{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T05:06:04Z","timestamp":1780376764949,"version":"3.54.1"},"reference-count":22,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T00:00:00Z","timestamp":1780358400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:p>Tonsillar adenoid cystic carcinoma (TACC) is an extremely rare malignancy with an unpredictable clinical course. We report an unusual case of TACC with a 47-year disease trajectory, characterized by prolonged indolent behavior followed by late and relatively rapid clinical progression. The patient was initially diagnosed with left tonsillar ACC in 1977 after presenting with dysphagia and underwent tonsillectomy followed by postoperative radiotherapy. A local recurrence involving the left soft palate occurred in 1996\u20131997 and was treated with surgical resection and tongue flap reconstruction, after which the disease remained clinically stable for many years. In 2018, recurrent ACC of the soft palate was confirmed in the radical resection specimen, and the patient underwent radical resection via a mandibulotomy approach followed by reconstruction and adjuvant concurrent chemoradiotherapy. Molecular profiling of the available 2018 recurrent soft palate lesion revealed NOTCH2 copy number gain and structural variants involving MTOR and SPEN. Regional cervical lymph node metastasis was confirmed in 2022, followed by intracranial disease progression in 2023. The patient died in 2024 from complications of progressive intracranial and systemic metastases. To our knowledge, this represents one of the longest documented clinical courses of TACC with integrated genomic analysis. This case highlights the potential for TACC to remain indolent for decades before transitioning to late regional and systemic progression, underscoring the importance of long-term surveillance and comprehensive clinicopathological and molecular evaluation.<\/jats:p>","DOI":"10.3389\/fonc.2026.1824507","type":"journal-article","created":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T04:29:10Z","timestamp":1780374550000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case Report: A rare case of over 45 years\u2019 survival in a patient with tonsillar adenoid cystic carcinoma"],"prefix":"10.3389","volume":"16","author":[{"given":"Mingzhu","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Thyroid and Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tingyao","family":"Ma","sequence":"additional","affiliation":[{"name":"Department of Thyroid and Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yue","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Thyroid and Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shujing","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Thyroid and Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xuelian","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Thyroid and Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guoliang","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Thyroid and Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xi","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Thyroid and Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yixuan","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Thyroid and Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tian","family":"Ye","sequence":"additional","affiliation":[{"name":"Department of Thyroid and Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mengmeng","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Thyroid and Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Thyroid and Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lanlan","family":"Xuan","sequence":"additional","affiliation":[{"name":"Department of Pathology, Anqing Hospital Affiliated to China Pharmaceutical University\/Anqing Municipal Hospital","place":["Anqing, Anhui, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaohong","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Thyroid and Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zheng","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Thyroid and Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,6,2]]},"reference":[{"key":"B1","doi-asserted-by":"crossref","DOI":"10.1016\/j.oraloncology.2015.04.005","article-title":"Adenoid cystic carcinoma of the head and neck \u2013 an update","volume":"51","author":"Coca-Pelaz","year":"2015","journal-title":"Oral Oncol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1634\/theoncologist.2018-0515","article-title":"Clinically integrated molecular diagnostics in adenoid cystic carcinoma","volume":"24","author":"Thierauf","year":"2019"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1038\/ng.2643","article-title":"The mutational landscape of adenoid cystic carcinoma","volume":"45","author":"Ho","year":"2013","journal-title":"Nat Genet"},{"key":"B4","doi-asserted-by":"crossref","first-page":"105135","DOI":"10.1016\/j.oraloncology.2020.105135","article-title":"Palatine tonsil adenoid cystic carcinoma","volume":"115","author":"Tretiakow","year":"2021","journal-title":"Oral Oncol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1177\/0145561320970691","article-title":"Adenoid cystic