Prognostic's Factors of Head and Neck Paragangliomas Evolution

Study Overview
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Brief Summary
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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.

Official Title
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Prognostic's Factors of Head and Neck Paragangliomas Evolution

Conditions
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Paraganglioma

Intervention / Treatment
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* Other: Characterization of a population with cervical paragangliomas


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Cohort group<br>Patients over 18 with a confirmed diagnosis of one or multiple cervical paragangliomas | Other: Characterization of a population with cervical paragangliomas<br>* 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<br>|

What is the study measuring?
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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 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Cervical paragangliomas