| H00033 | |
| H番号 | H00033 |
| 名称 | 副腎癌 |
| 概要 | 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. |
| カテゴリ | がん |
| ネットワーク | - |
| 病因遺伝子 | 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] |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | ミトタン [DR:D00420] |
| コメント | - |
| リンク | ICD-11: 2D11 ICD-10: C74 MeSH: D000306 |
| 文献 | PMID:17934868 著者 Barlaskar FM, Hammer GD タイトル The molecular genetics of adrenocortical carcinoma. 雑誌 Rev Endocr Metab Disord 8:343-8 (2007) DOI:10.1007/s11154-007-9057-x PMID:11281372 著者 Reincke M, Beuschlein F, Slawik M, Borm K. タイトル Molecular adrenocortical tumourigenesis. 雑誌 Eur J Clin Invest 30 Suppl 3:63-8 (2000) DOI:10.1046/j.1365-2362.2000.0300s3063.x PMID:12920217 著者 Stojadinovic A, Brennan MF, Hoos A, Omeroglu A, Leung DH, Dudas ME, Nissan A, Cordon-Cardo C, Ghossein RA. タイトル Adrenocortical adenoma and carcinoma: histopathological and molecular comparative analysis. 雑誌 Mod Pathol 16:742-51 (2003) DOI:10.1097/01.MP.0000081730.72305.81 PMID:12466322 著者 Koch CA, Pacak K, Chrousos GP. タイトル The molecular pathogenesis of hereditary and sporadic adrenocortical and adrenomedullary tumors. 雑誌 J Clin Endocrinol Metab 87:5367-84 (2002) DOI:10.1210/jc.2002-021069 |