carcinoma of the larynx: a SEER database review","volume":"101","author":"Mur","year":"2022","journal-title":"Ear Nose Throat J"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1177\/0194599817690138","article-title":"Risk of nodal metastasis in major salivary gland adenoid cystic carcinoma","volume":"156","author":"Megwalu","year":"2017","journal-title":"Otolaryngol Neck Surg"},{"key":"B7","doi-asserted-by":"publisher","first-page":"1240","DOI":"10.1001\/archoto.2010.213","article-title":"Factors predicting outcome in Malignant minor salivary gland tumors of the oropharynx","volume":"136","author":"Iyer","year":"2010","journal-title":"Arch Otolaryngol Neck Surg"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1007\/s11912-022-01233-z","article-title":"Treatment of recurrent or metastatic adenoid cystic carcinoma","volume":"24","author":"De Sousa","year":"2022","journal-title":"Curr Oncol Rep"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1155\/2021\/5511935","article-title":"Colonic metastasis of adenoid cystic carcinoma 19 years after the primary tumor resection","volume":"2021","author":"Mallikarjunappa","year":"2021","journal-title":"Case Rep Pathol"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.3969\/j.issn.1000-8179.2016.24.125","article-title":"Clinical study on cervical lymph node metastasis pattern in salivary gland adenoid cystic carcinoma","volume":"51","author":"Huang","year":"2016","journal-title":"Zhonghua Kou Qiang Yi Xue Za Zhi"},{"key":"B11","doi-asserted-by":"crossref","first-page":"11185","DOI":"10.1038\/s41598-023-38227-1","article-title":"Intraparotid lymph node metastasis affects distant metastasis in parotid adenoid cystic carcinoma","volume":"13","author":"Han","year":"2023","journal-title":"Sci Rep"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1172\/JCI128227","article-title":"Genetic hallmarks of recurrent\/metastatic adenoid cystic carcinoma","volume":"129","author":"Ho","year":"2019","journal-title":"J Clin Invest"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2016.67.5264","article-title":"Activating NOTCH1 mutations define a distinct subgroup of patients with adenoid cystic carcinoma who have poor prognosis, propensity to bone and liver metastasis, and potential responsiveness to Notch1 inhibitors","volume":"35","author":"Ferrarotto","year":"2017","journal-title":"J Clin Oncol"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2023.1191218","article-title":"Salivary glands adenoid cystic carcinoma: a molecular profile update and potential implications","volume":"13","author":"da Silva","year":"2023","journal-title":"Front Oncol"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.3390\/cancers14225698","article-title":"Approaches to the management of metastatic adenoid cystic carcinoma","volume":"14","author":"Lee","year":"2022","journal-title":"Cancers (Basel)"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.21.00449","article-title":"Management of salivary gland Malignancy: ASCO guideline","volume":"39","author":"Geiger","year":"2021","journal-title":"J Clin Oncol"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1177\/00368504241308735","article-title":"Adenoid cystic carcinoma of palatine tonsil: a case report","volume":"107","author":"Guo","year":"2024","journal-title":"Sci Prog"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1097\/scs.0b013e318231fdf8","article-title":"Bilateral tonsillar adenoid cystic carcinoma","volume":"22","author":"Azizli","year":"2011","journal-title":"J Craniofac Surg"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.4103\/0973-029x.140796","article-title":"Adenoid cystic carcinoma: an unusual presentation","volume":"18","author":"Pushpanjali","year":"2014","journal-title":"J Oral Maxillofac Pathol"},{"key":"B20","doi-asserted-by":"publisher","first-page":"1","DOI":"10.5693\/djo.02.2018.01.001","article-title":"Adenoid cystic carcinoma presenting with bilateral orbital extension from the soft palate","volume":"24","author":"Schwartz","year":"2018","journal-title":"Digit J Ophthalmol"},{"key":"B21","doi-asserted-by":"publisher","first-page":"156","DOI":"10.1186\/s13256-021-02781-x","article-title":"Stereotactic body radiotherapy for adenoid cystic carcinoma metastatic to the lung: a case report","volume":"15","author":"Kobayashi","year":"2021","journal-title":"J Med Case Rep"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1016\/j.radcr.2025.08.044","article-title":"Hard palate adenoid cystic carcinoma: surgical resection, radiotherapeutic strategy, and long-term surveillance","volume":"20","author":"Bouchlarhem","year":"2025","journal-title":"Radiol Case Rep"}],"container-title":["Frontiers in Oncology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1824507\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T04:29:17Z","timestamp":1780374557000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1824507\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,2]]},"references-count":22,"alternative-id":["10.3389\/fonc.2026.1824507"],"URL":"https:\/\/doi.org\/10.3389\/fonc.2026.1824507","relation":{},"ISSN":["2234-943X"],"issn-type":[{"value":"2234-943X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,2]]},"article-number":"1824507"}}