The Need for a Multidisciplplinary Approach in a Vagal Paraganglioma
- 1. Department of Otolaryngology, Head-Neck Surgery, Río Hortega University Hospital, Spain
CITATION
Pérez-Chacón P, Sañudo EGC, de las Heras-Flórez P (2024) The Need for a Multidisciplplinary Approach in a Vagal Paraganglioma. Ann Otolaryngol Rhinol 11(5): 1345.
INTRODUCTION
Quality of life is considered one of the most important parameters when treating a patient. As physicians we have treated countless pathologies for centuries, trying to cure the disease or reduce the symptoms. However, there are pathologies that require an approach that goes beyond medicine. In the field of ENT there are a lot of pathologies in which a speech therapist is needed to improve the patient’s quality of life. For that reason, we present a case in which without the approach between ENT and speech therapists the patient could not have a normal life.
CASE REPRESENTATION
A 43-year-old female patient, with no known risk factors, referred to the Otorhinolaryngology Service by her dentist after noticed an increase in volume in the right peritonsillar region during orthodontic treatment. She did not present any symptoms at the time of diagnosis.
An oropharyngoscopy was performed, observing a significant protrusion of the right tonsil towards the midline with a normalappearing right tonsil [Figure 1].
Figure 1: Significant protrusion of the right tonsil of normal appearance and size.
Cervical palpation did not reveal masses or lymph nodes of interest. Fibroscopy reflects anatomical and functional normality of the larynx.
A cervicothoracic computed tomography was performed in which a heterogeneous right laterocervical tumor measuring 7.2x3.5x3.6 cm was observed that extended from the carotid bifurcation to contact the base of the skull without entering the jugular foramen [Figure 2].
Figure 2: Cervicothoracic CT with evidence of a right cervical mass, corresponding to vagal paraganglioma.
A urinalysis was requested with negative catecholamine results due to radiological suspicion of paraganglioma.
After surgical resection of the vagal paraganglioma, the patient presented severe-moderate dysphagia due to paralysis of the right hemilarynx due to section of the vagus nerve and paresis of the right hypoglossal nerve due to vascular mechanical compromise during surgery. She presents with dysphonia and dysphagia with aspirations that trigger coughing spells. Dysphagia causes sialorrhea and the impossibility of oral feeding, requiring a nasogastric tube.
Follow-up is carried out in a specific unit with speech therapists in which it was decided to start speech therapy treatment for dysphagia. Among the exercises are training with postural maneuvers of the chin along with dietary adaptation according to the IDDSI scale. The treatment is accompanied by specific compensatory swallowing maneuvers and techniques for sensory enhancement of the tonsillar pillars, among others. Speech therapy treatments achieve safe feeding without the need for a nasogastric tube. To increase vocal quality and projection, it was decided to perform a surgical intervention to medialize the right vocal cord accompanied by speech therapy exercises.
The patient was diagnosed with right vagal paraganglioma in a heterozygous carrier of the c.286G>A change in the SDHB gene and is currently being treated with lutetium-177 dotatate, the first radioactive drug approved to treat advanced neuroendocrine tumors. This drug consists of a radioactive isotope, lutetium-177, linked to dotatate - a molecule that binds to neuroendocrine tumor cells that have a somatostatin receptor molecule on the surface.
Thanks to the functional unit made up of speech therapists and otorhinolaryngologists, the patient can currently lead a completely normal life, both on a personal and professional level.
DISCUSSION
Paragangliomas are neuroendocrine tumors derived from neural crest cells that arise in extra-adrenal autonomous paraganglia, both sympathetic and parasympathetic. Paragangliomas of the head and neck represent 0.6% of tumors in this area, with those of the carotid body being the most frequent, followed by those of the jugular foramen and vagal ones. Vagal paragangliomas constitute 5% of head and neck paragangliomas. The vagus nerve is altered in 28% of cases. Complete resection of these tumors usually requires removal of the vagus nerve [1,2].
There is a genetic predisposition, the highest known in a solid tumor, for paragangliomas caused by mutations in genes that encode subunits of the enzyme succinate dehydrogenase (SDH). SDH-related syndromes are transmitted in an autosomal dominant manner with incomplete penetrance and are responsible for 80% of familiar aggregations of paraganglioma and pheochromocytoma. Most paragangliomas are benign, but over time, approximately 15-35 percent show malignant behavior. In the skull base and neck the malignancy rate is slightly higher for vagal tumors. The highest rates of malignancy are observed in paragangliomas associated with hereditary pathogenic variants in the B subunit of the succinate dehydrogenase (SDHB) gene, as is the case of the patient under study and her daughter [3].
The therapeutic approach to head and neck paragangliomas is evolving. Therapeutic options include observation, radiation therapy, surgical excision, or combined therapy of surgical cytoreduction followed by radiation. Cases in which initial surgery should be considered include healthy young patients, such as our patient [4,5]. In our case, it is a non-secretory vagal paraganglioma, which is the most frequent form of presentation according to the location in the literature, since only 3-5% are symptomatic.
CONCLUSION
Surgical morbidity associated with vagal deficits is inevitable. Literature studies indicate that nerve preservation can only be achieved in 5-8% of patients. Therefore, cranial nerve dysfunction is a common complication after surgery for cervical paragangliomas and almost always after transection of the vagus nerve [4,5]. However, with interdisciplinary work between otorhinolaryngologists and speech therapists, the prognosis can improve the quality of life of affected patients.
