261784ORPHA:652Multiple endocrine neoplasia type 1HP:0040283 - Occasional2
HP:0100641HP:0006744Adrenocortical carcinoma1CDKN1B CL E G H10271785ORPHA:652Multiple endocrine neoplasia type 1HP:0040283 - Occasional102
HP:0100641HP:0008256Adrenocortical adenoma1CDKN1B CL E G H10271785ORPHA:276152Multiple endocrine neoplasia type 4HP:0040282 - Frequent102
HP:0100641HP:0006744Adrenocortical carcinoma1CDKN1C CL E G H10281786OMIM:130650Beckwith-Wiedemann syndrome.114
HP:0100641HP:0006744Adrenocortical carcinoma1CDKN2A CL E G H10291787ORPHA:1501Adrenocortical carcinomaHP:0040280 - Obligate289
HP:0100641HP:0006744Adrenocortical carcinoma1CDKN2A CL E G H10291787ORPHA:524Li-Fraumeni syndromeHP:0040283 - Occasional289
HP:0100641HP:0006744Adrenocortical carcinoma1CDKN2B CL E G H10301788ORPHA:652Multiple endocrine neoplasia type 1HP:0040283 - Occasional1
HP:0100641HP:0006744Adrenocortical carcinoma1CDKN2C CL E G H10311789ORPHA:652Multiple endocrine neoplasia type 1HP:0040283 - Occasional
HP:0100641HP:0006744Adrenocortical carcinoma1CHEK2 CL E G H1120016627ORPHA:524Li-Fraumeni syndromeHP:0040283 - Occasional833
HP:0100641HP:0008256Adrenocortical adenoma1CLCN2 CL E G H11812020ORPHA:404Familial hyperaldosteronism type II44
HP:0100641HP:0006744Adrenocortical carcinoma1CTNNB1 CL E G H14992514ORPHA:1501Adrenocortical carcinomaHP:0040280 - Obligate88
HP:0100641HP:0008256Adrenocortical adenoma1CYP11B1 CL E G H15842591ORPHA:403Familial hyperaldosteronism type I112
HP:0100641HP:0008256Adrenocortical adenoma1CYP11B2 CL E G H15852592ORPHA:403Familial hyperaldosteronism type I73
HP:0100641HP:0006744Adrenocortical carcinoma1H19-ICR CL E G H105259599OMIM:130650Beckwith-Wiedemann syndrome.
HP:0100641HP:0006744Adrenocortical carcinoma1IGF2 CL E G H34815466OMIM:130650Beckwith-Wiedemann syndrome.9
HP:0100641HP:0008256Adrenocortical adenoma1KCNE3 CL E G H100086243ORPHA:681Hypokalemic periodic paralysisHP:0040284 - Very rare73
HP:0100641HP:0006744Adrenocortical carcinoma1KCNQ1 CL E G H37846294OMIM:130650Beckwith-Wiedemann syndrome.730
HP:0100641HP:0006744Adrenocortical carcinoma1KCNQ1OT1 CL E G H109846295OMIM:130650Beckwith-Wiedemann syndrome.1
HP:0100641HP:0006744Adrenocortical carcinoma1MDM2 CL E G H41936973ORPHA:524Li-Fraumeni syndromeHP:0040283 - Occasional1
HP:0100641HP:0008256Adrenocortical adenoma1MEN1 CL E G H42217010OMIM:131100Multiple endocrine neoplasia 1462
HP:0100641HP:0006744Adrenocortical carcinoma1MEN1 CL E G H42217010ORPHA:652Multiple endocrine neoplasia type 1HP:0040283 - Occasional462
HP:0100641HP:0006744Adrenocortical carcinoma1PRKAR1A CL E G H55739388ORPHA:1501Adrenocortical carcinomaHP:0040280 - Obligate134
HP:0100641HP:0008256Adrenocortical adenoma1SCN4A CL E G H632910591ORPHA:681Hypokalemic periodic paralysisHP:0040284 - Very rare263
HP:0100641HP:0006744Adrenocortical carcinoma1TERT CL E G H701511730ORPHA:1501Adrenocortical carcinomaHP:0040280 - Obligate238
HP:0100641HP:0006744Adrenocortical carcinoma1TP53 CL E G H715711998ORPHA:1501Adrenocortical carcinomaHP:0040280 - Obligate911
HP:0100641HP:0006744Adrenocortical carcinoma1TP53 CL E G H715711998OMIM:202300Adrenocortical carcinoma, pediatric.911
HP:0100641HP:0006744Adrenocortical carcinoma1TP53 CL E G H715711998ORPHA:524Li-Fraumeni syndromeHP:0040283 - Occasional911
HP:0100641HP:0006744Adrenocortical carcinoma1TP53 CL E G H715711998OMIM:151623Li-Fraumeni syndrome.911
HP:0100641HP:0006744Adrenocortical carcinoma1ZNRF3 CL E G H8413318126ORPHA:1501Adrenocortical carcinomaHP:0040280 - Obligate
HP:0100641HP:0011745Non-secretory adrenocortical adenoma2 CL E G H
HP:0100641HP:0011746Secretory adrenocortical adenoma2CLCN2 CL E G H11812020ORPHA:404Familial hyperaldosteronism type IIHP:0040282 - Frequent44
HP:0100641HP:0011746Secretory adrenocortical adenoma2CYP11B1 CL E G H15842591ORPHA:403Familial hyperaldosteronism type IHP:0040283 - Occasional112
HP:0100641HP:0011746Secretory adrenocortical adenoma2CYP11B2 CL E G H15852592ORPHA:403Familial hyperaldosteronism type IHP:0040283 - Occasional73


Genes (28) :APC CACNA1S CDKN1A CDKN1B CDKN1C CDKN2A CDKN2B CDKN2C CHEK2 CLCN2 CTNNB1 CYP11B1 CYP11B2 H19-ICR IDH1 IDH2 IGF2 KCNE3 KCNQ1 KCNQ1OT1 MDM2 MEN1 PRKAR1A PTEN SCN4A TERT TP53 ZNRF3

Diseases (16) :OMIM:175100 ORPHA:247806 ORPHA:79665 ORPHA:681 ORPHA:652 ORPHA:276152 OMIM:130650 ORPHA:1501 ORPHA:524 ORPHA:404 ORPHA:403 ORPHA:163634 OMIM:131100 ORPHA:109 OMIM:202300 OMIM:151623
 

Human Phenotype Ontology(HPO) is developed by the Human Phenotype Ontology Consortium. The version used here is December 15 2022 release.