Oops, you're using an old version of your browser so some of the features on this page may not be displaying properly.

MINIMAL Requirements: Google Chrome 24+Mozilla Firefox 20+Internet Explorer 11Opera 15–18Apple Safari 7SeaMonkey 2.15-2.23

ESMO E-Learning: Rare Head and Neck Tumours. New Approaches in Systemic Treatment for Relapsed or Metastatic Disease

New E-Learning module by Dr Caroline Even is now available. Watch the presentation and take the CME test today!

ESMO members

Take the CME test and receive

1 ESMO - MORA point

45 slides / 60 minutes

Learning objectives

  1. To distinguish a profile of genomic alterations in salivary gland carcinoma and other rare head and neck tumours
  2. To understand the potential impact of targetable molecular alterations for the treatment of patients with rare head and neck tumours  
  3. To provide an overview of immunotherapy research status in patients with rare head and neck tumours


In this E-Learning module, the author underlines that the determination of the histologic type / subtype of salivary gland tumours is pivotal. Systemic treatments, in particularly cytotoxics, have limited efficacy. Targetable molecular alterations depend on tumour subtype and performing a molecular profile allows guiding into clinical trials or expanded access programme. This module provides an excellent overview of genomic alterations, efficacy of targeted treatments and immunotherapy in rare head and neck tumours.

Salivary gland carcinomas are uncommon neoplasms accounting for up to 5% of all cancers of the head and neck. They typically occur in young patients and are characterised by slow growth, multiple local recurrences and prolonged clinical course, often with delayed development of distant metastases.

There are around 20 histologic subtypes. In this E-Learning module, the author presents, in a systemic way, specificities of mucoepidermoid carcinoma, adenocarcinoma, adenoid cystic carcinoma and salivary duct carcinoma. Concurrent with pathologic diversity, there is a remarkable genetic diversity. Most targeted therapies have shown lack of efficacy, probably related to the fact that there was no biomarker selection.

More recent studies have been conducted on the basis of molecular driven selection and have demonstrated efficacy of androgen deprivation in androgen receptor-positive salivary duct carcinoma, trastuzumab and ado-trastuzumab emtansine in HER2-positive salivary duct carcinoma and NTRK inhibitors in mammary analogue secretory carcinoma.  

There is limited clinical activity of immune checkpoint inhibitors and predictive biomarkers are required. Combinatorial strategies should be considered to turn these cold tumours into hot tumours.

The author underlines that a multinational cooperation is required to conduct well designed clinical trials allowing homogeneity in terms of subtypes, disease stage, progressive disease at inclusion, quality of life analysis and patient reported outcomes.  

In terms of other rare head and neck tumours, new distinct tumour entities in the sinonasal tract have been described in the 2017 WHO classification. Most of these newly described diagnostic entities are defined in part on their underlying viral or genetic mechanisms, supporting their separate classification and in some cases, offering potential therapeutic targets for the future. In case of sinonasal undifferenciated carcinoma, the author’s advice is for ensuring NUT and INI1 determination.

There is no indication for immunotherapy outside of clinical trials except for squamous cell carcinoma of sinonasal cavities. In case of tumour mutational burden > 10, the author advises considering anti-PD1 therapy within clinical trial or as an approved indication, depending on the country of practice.

Last update: 20 Oct 2021

Dr Even has reported honoraria for Advisory Board from: BMS, Innate Pharma, Merck Serono, MSD.
No financial interest, Local PI, Institutional: AstraZeneca, Ayala, BMS, Debiopharma, ISA pharmaceutics, MSD.
No financial interest, Coordinating PI, Institutional: Novartis and BMS. 

This site uses cookies. Some of these cookies are essential, while others help us improve your experience by providing insights into how the site is being used.

For more detailed information on the cookies we use, please check our Privacy Policy.

Customise settings
  • Necessary cookies enable core functionality. The website cannot function properly without these cookies, and you can only disable them by changing your browser preferences.