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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 -
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 -
Annales D'endocrinologie Aug 2023Thoracic and cervical paragangliomas (PGLs) are rare neuroendocrine tumors arising from chromaffin cells of the neural crest progenitors located outside the adrenal... (Review)
Review
Thoracic and cervical paragangliomas (PGLs) are rare neuroendocrine tumors arising from chromaffin cells of the neural crest progenitors located outside the adrenal gland. We describe our current protocol as a multidisciplinary team for the management of cervical and thoracic PGLs. Surgery is generally considered the treatment of choice as it offers the best chance for cure. For resection of thoracic PGLs, video-assisted thoracoscopic surgery (VATS) is the main surgical approach, while open thoracotomy is preferred in case of tumors > 6cm, lacking confirmation of a plane of separation with adjacent structures, or with technical difficulties during VATS. In cervical PGLs, the surgical approach should be individualized according to location, mainly based on the Glasscock-Jackson and the Fisch-Mattox classifications. Surgery is the treatment of choice for most cervical and thoracic PGLs, but radiotherapy or observation could be more suitable options in unresectable cervical and thoracic PGLs or when resection has been incomplete.
Topics: Humans; Paraganglioma; Neuroendocrine Tumors; Adrenal Glands
PubMed: 36334803
DOI: 10.1016/j.ando.2022.10.013 -
Turkish Neurosurgery 2024Vagal paragangliomas (VPs) are rare tumors arising from paraganglionic tissue within the vagal nerve's perineurium. Usually, benign vascular tumors, VPs tend to invade...
Vagal paragangliomas (VPs) are rare tumors arising from paraganglionic tissue within the vagal nerve's perineurium. Usually, benign vascular tumors, VPs tend to invade the surrounding structures. Herein, we report the case of a VP presenting as a neck mass, which was evaluated as a glomus caroticum tumor preoperatively. A 65-year-old female complaining of a left-sided neck mass and intermittent hoarseness was assessed and operated on for possible glomus caroticum tumor. During the tumor excision, the vagal nerve was also involved, and hence, sacrificed. Histopathological examination revealed an encapsulated tumor associated with a nerve and ganglion and immunohistochemical staining tested positive for succinate dehydrogenase, confirming the diagnosis of VP. Postoperative residual hoarseness was corrected by vocal rehabilitation. While evaluating a retropharyngeal prestyloid neck mass, a VP should always be considered. Surgical excision involving vagal scarification, followed by vocal rehabilitation may be the appropriate treatment strategy.
Topics: Female; Humans; Aged; Hoarseness; Paraganglioma, Extra-Adrenal; Vagus Nerve; Immunohistochemistry; Paraganglioma
PubMed: 37846532
DOI: 10.5137/1019-5149.JTN.40702-22.2