Comprehensive Review of Adenoid Cystic Carcinoma: Pathogenesis, Diagnosis, and Emerging Therapeutic Approaches

Year : 2025 | Volume : 14 | Issue : 03 | Page : 1 17
    By

    Gaurav Kumar Rajput,

  • Chitra Singh,

  • Megha Singh,

  • Khushi Chauhan,

  1. Assistant Professor, Department of Computing Sciences & IT, College of Computing Sciences & Information Technology TMU, Moradabad, Uttar Pradesh, India
  2. M.Tech. (Student), Department of Biotechnology, Jaypee Institute of Information Technology, Noida, Uttar Pradesh, India
  3. M.Tech. (Student), Department of Biotechnology, Jaypee Institute of Information Technology, Noida, Uttar Pradesh, India
  4. M.Tech. (Student), Department of Biotechnology, Jaypee Institute of Information Technology, Noida, Uttar Pradesh, India

Abstract

Adenoid cystic carcinoma (ACC)is an infrequent neoplasm, highly malignant, that develops mainly in the salivary glands with the potential to exist in any secretory glandular sites, including the lacrimal glands, breast, and respiratory tract. ACC usually has a benign initial course, but conversely, it is notoriously aggressive in behavior with high incidence of perineural invasion, local recurrence, and distant metastasis, mostly to the lungs. The tumor’s molecular features are characterized by recurrent MYB-NFIB fusion gene aberrations, aberrations in the regulation of the PI3K/AKT/mTOR and Wnt/β-catenin pathways, and other epigenetic changes driving oncogenesis. As a result, major advancements in diagnosis, including multiparametric imaging, next-generation sequencing (NGS), and immunohistochemical profiling, have facilitated the early detection and molecular subclassification. Although surgical resection with negative margins remains the gold standard treatment, the infiltrative and neurotropic behavior of ACC often results in incompletely excised tumors, which could lead to a high recurrence rate. IMRT, CIRT, and SRS are being developed as effective adjuncts to surgery. However, cytotoxic chemotherapy remains almost entirely ineffective owing mainly to inherent tumor-resistance mechanisms. Here new options are being investigated as part of targeted molecular therapies such as TKIs, inhibitors of the NOTCH pathway, and PD-1/PD-L1 immune checkpoint blockade. AI and machine learning are revolutionizing genomic analysis and personalized medicine-oriented applications that further refine treatment stratification and prognostic modeling. Due to the heterogeneous and complex nature of ACC, a multimodal precision oncology-based approach is, therefore, very much warranted for improving clinical outcomes. This review addresses the molecular pathogenesis understanding, simultaneous advances in diagnostic technologies, and innovative therapeutic paradigms of ACC, all together leading toward improved prognosis and personalized management.

Keywords: Adenoid cystic carcinoma, MYB-NFIB fusion, perineural invasion, targeted therapy, immunotherapy, tyrosine kinase inhibitors, PI3K/AKT/mTOR pathway, epigenetic modifications, next-generation sequencing, artificial intelligence

[This article belongs to Research and Reviews: Journal of Oncology and Hematology ]

How to cite this article:
Gaurav Kumar Rajput, Chitra Singh, Megha Singh, Khushi Chauhan. Comprehensive Review of Adenoid Cystic Carcinoma: Pathogenesis, Diagnosis, and Emerging Therapeutic Approaches. Research and Reviews: Journal of Oncology and Hematology. 2025; 14(03):1-17.
How to cite this URL:
Gaurav Kumar Rajput, Chitra Singh, Megha Singh, Khushi Chauhan. Comprehensive Review of Adenoid Cystic Carcinoma: Pathogenesis, Diagnosis, and Emerging Therapeutic Approaches. Research and Reviews: Journal of Oncology and Hematology. 2025; 14(03):1-17. Available from: https://journals.stmjournals.com/rrjooh/article=2025/view=233006


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Regular Issue Subscription Review Article
Volume 14
Issue 03
Received 04/07/2025
Accepted 20/07/2025
Published 25/11/2025
Publication Time 144 Days
100

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