prognostic's factors of head and neck paragangliomas evolution

Conditions: Paraganglioma

Summary: 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.


Eligibility criteria:
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

Keywords: cervical paragangliomas