Clinical features and outcome in patients with functional head and neck paragangliomas: French national cohort and literature review

Iuliia Pinigina; Philippe Ceruse; Philippe Herman; Cardot Catherine; Magalie Haissaguerre; Erika Cornu; Laurence Amar; Marie Batisse-Lignier; Laurence Chardon; Sara Barraud; Loic Feuvret; Marc Janier; Lasolle Helene; Francoise Borson-Chazot; Abeillon Juliette

doi:10.1530/endoabs.99.P501

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Endocrine Abstracts (2024) 99 P501 | DOI: 10.1530/endoabs.99.P501

ECE2024 Poster Presentations Endocrine-Related Cancer (40 abstracts)

Clinical features and outcome in patients with functional head and neck paragangliomas: French national cohort and literature review

Iuliia Pinigina ¹ , Philippe Ceruse ¹ , Philippe Herman ² , Cardot Catherine ³ , Magalie Haissaguerre ⁴ , Erika Cornu ⁵ , Laurence Amar ⁶ , Marie Batisse-Lignier ⁷ , Laurence Chardon ¹ , Sara Barraud ⁸ , Loic Feuvret ¹ , Marc Janier ¹ , Lasolle Hélène ¹ , Francoise Borson-Chazot ¹ & Abeillon Juliette ¹

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¹Hospices Civils de Lyon – HCL, Lyon, France; ²Lariboisière Hospital AP-HP, Paris, France; ³Hospital Center University De Lille, Lille, France; ⁴Hospital Center University De Bordeaux, Bordeaux, France; ⁵Médipôle Lyon-Villeurbanne, Villeurbanne, France; ⁶European Hospital Georges Pompidou, Paris, France; ⁷Jean Perrin Center, Clermont-Ferrand, France; ⁸Hospital Robert Debré, Reims, France

Objectives: Management of functional head and neck paragangliomas (HNPGLf) presents a substantial challenge due to their rarity, the lack of standardized treatment protocols, their complex neurovascular anatomical location, and the cardiovascular risks associated with catecholamine (CA) hypersecretion. This study aimed to describe the clinical characteristics of HNPGLf and their treatment outcomes.

Methods: We retrospectively analyzed a cohort of HNPGLf patients treated at seven French EURACAN-COMETE centers from 2000 to 2023. The secretory nature was defined by CA-methylated metabolites (MM) exceeding 3 times the upper limit of normal. In addition, a comprehensive literature review was conducted.

Results: The French cohort included 20 patients (70% female, median age: 39.5 years). HNPGLf were vagal (n=10), jugulo-tympanic (n=10), and carotid (n=3), with a median largest dimension of 43 mm (range: 23–80), secreting noradrenaline and/or dopamine. Diagnoses were established based on tumor syndrome (n=15), signs of CA hypersecretion (n=1), germline SDHD mutation (n=1), and incidental findings (n=1). Eight out of 19 tested patients had SDHx mutations. Treatment modalities included surgery (n=8), external beam radiation therapy (EBRT) (n=5), radiosurgery (n=1), proton therapy (PT) (n=1), and ¹⁷⁷Lu-DOTATATE (n=2). The median post-therapeutic follow-up was 39.7 months (range: 4.1 – 176.9). There were 3 cases of morphological progression, but no metastasis. 70.6% of patients showed significant secretory improvement, with 7 patients normalizing MM levels (all had surgery), and 5 showing partial biochemical response (EBRT n=2, radiosurgery n=1, PT n=1, ¹⁷⁷Lu-DOTATATE n=1). Neurological complications occurred in 64.7% cases, primarily after surgery. The Literature cohort comprised 17 patients with demographic characteristics, clinical presentations, and tumor features similar to the French cohort. However, the proportion of females was lower (35.5%), and the secretory profile was unspecified in 41.2% of cases because total metanephrine levels were used for diagnosis. Three out of 4 tested patients had SDHx mutations. Outcomes and complications were comparable to that observed in the French cohort, except for a shorter median post-treatment follow-up (9.5 months).

Conclusion: This study presents, to the best of our knowledge, the largest reported HNPGLf cohort to date. Surgical, radiotherapeutic and radiometabolic interventions achieved tumor and secretory control in 70.6% of cases. Longer-term studies are warranted to identify the best therapeutic strategy.