Adenoid cystic cancer of nasopharynx: a single-institution retrospective study
- Authors: Alieva S.B.1, Chernykh M.V.1, Zaderenko I.A.1, Saprina O.A.1
-
Affiliations:
- N. N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia
- Issue: Vol 16, No 1 (2026)
- Pages: 27-37
- Section: DIAGNOSIS AND TREATMENT OF HEAD AND NECK TUMORS
- Published: 09.06.2026
- URL: https://ogsh.abvpress.ru/jour/article/view/1141
- DOI: https://doi.org/10.17650/2222-1468-2026-16-1-27-37
- ID: 1141
Cite item
Abstract
Introduction. Adenocystic nasopharyngeal carcinoma is a rare but highly aggressive malignancy arising from the tissue of the minor salivary glands. Currently, there is no consensus in the medical community regarding the optimal treatment method. The priority of surgery over radiation therapy, as well as the advisability of monotherapy versus combination approaches, remain controversial.
Aim. To summarize the clinical characteristics of adenocystic nasopharyngeal carcinoma based on an analysis of 13 cases from a single institution, with the aim of optimizing therapeutic approaches for treating patients with these rare tumors.
Materials and methods. A retrospective analysis of data from 13 patients with nasopharyngeal adenocarcinoma treated at the N. N. Blokhin National Medical Research Center of Oncology from 1989 to 2023 was conducted. All patients underwent conservative chemoradiation therapy: in 6 cases – concurrent chemoradiation therapy, in 7 cases – induction chemotherapy followed by radiation or chemoradiation.
Results. A complete clinical effect was achieved in 9 patients, a partial effect in 4. Disease progression was noted in 7 cases: local recurrence in 3 patients, continued tumor growth in 1, distant metastases in 3. The five-year survival rate was 38.5 %. Five patients died during the first two months, another three were lost to follow-up 5–15 months after the start of treatment. The median overall survival was 28 months. Conclusion. Adenoid cystic carcinoma of the nasopharynx is an extremely rare pathology. Although the standard treatment is surgery followed by radiation therapy, when radical surgery is not possible in patients with locally advanced disease, chemoradiation therapy becomes the treatment of choice.
About the authors
S. B. Alieva
N. N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia
Author for correspondence.
Email: drsevil@mail.ru
ORCID iD: 0000-0002-6835-5567
Russian Federation, 24 Kashirskoe Shosse, Moscow, 115522
M. V. Chernykh
N. N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia
Email: drsevil@maul.ru
ORCID iD: 0000-0003-4944-4035
Russian Federation, 24 Kashirskoe Shosse, Moscow, 115522
I. A. Zaderenko
N. N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia
Email: drsevil@mail.ru
ORCID iD: 0000-0003-0183-4827
Russian Federation, 24 Kashirskoe Shosse, Moscow, 115522
O. A. Saprina
N. N. Blokhin National Medical Research Center of Oncology, Ministry of Health of Russia
Email: drsevil@mail.ru
ORCID iD: 0000-0002-2283-1812
Russian Federation, 24 Kashirskoe Shosse, Moscow, 115522
References
- Liu T.R., Yang A.K., Guo X. et al. Adenoid cystic carcinoma of the nasopharynx: 27-year experience. Laryngoscope 2008;118(11):1981–8. doi: 10.1097/MLG.0b013e3181801d23
- Amit M., Binenbaum Y., Sharma K. Adenoid cystic carcinoma of the nasal cavity and paranasal sinuses: a meta-analysis. J Neurol Surg B Skull Base 2013;74(3):118–25. doi: 10.1055/s-0033-1347358
- Lloyd S., Yu J.B., Wilson L.D., Decker R.H. Determinants and patterns of survival in adenoid cystic carcinoma of the head and neck, including an analysis of adjuvant radiation therapy. Am J Clin Oncol 2011;34(1):76–81. doi: 10.1097/COC.0b013e3181d26d45
- Kokemueller H., Eckardt A., Brachvogel P., Hausamen J.E. Adenoid cystic carcinoma of the head and neck – a 20-year experience. Int J Oral Maxillofac Surg 2004;33(1):25–31. doi: 10.1054/ijom.2003.0448
- Van Weert S., van der Waal I., Witte B.I. et al. Histopathological grading of adenoid cystic carcinoma of the head and neck: analysis of currently used grading systems and proposal for a simplified grading scheme. Oral Oncol 2015;51(1):71–6. doi: 10.1016/j.oraloncology.2014.10.007
- Hellquist H., Skalova A., Barnes L. et al. Cervical lymph node metastasis in high-grade transformation of head and neck adenoid cystic carcinoma: a collective international review. Adv Ther 2016;33(3):357–68. doi: 10.1007/s12325-016-0298-5
- Weissferdt A., Moran C.A. Primary thymic adenoid cystic carcinoma with high-grade transformation: a clinicopathological and immunohistochemical analysis of 4 cases. Pathol Res Pract 2024;259:155356. doi: 10.1016/j.prp.2024.155356
- Maurya S., Gupta S., Soni A. et al. High-grade transformation in adenoid cystic carcinoma: can it be diagnosed on cytology? A cytohistological correlation. Cytojournal 2024;21:10. doi: 10.25259/Cytojournal_38_2023
- Wen Q.L., Zhu S.M., Jiang L.H. et al. Expression and prognostic significance of MCM-3 and MCM-7 in salivary adenoid cystic carcinoma. Int J Clin Exp Pathol 2018;11(11):5359–69.
