Abstract

Simple SummaryAdenoid cystic carcinoma is a rare histology arising in the head and neck region that has a high propensity for perineural invasion. While surgical resection is the preferred primary treatment modality, adjuvant radiotherapy is often indicated to improve local control. For unresectable disease, definitive radiotherapy can be utilized. Given the predilection for perineural tumor spread, target volumes often must encompass relevant nerve pathways back to the base of skull. Treatment strategies for ACC must therefore balance the disease burden and risk of failure against the morbidity of treatment.Adenoid cystic carcinoma of the head and neck is an uncommon malignancy that can arise in the major or minor salivary glands. Perineural invasion (PNI) is an extremely frequent finding in cases of adenoid cystic carcinoma (ACC) that can be associated with significant patient morbidity and poor prognosis. By contrast, ACC rarely demonstrates lymphovascular space invasion thereby making PNI the major avenue for metastasis and a driver of treatment rationale and design. Radiotherapy is often utilized post-operatively to improve locoregional control or as a primary therapy in unresectable disease. Here we aim to review the role of radiotherapy in the management of this malignancy with a focus on target delineation and treatment regimens in the definitive, recurrent, and metastatic settings.

Highlights

  • Adenoid cystic carcinoma (ACC) of the head and neck region, which accounts for 1–2%of all head and neck cancers, is a challenging clinical entity to treat due to its unique clinical and pathologic features and the lack of prospective data guiding ideal treatment approach.This disease is often characterized by a deceivingly indolent presentation followed by perineural invasion (PNI), local recurrence, and metastatic spread

  • This review aims to summarize the current clinical management of adenoid cystic carcinoma of the head and neck as relevant to the radiation oncologist, with a focus on pathologic risk factors, radiation target delineation, relevant anatomy, and imaging techniques

  • Perineural invasion (PNI) refers to a histopathologic finding of tumor infiltration into or around nerve tissue; it is important to distinguish this from perineural tumor spread (PNTS), which is clinically or imaging-identified macroscopic extension of a tumor along a nerve

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Summary

Introduction

Adenoid cystic carcinoma (ACC) of the head and neck region, which accounts for 1–2%. of all head and neck cancers, is a challenging clinical entity to treat due to its unique clinical and pathologic features and the lack of prospective data guiding ideal treatment approach. Adenoid cystic carcinoma (ACC) of the head and neck region, which accounts for 1–2%. Of all head and neck cancers, is a challenging clinical entity to treat due to its unique clinical and pathologic features and the lack of prospective data guiding ideal treatment approach. This disease is often characterized by a deceivingly indolent presentation followed by perineural invasion (PNI), local recurrence, and metastatic spread. This review aims to summarize the current clinical management of adenoid cystic carcinoma of the head and neck as relevant to the radiation oncologist, with a focus on pathologic risk factors, radiation target delineation, relevant anatomy, and imaging techniques

Pathologic Considerations
Radiologic Evaluation of PNTS
Imaging Techniques
PNTS as seen on Postcontrast
Submandibular Gland
Hard Palate
Nerve Interconnections
Imaging Pitfalls
Rationale for Radiation
Radiation Therapy Design
10. Dose Regimen
11. Treatment Related Toxicity
12. Particle Therapy
13. Re-Irradiation
14. Concurrent Chemo-Radiotherapy
15. Treatment of Metastatic Disease
Findings
16. Conclusions

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