Introduction

A comprehensive and uniform collection of data on head and neck paragangliomas (HNPGLs) is lacking due to its rarity and involvement of multiple medical specialties. We have initiated the Head and Neck Paraganglioma Registry (HNPGLR) to improve data collection through a predefined protocol, provide insight in to long-term outcomes using patient reported outcome measures (PROM’s), guide diagnostic and clinical management, and contribute to future (randomized) research. Additionally, we aim to expand the registry nationally to create a shared database. This abstract highlights the inclusion of the first 100 HNPGL patients or patients with genetic alterations causative of HNPGLS at the University Medical Center Utrecht (UMCU).

Methods

The HNPGLR is designed as an international prospective longitudinal observational registry for data collection on HNPGL patients and carriers of genetic alterations causative of HNPGLs. Patients can be included in the registry after obtaining informed consent, regardless of the therapy received.  All relevant data regarding initial presentation, diagnostics, treatment and follow-up will be prospectively collected. The registry protocol has been approved by the Medical Ethical Review Board.

Results

The first 100 patients have been successfully included in the HNPGL Registry, consisting of 44 males (44 %) and 56 females (56 %). In 17 patients (17%) no pathogenic variant was found, SDHx pathogenic variants were found in 78 patients (78 %) and 1 patients carried a TMEM127 pathogenic variant. Watchful waiting was performed in 46 patients (46 %), surgical resection in 48 patients (48 %), followed by radiotherapy in 5 patients (5 %), primary radiotherapy in 4 patients (4 %) and peptide receptor radionuclide therapy (PRRT) in 2 patients (2 %).

Conclusion

Data of this registry will contribute to establishing optimal management strategies and laying the foundation for future research and guidelines. While it is currently limited to a localized registry, we aspire to expand this collaboration nationally and internationally.