Adrenal disease

Clinical management and outcome of head and neck paragangliomas (HNPGLs): A single centre retrospective study

Clin Endocrinol (Oxf)Published Retrospective cohort study

Authors' conclusion

The majority of our patients underwent initial surgical intervention and achieved disease stability. Our results suggest that carefully selected asymptomatic or medically unfit patients can be safely observed provided lifelong surveillance is maintained. We advocate for the establishment of a UK and Ireland national HNPGL registry, to delineate optimal management strategies for these rare tumours and improve long term outcomes.

Study context

Population
Patients with head and neck paragangliomas (HNPGLs), Northern Ireland, January 2011 through December 2023; mean age 52.3 ± 14.2 years, 50 females: 37 males
Participants
87 patients
Outcome
Clinical features, management and recurrence risk factors
Methodological detailssingle-centre, Data from The Northern Ireland Electronic Care Record (NIECR) and a prospectively maintained HNPGL database
  • Single-centre
  • Data from The Northern Ireland Electronic Care Record (NIECR) and a prospectively maintained HNPGL database

Main finding

As reported in the abstract

Carotid body tumours
58.6% (n = 51)
Glomus vagal tumours
25.2% (n = 22)
Middle ear tumours
6.8% (n = 6)
Parapharyngeal space tumours
2.2% (n = 2)
Sphenoid sinus tumours
1.1% (n = 1)
Multifocal disease
5.7% (n = 5)
Mean tumour size at presentation
3.2 ± 1.4 cm (range 0.5-6.9 cm)
Pathogenic SDHD mutations
41.3% (n = 36)
SDHB mutations
12.6% (n = 11)
SDHC mutations
2.2% (n = 2)
SDHA mutations
1.1% (n = 1)
Surgery alone
51.7% (n = 45)
Radiotherapy
14.9% (n = 13)
Observation
28.7% (n = 25)
SSA therapy with octreotide
4.5% (n = 4)
Higher recurrence risk: age over 60 years
p = .04
Higher recurrence risk: tumour size exceeding 2 cm
p = .03
Higher recurrence risk: positive SDHx variants
p = .01
Higher recurrence risk: vagal and jugular tumours
p = .04

Observational data: associations, not effects.

Original publication

Clinical management and outcome of head and neck paragangliomas (HNPGLs): A single centre retrospective study. Clin Endocrinol (Oxf). Published 2 May 2024. doi:10.1111/cen.15070

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