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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.
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.

Recent clinical studies

Diagnosis

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
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

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

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