Clinical Report: Uncommon Case of a Patient with Tonsillar Adenoid Cystic Carcinoma

Overview

This report details a rare case of tonsillar adenoid cystic carcinoma (TACC) with a 47-year disease course, characterized by prolonged indolence followed by rapid progression.

Background

Tonsillar adenoid cystic carcinoma is an exceedingly rare malignancy, typically characterized by slow growth and late metastases.

Data Highlights

Key Findings

• The patient exhibited a 47-year disease trajectory with prolonged indolent behavior.
• Molecular profiling revealed NOTCH2 copy number gain and structural variants in MTOR and SPEN.
• Local recurrence occurred in 1996-1997, followed by a long period of clinical stability.
• Late regional and systemic progression was observed, culminating in intracranial metastasis.
• This case represents one of the longest documented clinical courses of TACC.

Clinical Implications

This case highlights the necessity for long-term surveillance in patients with TACC due to the potential for late recurrence and metastasis. Comprehensive molecular evaluation may provide insights into tumor behavior and inform management strategies.

Conclusion

The documented case of TACC illustrates the complexity of its clinical course and the importance of ongoing monitoring and molecular analysis in affected patients.

Related Resources & Content

1. NCI, Salivary Gland Cancer Treatment (PDQ®), 2025 -- Salivary Gland Cancer Treatment
2. PubMed, Survival Outcomes in Adenoid Cystic Carcinoma of the Head and Neck, 2025 -- Survival Outcomes in Adenoid Cystic Carcinoma
3. Frontiers in Surgery — Giant Adenoid Cystic Carcinoma in the Subglottic Region: A Case Study
4. Frontiers in Oncology — Complete Response of a Giant Metastatic Neck Mass in Tonsillar Squamous Cell Carcinoma Achieved with Radiotherapy Combined with Immunotherapy: A Case Report
5. Frontiers in Oncology — Successful management of metachronous dual primary esophageal carcinomas: first report of consecutive adenoid cystic carcinoma and squamous cell carcinoma with exceptional treatment outcome—a case report
6. Frontiers in Medicine — Case Report: A seventy-year-stable chest wall bronchogenic cyst with sudden enlargement: coincidence or connection with a concurrent thymic carcinoma?
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9. https://www.oregon.gov/oha/HPA/DSI-HERC/MembersOnly/7.5f%20NCCN%201.2026%20head-and-neck.pdf
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13. Genomic profiling of head and neck adenoid cystic carcinoma: A systematic review and meta-analysis - PubMed