Prognostic's Factors of Head and Neck Paragangliomas Evolution (PRONO-PARAG)

Cervical paragangliomas (HNPG) are rare tumors (0.6% of head and neck tumors) arising from chromaffin tissue, of cervical paraganglia (PGL). The most common locations are carotid body (60% of cases), jugulo-tympanic region and vagal body.

More than 30% are proved to occur in a context of genetic predisposition, more often in young people, and genetic screening is recommended in all patients. Multifocal tumors represent 12% of all HNPG and until 50% of familial forms. Most of HNPG are non-secreting, benign and slow growing tumors, but up to 30% present complications of local growth, and up to 10% can develop distant metastasis that define malignancy since there is no pathological marker.

Historically, surgical treatment was the standard of care but represents nowadays around 50% of the treatment, mostly due to the identification of high morbidity rates. The rate of recurrence is probably around 10% at 5 years. Radiotherapy and active follow-up represent the main therapeutic alternatives.

The standard of care is classically surgical but may expose to important sequelae leading to a review of its primary indication. Indeed, cranial nerve palsies (VII, IX, X, XI and XII) may complicate up to 20% of carotid PGL surgeries and up to 95% of vagal PGL surgeries. They are leading to significant functional sequelae, sometimes requiring recourse to a gastrostomy (4/79 patients operated on in a retrospective cohort). First bite syndrome, Claude Bernard Horner syndrome, baroreceptor failure, xerostomia, and ischemic events complicate 5.8%, 4.9%, 1.9%, 1%, and 1% of surgeries respectively. In a local retrospective study conducted by the Hospices Civils de Lyon on 34 operated cervical PGL, the overall complication rate reached 62%. These complications depend mainly on the location tumor and its size.

Control rate of irradiated HNPG at 5 years from retrospective series seems to be around 90%. They seems also to have a possible better progression-free survival at 15 years than surgery. The tolerance is correct, the risk of induced malignancy is estimed at 1/1000 to 1/2000. Without treatment, 44% of cervical PGL show a significant progression (median follow-up 51 months). Progression is estimated at 0.41 mm/year for jugulo-tympanic PGL and 1.6 mm/year for vagal and carotid PGL.

Currently, there is no clear and robust consensus regarding the follow-up of cervical PGL and the indications for different therapeutic strategies. Data available are represented by retrospective studies only, mostly small in size, with heterogeneous and often inadequate follow-up compared to slow tumor growth.

Thus, this prospective cohort study with a standardized long-term follow-up will allow to characterize the management modalities and the evolution of this population.

Study Overview

• Status: Active, not recruiting
• Conditions: Paraganglioma
• Intervention / Treatment: Other: Characterization of a population with cervical paragangliomas

• Study Type: Observational
• Enrollment (Estimated): 25

Contacts and Locations

Study Locations

• France
  • Bron, France, 69500
    • Fédération d'endocrinologie Hôpital Cardiologique/Groupement Hospitalier Est

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

Adult patients with a confirmed diagnosis of one or multiple cervical paragangliomas

Description

Inclusion Criteria:

• Man or woman aged 18 or over
• Patient with a confirmed diagnosis of one or multiple cervical paragangliomas regardless of genetic predisposition without specific therapy
• Patient informed of the study details and who didn't opposed to participate in this research

Exclusion Criteria:

• Patient already treated without initial clinical and radiologic assessment standardized
• Patient already treated with surgery, radiotherapy or systemic therapy for another paraganglioma
• Patient with evolutive disease and life expectancy less than 2 years
• Patient placed under legal protection
• Patient participating in another interventional clinical study that may interfere with the results of this study

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Cohort group; Patients over 18 with a confirmed diagnosis of one or multiple cervical paragangliomas	Other: Characterization of a population with cervical paragangliomas; Characterization of a population with sporadic or genetically predisposed cervical paragangliomas in order to evaluate : the initial presentation of the PGL, patients management and patients outcome

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Characterization of a population with sporadic or genetically predisposed cervical paragangliomas	Evaluation of the initial presentation of the PGL, patients management and patients outcome	24 months

Collaborators and Investigators

• Sponsor: Hospices Civils de Lyon

Study record dates

Study Major Dates

• Study Start (Actual): July 19, 2022
• Primary Completion (Estimated): June 1, 2027
• Study Completion (Estimated): June 1, 2027

Study Registration Dates

• First Submitted: January 31, 2022
• First Submitted That Met QC Criteria: January 31, 2022
• First Posted (Actual): February 10, 2022

Study Record Updates

• Last Update Posted (Actual): June 30, 2025
• Last Update Submitted That Met QC Criteria: June 26, 2025
• Last Verified: June 1, 2025

More Information

Terms related to this study

• Keywords: Cervical paragangliomas
• Additional Relevant MeSH Terms: Neoplasms; Neoplasms by Histologic Type; Neuroectodermal Tumors; Neoplasms, Germ Cell and Embryonal; Neoplasms, Nerve Tissue; Neuroendocrine Tumors; Paraganglioma
• Other Study ID Numbers: 69HCL21_0295

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No