a Head and Neck Cancer Medical Oncology 3 Unit . Fondazione IRCCS Istituto Nazionale dei Tumori di Milano , Milan , Italy.
b Pathology Department . Fondazione IRCCS Istituto Nazionale dei Tumori di Milano , Milan , Italy.
Expert Opin Investig Drugs. 2019 May;28(5):435-443. doi: 10.1080/13543784.2019.1598376. Epub 2019 Apr 4.
Salivary gland cancers (SGCs) are a rare and heterogeneous group of malignant tumors arising from either major or minor salivary glands. Among SGCs patients, adenoid cystic carcinoma (ACC) is the most frequent histotype and its genetic aberrations are well known even though they are generally uncommon. Non-ACC subtypes are rarer and more heterogeneous than ACC from a histological and genomic point of view. In non-ACC, some altered molecular pathways [e.g. BRAF or RET mutations, Androgen Receptor (AR)] are potentially targetable with specific drugs.
A literature search was performed to summarize the main druggable genomic aberrations involving non-ACC SGCs. An overview of the genomics of non-ACC salivary gland malignancies is discussed. We describe the pattern of potentially targetable genomic alterations in non-ACC salivary gland malignancies according to their frequency rather than to the single non-ACC histotype.
EXPERT OPINION/COMMENTARY: The genetic profiling through in-depth molecular analyses [e.g. Next-generation sequencing (NGS)] is advised in all patients affected by recurrent and/or metastatic non-ACC SGCs to find any potentially druggable target. Some histotypes may carry driving mutations that must be investigated and defined. For the rare cancers, access to a referral center is recommended to optimize the management of these patients.
唾液腺癌(SGCs)是一组罕见且异质性的恶性肿瘤,起源于大唾液腺或小唾液腺。在 SGCs 患者中,腺样囊性癌(ACC)是最常见的组织学类型,其遗传异常虽然不常见,但已被广泛研究。从组织学和基因组学的角度来看,非 ACC 亚型比 ACC 更为罕见和多样化。在非 ACC 中,一些改变的分子途径[例如 BRAF 或 RET 突变、雄激素受体(AR)]可以用特定的药物进行靶向治疗。
进行了文献检索,以总结涉及非 ACC SGCs 的主要可用药基因组异常。讨论了非 ACC 唾液腺癌的基因组学概述。我们根据潜在可靶向基因组改变的频率而不是单一的非 ACC 组织学类型来描述非 ACC 唾液腺癌中潜在可靶向的基因组改变模式。
专家意见/评论:建议对所有复发性和/或转移性非 ACC SGC 患者进行深入分子分析(例如下一代测序(NGS))的基因谱分析,以寻找任何潜在的可靶向治疗目标。某些组织学类型可能存在必须进行研究和定义的驱动突变。对于罕见癌症,建议患者到转诊中心就诊,以优化对这些患者的管理。