KEGG DISEASE: Adrenal carcinoma

DISEASE: Adrenal carcinoma

Entry

H00033                      Disease

Name

Adrenal carcinoma

Subgroup

Adrenocortical carcinoma, hereditary

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 in ICD-11 classification [BR:br08403]
02 Neoplasms
  Malignant neoplasms, except primary neoplasms 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

Pathway

hsa04925

Aldosterone synthesis and secretion

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]

Other DBs

ICD-11:

2D11

MeSH:

D000306

OMIM:

202300

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

Reference

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

Reference

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

Reference

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

» Japanese version

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   OMIM (1)   
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