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Adrenocortical carcinoma, hereditary(ADCC)

MedGen UID:
348508
•Concept ID:
C1859972
•
Neoplastic Process
Synonym: ADCC
 
Gene (location): TP53 (17p13.1)
 
Monarch Initiative: MONDO:0008734
OMIM®: 202300

Definition

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). [from OMIM]

Clinical features

From HPO
Adrenal cortex carcinoma
MedGen UID:
104917
•Concept ID:
C0206686
•
Neoplastic Process
A malignant neoplasm of the adrenal cortex that may produce hormones such as cortisol, aldosterone, estrogen, or testosterone.
See: Feature record | Search on this feature
Choroid plexus carcinoma
MedGen UID:
96557
•Concept ID:
C0431109
•
Neoplastic Process
Intraventricular papillary neoplasm derived from choroid plexus epithelium. Plexus tumors are most common in the lateral and fourth ventricles; while 80% of lateral ventricle tumors present in children, fourth ventricle tumors are evenly distributed in all age groups. Clinically, choroid plexus tumors tend to cause hydrocephalus and increased intracranial pressure. Histologically, choroid plexus papillomas correspond to WHO grade I, choroid plexus carcinomas to WHO grade III.
See: Feature record | Search on this feature
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Recent clinical studies

Diagnosis

Ectopic, retroperitoneal adrenocortical carcinoma in the setting of Lynch syndrome.
Wright JP, Montgomery KW, Tierney J, Gilbert J, Solórzano CC, Idrees K
Fam Cancer 2018 Jul;17(3):381-385. doi: 10.1007/s10689-017-0042-6. PMID: 28940135Free PMC Article
Familial Adrenocortical Carcinoma in Association With Lynch Syndrome.
Challis BG, Kandasamy N, Powlson AS, Koulouri O, Annamalai AK, Happerfield L, Marker AJ, Arends MJ, Nik-Zainal S, Gurnell M
J Clin Endocrinol Metab 2016 Jun;101(6):2269-72. Epub 2016 May 4 doi: 10.1210/jc.2016-1460. PMID: 27144940Free PMC Article

Prognosis

Familial Adrenocortical Carcinoma in Association With Lynch Syndrome.
Challis BG, Kandasamy N, Powlson AS, Koulouri O, Annamalai AK, Happerfield L, Marker AJ, Arends MJ, Nik-Zainal S, Gurnell M
J Clin Endocrinol Metab 2016 Jun;101(6):2269-72. Epub 2016 May 4 doi: 10.1210/jc.2016-1460. PMID: 27144940Free PMC Article

Supplemental Content

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    Clinical resources

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    Related information

    • ClinVar
      Related medical variations
    • Gene
      Related information in NCBI Gene
    • GTR
      Related information in GTR
    • GTR(Clinical)
      Clinical tests in GTR
    • MeSH
      Related Medical Subject Headings
    • OMIM
      Related records in OMIM
    • OMIM(Genes)
      OMIM records containing genes associated with phenotypes registered in MedGen
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed
    • PubMed (OMIM)
      Related literature resources in PubMed

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