KEGG   DISEASE: Adrenal carcinomaHelp
Entry
H00033                      Disease                                

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
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of endocrine organs
   H00033  Adrenal carcinoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Malignant neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
   Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
    Malignant neoplasms of endocrine glands
     2D11  Malignant neoplasms of adrenal gland
      H00033  Adrenal carcinoma
BRITE hierarchy
Network
N00290  Mutation-inactivated MEN1 to transcription
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]
Drug
Mitotane [DR:D00420]
Comment
ICD-O: 8370/3, Tumor type: Adrenal cortical carcinoma
Other DBs
ICD-11: 2D11
ICD-10: C74
MeSH: D000306
Reference
PMID:17934868 (description, tumor type)
  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
Reference
PMID:11281372 (description, gene, tumor type)
  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
Reference
PMID:12920217 (marker, tumor type)
  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
Reference
  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

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