Abstract

Surgical resection remains the first line treatment for salivary gland cancer (SGC). In the case of locally advanced disease, surgery is followed by adjuvant radiotherapy. Surgical resection should be favored in resectable locoregional recurrent disease as well, and even the complete resection of all distant oligometastases has clinical benefit for the patients. For inoperable and disseminated metastatic disease, a multitude of systemic therapies including chemotherapy, targeted therapy, and immunotherapy are available. In this review, the current therapeutic options for inoperable recurrent or metastatic SGCs are summarized. Systemic treatment can achieve prolonged progression-free and overall survival, while the overall prognosis remains poor. Current clinical trials include only a limited number of patients and mostly combine different histologic subtypes. Additionally, no randomized controlled trial comparing different therapeutic options has been performed. In the future, further studies with a larger patient cohort and ideally only one histologic subtype are needed in order to improve the outcome for SGC patients. However, this may be difficult to accomplish due to the rarity and diversity of the disease. Additionally, molecular analyses need to be performed routinely in order to individualize treatment and to go one step further towards precision medicine.

Highlights

  • Salivary gland cancer (SGC) is a rare (0.6–1.4 per 100,000 [1]) and heterogenous group of head and neck tumors

  • SGCs are classified by their source of origin and their histopathologic grading [3]

  • Targeted therapies investigating mucoepidermoid carcinoma (MEC) included cetuximab [45], nintedanib [52], and sorafenib [62], with the results shown in the adenoid cystic carcinoma (ACC) section

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Summary

Introduction

Salivary gland cancer (SGC) is a rare (0.6–1.4 per 100,000 [1]) and heterogenous group of head and neck tumors. SGCs are classified by their source of origin (major salivary glands versus minor salivary glands) and their histopathologic grading (high grade, intermediate grade, low grade) [3]. Due to their rare occurrence, published studies tend to look at different histopathologic types, grades, and localizations conjointly, diminishing the validity of the data. For inoperable primary and recurrent SGC as well as oligometastatic SGC different systemic options are available, with varying success rates.

Histopathologic Types
Mucoepidermoid Carcinoma
Acinic Cell Carcinoma
Salivary Duct Carcinoma
Adenocarcinoma NOS
Systemic Therapy for Advanced and Recurrent Disease and Distant Metastases
C-Kit and EGFR
Single and Combination Cytotoxic Chemotherapy
Acinic Cell Carcinomas
Clinical Trials
Potential New Systemic Therapeutic Strategies
Targeted Therapies Based on Actionable Molecular Alterations
Findings
Conclusions
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