Endocrine Abstracts

Background: Head and neck paragangliomas (HNPGL) are rare, usually benign slow-growing tumours arising from neural crest-derived tissue. Definitive treatment strategies for HNPGL have not been clearly defined.

Aims: To provide a comprehensive review of our institutional experience of the clinical features, investigations, management and follow up of this cohort.

Methods: Baseline clinical information was taken from a prospectively maintained HNPGL database between January 2017 to January 2022. Further clinical, radiological and laboratory data as well as outcomes were obtained from electronic medical records (NIECR).

Results: There were 21 patients; 10 M; 11F with a mean age of 54 years (range 17-76). Seven (33.3%) patients had glomus jugulare tumour, six (28.5%) had carotid body tumour, four (19%) had glomus tympanicum, four (19%) were in other locations. Tumour size ranged from 20 mm to 5 cm. Thirteen (61.9%) patients had a pre-operative scans, six of which had octreotide avid scans. To date five (23%) patients had SDHD gene mutation and three (14%) patients had SDHB gene mutation identified. All tumours were non-functioning with normal urine metanephrines. Ten (47%) patients underwent surgical resection with five subsequently receiving adjuvant radiotherapy. Recurrence was present in three patients who had surgery for jugulare tumours. Four (19%) patients were deemed not suitable candidates for surgery either due to their extensive disease burden, tumour location or the presence of multiple co-morbidities. Three (14%) patients received monthly sandostatin/Octreotide-therapy. Two of these three patients had stable tumour size without any significant growth since they started on Octreotide-therapy. The remaining four patients (19%) were managed conservatively with serial imaging and observation.

Conclusion: Management of patients with HNPGL requires a multidisciplinary approach and should be individualized and tailored to each patient. Consideration for primary surgical treatment should consider performance status, tumour location and size. Initial surgery still provides excellent outcomes for patients, with adjunctive radiotherapy as second line treatment. The role of Octreotide therapy should be studied in a larger cohort to determine longer term outcomes.