- Shamloo N., Taghavi N., Ahmadi S., Shalpoush S. Immunohistochemical analysis of proliferating cell nuclear antigen and minichromosome maintenance complex component 7 in benign and malignant salivary gland tumors. Dent Res J (Isfahan) 2022;19:17. doi: 10.4103/1735-3327.338780
- Lee D.J., Smith R.R., Spaziani J.T. et al. Adenoid cystic carcinoma of the nasopharynx. Case reports and literature review. Ann Otol Rhinol Laryngol 1985;94:269–72.
- Yin Z.Y., Wu X.L., Hu Y.H., Gu X.Z. Cylindroma of the nasopharynx: a chronic disease Int J Radiat Oncol Biol Phys 1986;12(1):25–30. doi: 10.1016/0360-3016(86)90411-6
- Wang C.C., See L.C., Hong J.H., Tang S.G. et al. Nasopharyngeal adenoid cystic carcinoma: five new cases and a literature review J Otolaryngol 1996;25(6):399–403.
- Schramm V.L., Imola M.J. Management of nasopharyngeal salivary gland malignancy. Laryngoscope 2001;111(119):1533–44. doi: 10.1097/00005537-200109000-00009
- Wen S.X., Tang P.Z., Xu Z.G. et al. Therapeutic modalities of nasopharyngeal adenoid cystic carcinoma. Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2006;41(5):359–61.
- Khademi B., Bahranifard H., Kabiri S. et al. Nasopharyngeal adenoid cystic carcinoma: report of five cases and treatment outcome. Middle East J Cancer 2011;2(2):81–5.
- Samant S., van den Brekel M.W., Kies M.S. et al. Concurrent chemoradiation for adenoid cystic carcinoma of the head and neck. Head Neck 2012;34(9):1263–8. doi: 10.1002/hed.21905
- Cao C.N., Luo J.W., Xu G.Z. et al. Management of nasopharyngeal adenoid cystic carcinoma. J Oral Maxillofac Surg 2013;71(4): e203–9. doi: 10.1016/j.joms.2012.12.024
- Takagi M., Demizu Y., Hashimoto N. et al. Treatment outcomes of particle radiotherapy using protons or carbon ions as a single-modality therapy for adenoid cystic carcinoma of the head and neck. Radiother Oncol 2014;113(3):364–70. doi: 10.1016/j.radonc.2014.11.031
- Ding S., Jin T., Xu M., Liang Y. Adenoid cystic carcinoma of nasopharynx: a single-center retrospective study. Biomed Res 2017;28(14).
- Gentile M.S., Yip D., Liebsch N.J. et al. Definitive proton beam therapy for adenoid cystic carcinoma of the nasopharynx involving the base of skull. Oral Oncol 2017;65:38–44.
