医学
腺样囊性癌
内科学
涎腺癌
头颈部癌
临床试验
背景(考古学)
疾病
肿瘤科
癌
癌症
生物
古生物学
作者
Luigi Lorini,Laura Ardighieri,Anna Bozzola,Chiara Romani,Eliana Bignotti,Michela Buglione,Andrea Emanuele Guerini,Davide Lombardi,Alberto Deganello,Michele Tomasoni,Sara Anna Bonini,Sandra Sigala,Davide Farina,Marco Ravanelli,Paolo Bossi
出处
期刊:Oral Oncology
[Elsevier]
日期:2021-02-09
卷期号:115: 105213-105213
被引量:46
标识
DOI:10.1016/j.oraloncology.2021.105213
摘要
Adenoid cystic carcinoma (ACC) is a rare tumor, usually arising in the salivary gland, accounting for 1% of all head and neck cancers. ACC may have a long-term poor prognosis, as about 40% of radically treated patients will recur locoregionally and up to 60% will develop distant metastasis. Factors influencing risk of recurrence have been well studied, but few data exist about prognostic factors in Recurrent/Metastatic (RM) setting. Moreover, treatment of RM ACC is often a challenge for clinicians, in the context of a rare disease, which may have an indolent clinical behavior or less frequently a quicker growth and with a paucity of available clinical trials. This review critically analyzes pathological and molecular prognostic factors in RM ACC and make an overview on actual therapeutic choices and future direction of therapy. Recognized prognostic factors in RM ACC are the presence and site of distant metastasis (lung vs other), the presence of nodal metastasis and of extranodal extension, skull base recurrence, disease free interval, lymphovascular invasion, solid histotypes and grading of disease, and the presence of mutation of NOTCH1 family, PI3K, and TP53. Due to disappointing results with chemotherapy, new approaches are under study, also on the basis of biomolecular research. Ongoing clinical trials are evaluating treatment targeting MYB and NOTCH1 alterations, immunotherapy or combination of targeted treatments and immune checkpoint inhibitors.
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