Moskaluk Christopher A
Department of Pathology, University of Virginia Health System, Charlottesville, VA 22908, USA.
Head Neck Pathol. 2013 Mar;7(1):17-22. doi: 10.1007/s12105-013-0426-3. Epub 2013 Mar 5.
The clinical features and common molecular alterations of adenoid cystic carcinoma (ACC) are reviewed in this paper. ACC is an uncommon neoplasm that most frequently arises in salivary glands and related tissue in the head and neck region. ACC has distinct histologic features, with cribriform and tubular growth patterns of basaloid cells displaying a predominantly myoepithelial cellular phenotype. This neoplasm also has uncommon clinical features of rare regional lymph node metastasis and a prolonged but relentlessly progressive clinical course. Clinical outcome in ACC is correlated to histologic grade, which is correlated to the degree of aneuploidy and genetic alterations present in the tumor genomes. Recent studies have identified that the majority of ACC contain alterations of the MYB gene, usually resulting in a fusion gene product with the NFIB gene by a t(6;9) translocation event. The molecular consequences of this alteration are incompletely understood, as are secondary molecular alterations that contribute to the neoplastic phenotype of ACC.
本文综述了腺样囊性癌（ACC）的临床特征和常见分子改变。ACC是一种罕见肿瘤，最常发生于头颈部区域的唾液腺及相关组织。ACC具有独特的组织学特征，基底样细胞呈筛状和管状生长模式，主要表现为肌上皮细胞表型。该肿瘤还具有罕见区域淋巴结转移这一不常见临床特征，以及病程迁延但持续进展的特点。ACC的临床结局与组织学分级相关，而组织学分级又与肿瘤基因组中存在的非整倍体程度和基因改变相关。最近的研究发现，大多数ACC存在MYB基因改变，通常通过t(6;9)易位事件导致与NFIB基因形成融合基因产物。这种改变的分子后果尚未完全明确，导致ACC肿瘤表型的继发分子改变也是如此。