- Ryckman J.M., Kusi Appiah A., Simone C.B. 2nd, Verma V. Treatment approaches for nasopharyngeal adenoid cystic carcinoma. Acta Oncol 2018;57(7):995–1001. doi: 10.1080/0284186X.2018.1426878
- Wu W.B., Cai W.L., Zou Y.H. et al. Outcomes of patients in nasopharyngeal adenoid cystic carcinoma in the IMRT era: a single-center experience. BMC Cancer 2024;24(1):576. doi: 10.1186/s12885-024-12159-z
- Mehta G.U., Hanna E.Y., DeMonte F., Raza S.M. Endoscopic endonasal resection of sinonasal/anterior skull base malignancy (Kadish C esthesioneuroblastoma). Acta Neurochir 2018;160(2):361–6. doi: 10.1007/s00701-017-3437-0
- Nicolai P., Battaglia P., Bignami M. et al. Endoscopic surgery for malignant tumors of the sinonasal tract and adjacent skull base: a 10-year experience. Am J Rhinol 2008;22(3):308–16. doi: 10.2500/ajr.2008.22.3170
- McDonald M.W., Liu Y., Moore M.G., Johnstone P.A. Acute toxicity in comprehensive head and neck radiation for nasopharynx and paranasal sinus cancers: cohort comparison of 3D conformal proton therapy and intensity modulated radiation therapy. Radiat Oncol 2016;11:32. doi: 10.1186/s13014-016-0600-3
- Pommier P., Liebsch N.J., Deschler D.G. et al. Proton beam radiation therapy for skull base adenoid cystic carcinoma. Arch Otolaryngol Head Neck Surg 2006;132(11):1242–9. doi: 10.1001/archotol.132.11.1242
- Jia Y., Liu Y., Yang H., Yao F. Adenoid cystic carcinoma: insights from molecular characterization and therapeutic advances. MedComm 2024;5(9):e734. doi: 10.1002/mco2.734
- Persson M., Andrén Y., Mark J. et al. Recurrent fusion of MYB and NFIB transcription factor genes in carcinomas of the breast and head and neck. Proc Natl Acad Sci USA 2009;106(44):18740–4. doi: 10.1073/pnas.0909114106
- Hanna G.J., ONeill A., Cutler J.M. et al. A phase II trial of all-trans retinoic acid (ATRA) in advanced adenoid cystic carcinoma. Oral Oncol 2021;119:105366. doi: 10.1016/j.oraloncology.2021.105366
- Pham T., Pereira L., Roth S. et al. First-in-human phase I clinical trial of a combined immune modulatory approach using TetMYB vaccine and anti-PD-1 antibody in patients with advanced solid cancer including colorectal or adenoid cystic carcinoma: the MYPHISMO study protocol (NCT03287427). Contemp Clin Trials Commun 2019;16:100409. doi: 10.1016/j.conctc.2019.100409
- Xi S., Soulard P., Li K. et al. Effect of RGT-61159 on inhibition of oncogene c-MYB synthesis and tumor growth inhibition in a broad range of ACC PDX models, at well tolerated doses in rodents and non-human primates. Meeting Abstract: 2024 ASCO Annual Meeting J Clin Oncol 2024;42(16):6107. doi: 10.1200/JCO.2024.42.16_suppl.6107
- Ghosal N., Mais K., Shenjere P. et al. Phase II trial of imatinib in patients with adenoid cystic carcinoma of the salivary glands. Br J Oral Maxillofac Surg 2011;49(7):510–5. doi: 10.1016/j.bjoms.2010.09.013
- Slevin N.J., Mais K.L., Bruce I. Imatinib with cisplatin in recurrent and/or metastatic salivary adenoid cystic carcinoma: response assessed by FDG-PET scanning. J Clin Oncol 2004;22(14):5604. doi: 10.1200/jco.2004.22.90140.5604
- Tchekmedyian V., Sherman E.J., Dunn L. et al. Phase II study of lenvatinib in patients with progressive, recurrent or metastatic adenoid cystic carcinoma. J Clin Oncol 2019;37(18):1529–37. doi: 10.1200/JCO.18.01859
- Han J., Ma I., Hendzel M.J., Allalunis-Turner J. The cytotoxicity of gamma-secretase inhibitor I to breast cancer cells is mediated by proteasome inhibition, not by gamma-secretase inhibition. Breast Cancer Res 2009;11(4):R57. doi: 10.1186/bcr2347
- Hanna G.J., Stathis A., Lopez-Miranda E. et al. A phase I study of the Pan-NOTCH inhibitor CB-103 for patients with advanced adenoid cystic carcinoma and other tumors. Cancer Res Commun 2023;3(9):1853–61. doi: 10.1158/2767-9764.CRC-23-0333
- Mosconi C., de Arruda J.A.A., de Farias A.C.R. et al. Immune microenvironment and evasion mechanisms in adenoid cystic carcinomas of salivary glands. Oral Oncol 2019;88:95–101. doi: 10.1016/j.oraloncology.2018.11.028
- Ho A.S., Kannan K., Roy D.M. et al. The mutational landscape of adenoid cystic carcinoma. Nat Genet 2013;45(7):791–8. doi: 10.1038/ng.2643
- Vos J.L., Burman B., Jain S. et al. Nivolumab plus ipilimumab in advanced salivary gland cancer: a phase 2 trial. Nat Med 2023;29(12):3077–89. doi: 10.1038/s41591-023-02518-x
- Mahmood U., Bang A., Chen Y.H. et al. A phase II study of pembrolizumab in combination with radiotherapy in patients with recurrent or metastatic adenoid cystic carcinoma. Int J Radiat Oncol Biol Phys 2020;109(1):134–44. doi: 10.1016/j.ijrobp.2020.08.018
- Zhu G., Zhang L., Dou S. et al. Apatinib in patients with recurrent or metastatic adenoid cystic carcinoma of the head and neck: a single-arm, phase II prospective study. Ther Adv Med Oncol 2021;13:17588359211013626. doi: 10.1177/17588359211013626
Supplementary files


