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2015 ; 6
(35
): 37117-34
Nephropedia Template TP
gab.com Text
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English Wikipedia
Adenoid cystic carcinoma: current therapy and potential therapeutic advances
based on genomic profiling
#MMPMID26359351
Chae YK
; Chung SY
; Davis AA
; Carneiro BA
; Chandra S
; Kaplan J
; Kalyan A
; Giles FJ
Oncotarget
2015[Nov]; 6
(35
): 37117-34
PMID26359351
show ga
Adenoid cystic carcinoma (ACC) is a rare cancer with high potential for
recurrence and metastasis. Efficacy of current treatment options, particularly
for advanced disease, is very limited. Recent whole genome and exome sequencing
has dramatically improved our understanding of ACC pathogenesis. A balanced
translocation resulting in the MYB-NFIB fusion gene appears to be a fundamental
signature of ACC. In addition, sequencing has identified a number of other driver
genes mutated in downstream pathways common to other well-studied cancers.
Overexpression of oncogenic proteins involved in cell growth, adhesion, cell
cycle regulation, and angiogenesis are also present in ACC. Collectively, studies
have identified genes and proteins for targeted, mechanism-based, therapies based
on tumor phenotypes, as opposed to nonspecific cytotoxic agents. In addition,
although few studies in ACC currently exist, immunotherapy may also hold promise.
Better genetic understanding will enable treatment with novel targeted agents and
initial exploration of immune-based therapies with the goal of improving outcomes
for patients with ACC.