{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T16:23:48Z","timestamp":1791563028578,"version":"4.3.4"},"reference-count":104,"publisher":"MDPI AG","issue":"18","license":[{"start":{"date-parts":[[2023,9,6]],"date-time":"2023-09-06T00:00:00Z","timestamp":1693958400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Adenoid cystic carcinoma (ACC) has a worldwide incidence of three to four cases per million population. Although more cases occur in the minor and major salivary glands, it is the most common lacrimal gland malignancy. ACC has a low-grade, indolent histological appearance, but is relentlessly progressive over time and has a strong proclivity to recur and\/or metastasise. Current treatment options are limited to complete surgical excision and adjuvant radiotherapy. Intra-arterial systemic therapy is a recent innovation. Recurrent\/metastatic disease is common due to perineural invasion, and it is largely untreatable as it is refractory to conventional chemotherapeutic agents. Given the rarity of this tumour, the molecular mechanisms that govern disease pathogenesis are poorly understood. There is an unmet, critical need to develop effective, personalised targeted therapies for the treatment of ACC in order to reduce morbidity and mortality associated with the disease. This review details the evidence relating to the molecular underpinnings of ACC of the lacrimal gland, including the MYB\u2013NFIB chromosomal translocations, Notch-signalling pathway aberrations, DNA damage repair gene mutations and epigenetic modifications.<\/jats:p>","DOI":"10.3390\/ijms241813755","type":"journal-article","created":{"date-parts":[[2023,9,6]],"date-time":"2023-09-06T10:07:37Z","timestamp":1693994857000},"page":"13755","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["A Review of the Molecular Landscape of Adenoid Cystic Carcinoma of the Lacrimal Gland"],"prefix":"10.3390","volume":"24","author":[{"given":"Sarah Kate","family":"Powell","sequence":"first","affiliation":[{"name":"Research Foundation, Royal Victoria Eye and Ear Hospital, D02 XK51 Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-1289-084X","authenticated-orcid":false,"given":"Karina","family":"Kulakova","sequence":"additional","affiliation":[{"name":"Research Foundation, Royal Victoria Eye and Ear Hospital, D02 XK51 Dublin, Ireland"},{"name":"Department of Biotechnology, Dublin City University, D09 V209 Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan","family":"Kennedy","sequence":"additional","affiliation":[{"name":"Research Foundation, Royal Victoria Eye and Ear Hospital, D02 XK51 Dublin, Ireland"},{"name":"National Ophthalmic Pathology Laboratory, D04 T6F6 Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,9,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"652","DOI":"10.1016\/j.oraloncology.2015.04.005","article-title":"Adenoid cystic carcinoma of the head and neck\u2013An update","volume":"51","author":"Rodrigo","year":"2015","journal-title":"Oral Oncol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1356","DOI":"10.1634\/theoncologist.2018-0515","article-title":"Clinically Integrated Molecular Diagnostics in Adenoid Cystic Carcinoma","volume":"24","author":"Thierauf","year":"2019","journal-title":"Oncologist"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1054\/bjps.2001.3636","article-title":"Adenoid cystic carcinoma of the head and neck","volume":"54","author":"Chummun","year":"2001","journal-title":"Br. 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