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Current Opinion in Otolaryngology &... Apr 2023A vagal paraganglioma is a rare head and neck tumor arising from the paraganglionic tissue within the perineurium of the vagus nerve, anywhere along the course of the... (Review)
Review
PURPOSE OF REVIEW
A vagal paraganglioma is a rare head and neck tumor arising from the paraganglionic tissue within the perineurium of the vagus nerve, anywhere along the course of the nerve. Due to its proximity to the internal carotid artery, the internal jugular vein and the lower cranial nerves, this disorder poses significant diagnostic and therapeutic challenges. The diagnostic workup and management keep on evolving.
RECENT FINDINGS
This article gives a concise update of the clinical spectrum and the current state-of-the-art diagnostic workup and management of vagal paraganglioma.
SUMMARY
Every patient with suspected vagal paraganglioma needs to be evaluated by a multidisciplinary team. The management strategy is selected depending on the growth rate of the tumor, the age and fitness of the patient, the number of affected cranial nerves, the metabolic activity of the paraganglioma, and the eventual multicentricity. An algorithm guiding the clinician through the different treatment options is presented.
Topics: Humans; Paraganglioma, Extra-Adrenal; Paraganglioma; Head and Neck Neoplasms
PubMed: 36912227
DOI: 10.1097/MOO.0000000000000876 -
Neuroimaging Clinics of North America May 2016Paragangliomas of the head and neck are rare vascular skull-base tumors derived from the paraganglionic system with an estimated incidence of 1:30,000 accounting for 3%... (Review)
Review
Paragangliomas of the head and neck are rare vascular skull-base tumors derived from the paraganglionic system with an estimated incidence of 1:30,000 accounting for 3% of all paragangliomas. The most common paraganglioma locations of the head and neck in descending order are the carotid body, jugular, tympanic, and vagal paragangliomas. This article discusses the clinical characterics, normal anatamy, imaging findings and protocols, pathology, staging, and differential diagnosis for paragangliomas of the head and neck.
Topics: Aortic Bodies; Carotid Arteries; Ear, Middle; Head and Neck Neoplasms; Humans; Jugular Veins; Magnetic Resonance Imaging; Paraganglioma; Tomography, X-Ray Computed
PubMed: 27154608
DOI: 10.1016/j.nic.2015.12.005 -
Head and Neck Pathology Jun 2016Paragangliomas are rare, typically benign neuroendocrine tumors that represent a small portion of head and neck tumors. A small percentage of these are known to have...
Paragangliomas are rare, typically benign neuroendocrine tumors that represent a small portion of head and neck tumors. A small percentage of these are known to have malignant potential. They arise from the carotid body, jugular bulb or vagus nerves. There is limited literature discussing the management of malignant vagal paragangliomas. We present a case of a 25 year old female with a left malignant vagal paraganglioma. The following case presentation will describe the presentation, classic radiologic findings, and management of a malignant vagal paraganglioma along with a review of the literature.
Topics: Adult; Female; Humans; Lymphatic Metastasis; Paraganglioma, Extra-Adrenal; Vagus Nerve Diseases
PubMed: 25712400
DOI: 10.1007/s12105-015-0621-5 -
Khirurgiia 2022Neck paragangliomas are orphan diseases with incidence 1:30 000-1:100 000. Life expectancy is poor in patients with distant metastasis (5-year overall survival 11.8%),... (Review)
Review
Neck paragangliomas are orphan diseases with incidence 1:30 000-1:100 000. Life expectancy is poor in patients with distant metastasis (5-year overall survival 11.8%), whereas 5-year overall survival in patients with regional metastasis is 76.8-82.4%. Meanwhile, there is still no any reliable tool for prediction of malignant potential of paraganglioma. Above-mentioned data indicate an importance of early diagnosis and timely treatment of neck paragangliomas. Total resection of tumor in ablastic conditions is a gold standard of treatment. However, surgery is associated with a high risk of neurovascular complications and requires multidisciplinary approach. Nevertheless, new knowledge dedicated to different aspects of pathogenesis of neck paraganglioma, diagnosis and treatment arise every year. This review is devoted to modern data on neck paragangliomas.
Topics: Head and Neck Neoplasms; Humans; Paraganglioma
PubMed: 35775846
DOI: 10.17116/hirurgia202207164 -
International Journal of Molecular... Oct 2020Pheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors. Head and neck paragangliomas (HNPGL) can be categorized into carotid body tumors, which... (Review)
Review
Pheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors. Head and neck paragangliomas (HNPGL) can be categorized into carotid body tumors, which are the most common, as well as jugular, tympanic, and vagal paraganglioma. A review of the current literature was conducted to consolidate knowledge concerning PGL mutations, familial occurrence, and the practical application of this information. Available scientific databases were searched using the keywords head and neck paraganglioma and genetics, and 274 articles in PubMed and 1183 in ScienceDirect were found. From these articles, those concerning genetic changes in HNPGLs were selected. The aim of this review is to describe the known genetic changes and their practical applications. We found that the etiology of the tumors in question is based on genetic changes in the form of either germinal or somatic mutations. 40% of PCC and PGL have a predisposing germline mutation (including and ). Approximately 25-30% of cases are due to somatic mutations, such as , and . The tumors were divided into three main clusters by the Cancer Genome Atlas (TCGA); namely, the pseudohypoxia group, the Wnt signaling group, and the kinase signaling group. The review also discusses genetic syndromes, epigenetic changes, and new testing technologies such as next-generation sequencing (NGS).
Topics: Genetic Predisposition to Disease; Head and Neck Neoplasms; Humans; Mutation; Paraganglioma
PubMed: 33081307
DOI: 10.3390/ijms21207669 -
Cancer Control : Journal of the Moffitt... Jul 2016Commonly occurring in the head and neck, paragangliomas are typically benign, highly vascular neoplasms embryologically originating from the extra-adrenal paraganglia of... (Review)
Review
BACKGROUND
Commonly occurring in the head and neck, paragangliomas are typically benign, highly vascular neoplasms embryologically originating from the extra-adrenal paraganglia of the neural crest. Frequently, these tumors are associated with the vagus or tympanic plexus nerve or the carotid artery, or jugular bulb. Their clinical presentation can vary across a wide spectrum of signs and symptoms.
METHODS
We reviewed and compared standard treatment approaches for paragangliomas of the head and neck.
RESULTS
In general, surgery is the first-line choice of therapy for carotid body tumors, whereas radiotherapy is the first-line option for jugular and vagal paragangliomas.
CONCLUSIONS
Because of the complexity of clinical scenarios and treatment options for paragangliomas, a multidisciplinary algorithmic approach should be used for treating paragangliomas. The approach should emphasize single-modality treatment that yields excellent rates of tumor control, low rates of severe, iatrogenic morbidity, and the preservation of long-term function in this patient population.
Topics: Female; Head and Neck Neoplasms; Humans; Male; Paraganglioma
PubMed: 27556663
DOI: 10.1177/107327481602300306