| H00033 | |
| H number | H00033 |
| Name | Adrenal carcinoma |
| Description | Adrenocortical carcinoma (ACC) is a rare endocrine malignancy defined by a heterogeneous clinical presentation, dismal prognosis, and lack of effective therapeutic regimens. The incidence of ACC ranges from 0.5 to 2 cases per million people per year, accounting for 0.02% of all reported cancers. Unfortunately, most patients present with metastatic disease which reduces the 5 year survival rate to less than 10%. Oncogenes and tumor-suppressor genes involved in adrenal carcinomas include mutations in the p53 tumor-suppressor gene and rearrangements of the chromosomal locus 11p15.5 associated with IGF II hyperexpression. Deletions of the ACTH receptor gene have recently been found in undifferentiated adenomas and in aggressive ACCs. |
| Category | Cancer |
| Network | - |
| Gene | ACTH-R (deletion) [HSA:4158] [KO:K04200] GNAI2 (mutation) [HSA:2771] [KO:K04630] N-ras (mutation) [HSA:4893] [KO:K07828] IGF II (overexpression) [HSA:3481] [KO:K13769] p53 (LOH, mutation) [HSA:7157] [KO:K04451] p16/INK4A (LOH, low expression) [HSA:1028] [KO:K09993] MEN1 (LOH, mutation) [HSA:4221] [KO:K14970] |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | Mitotane [DR:D00420] |
| Comment | - |
| Other DBs | ICD-11: 2D11 ICD-10: C74 MeSH: D000306 |
| Reference | PMID:17934868 AUTHORS Barlaskar FM, Hammer GD TITLE The molecular genetics of adrenocortical carcinoma. JOURNAL Rev Endocr Metab Disord 8:343-8 (2007) DOI:10.1007/s11154-007-9057-x PMID:11281372 AUTHORS Reincke M, Beuschlein F, Slawik M, Borm K. TITLE Molecular adrenocortical tumourigenesis. JOURNAL Eur J Clin Invest 30 Suppl 3:63-8 (2000) DOI:10.1046/j.1365-2362.2000.0300s3063.x PMID:12920217 AUTHORS Stojadinovic A, Brennan MF, Hoos A, Omeroglu A, Leung DH, Dudas ME, Nissan A, Cordon-Cardo C, Ghossein RA. TITLE Adrenocortical adenoma and carcinoma: histopathological and molecular comparative analysis. JOURNAL Mod Pathol 16:742-51 (2003) DOI:10.1097/01.MP.0000081730.72305.81 PMID:12466322 AUTHORS Koch CA, Pacak K, Chrousos GP. TITLE The molecular pathogenesis of hereditary and sporadic adrenocortical and adrenomedullary tumors. JOURNAL J Clin Endocrinol Metab 87:5367-84 (2002) DOI:10.1210/jc.2002-021069 |