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Indian Journal of Otolaryngology and... Dec 2022Paragangliomas of the head and neck can arise in many locations along the carotid sheath and middle ear. A hypervascular tumor in relation to the major cervical vessels...
Paragangliomas of the head and neck can arise in many locations along the carotid sheath and middle ear. A hypervascular tumor in relation to the major cervical vessels can be either a carotid body tumor or vagal paraganglioma. Carotid bifurcation separation is a characteristic feature of carotid body tumours. We present a case of vagal paraganglioma of the neck, causing carotid bifurcation separation similar to that of a carotid body tumor. In this case report, we highlight the imaging features that can differentiate these two paragangliomas in such a confusing situation.
PubMed: 36742569
DOI: 10.1007/s12070-021-03005-w -
Cancers Apr 2024Head and neck paragangliomas (HNPGLs) are rare and have high rates of genetic mutations. We conducted a retrospective review of 187 patients with 296 PGLs diagnosed...
Head and neck paragangliomas (HNPGLs) are rare and have high rates of genetic mutations. We conducted a retrospective review of 187 patients with 296 PGLs diagnosed between 1974 and 2023. The mean age of diagnosis was 48.8 years (range 10 to 82) with 69.0% female and 26.5% patients with multiple PGLs. Among 119 patients undergoing genetic testing, 70 (58.8%) patients had mutations, with SDHB (30) and SDHD (26) being the most common. The rates of metastasis and recurrence were higher among patients with SDHB mutations or SDHD mutations associated with multiple PGLs. Metabolic evaluation showed elevated plasma dopamine levels were the most common derangements in HNPGL. MRI and CT were the most common anatomic imaging modalities and DOTATATE was the most common functional scan used in this cohort. Most patients (81.5%) received surgery as the primary definitive treatment, while 22.5% patients received radiation treatment, mostly as an adjuvant therapy or for surgically challenging or inoperable cases. Systemic treatment was rarely used in our cohort. Our single-center experience highlights the need for referral for genetic testing and metabolic evaluation and for a team-based approach to improve the clinical outcomes of patients with HNPGLs.
PubMed: 38672605
DOI: 10.3390/cancers16081523 -
Head & Neck Oct 2023Vagus nerve paragangliomas are rare tumors, comprising 0.03% of head and neck neoplasms. These tumors are usually located cephalad to the hyoid bone, and there is only...
BACKGROUND
Vagus nerve paragangliomas are rare tumors, comprising 0.03% of head and neck neoplasms. These tumors are usually located cephalad to the hyoid bone, and there is only one previously reported case that arose from the lower third of the neck.
METHODS
We describe the second reported case of a lower neck vagus nerve paraganglioma that was managed with a limited sternotomy for access and surgical removal.
RESULTS
A 66-year-old male presented with a long-standing lesion of the cervicothoracic junction. CT, MRI, and Ga-68 DOTATATE PET/CT showed an avidly enhancing 5.2 × 4.2 × 11.5 cm mass extending from C6 to approximately T4 level. FNA confirmed the diagnosis. The patient underwent catheter angiography and embolization via direct puncture technique followed by excision of the mass via a combined transcervical and limited sternotomy approach.
CONCLUSION
We describe an unusual case of vagal paraganglioma at the cervicothoracic junction with retrosternal extension requiring a sternotomy for surgical excision.
Topics: Male; Humans; Aged; Gallium Radioisotopes; Positron Emission Tomography Computed Tomography; Vagus Nerve; Paraganglioma, Extra-Adrenal; Cranial Nerve Neoplasms; Vagus Nerve Diseases; Head and Neck Neoplasms; Paraganglioma
PubMed: 37548094
DOI: 10.1002/hed.27481 -
Cureus Oct 2021Vagal paraganglioma is a rare finding that develops from paraganglionic tissue found around the vagus nerve; it has a prevalence of 0.012% of all tumors. It is the...
Vagal paraganglioma is a rare finding that develops from paraganglionic tissue found around the vagus nerve; it has a prevalence of 0.012% of all tumors. It is the third most common paraganglioma of the head and neck but still accounts for less than 5% of these tumors, and it has a well-established female prevalence. It is a difficult tumor to identify early based on its symptoms alone and only a thorough investigation can help solidify its diagnosis. In this report, we discuss a presentation of this phenomenon that is not only unique in its manifestation but also a very difficult diagnosis due to its deceptive presentation and multiple extensions. These masses need a good surgical regime to be removed properly and postoperative complications are very frequent in most of these cases.
PubMed: 34733595
DOI: 10.7759/cureus.18423 -
Ear, Nose, & Throat Journal May 2024To summarize the clinicopathological and genetic features of malignant paragangliomas in head and neck cancer and to explore the appropriate treatment options for this...
To summarize the clinicopathological and genetic features of malignant paragangliomas in head and neck cancer and to explore the appropriate treatment options for this rare lesion. Six patients harboring head and neck malignant paraganglioma from Beijing Tongren Hospital were retrospectively reviewed. The clinicopathological characteristics, gene mutations, and prognosis of these patients were analyzed. Of these 6 patients, 3 were male and 3 were female; 4 patients harbored malignant carotid body tumors, and two had malignant vagal paragangliomas. Three patients had cervical lymph node metastasis, two presented with lung and bone metastasis, and 1 had lung and liver metastasis. Of the 6 patients, four underwent surgical resection, and the other two patients denied surgery and instead received chemotherapy with paclitaxel, ifosfamide, and dacarbazine. These 2 patients with vagal paraganglioma received postoperative radiotherapy. All 6 patients are still alive at the present time, with a median follow-up time of 66 months. Positive Ki-67 expression in tumor tissue ranged from 1% to 40%. Genetic mutations in SDHD, SDHB, ATR, and MAP3K13 were identified in 4 patients. After comprehensive treatment, head and neck malignant paraganglioma can attain a favorable prognosis. Genetic mutations are commonly detected in patients with malignant paragangliomas. This study also identified mutations in ATR and MAP3K13 in these patients.
Topics: Humans; Female; Male; Middle Aged; Retrospective Studies; Head and Neck Neoplasms; Adult; Mutation; Paraganglioma; Prognosis; Carotid Body Tumor; Succinate Dehydrogenase; Aged; Lymphatic Metastasis
PubMed: 34654328
DOI: 10.1177/01455613211052338 -
Der Radiologe Dec 2019Glomus tumors (paragangliomas) are rare, usually benign, neuroendocrine tumors that arise from nonchromaffin cells and can be multifocal or familial. They grow slowly,... (Review)
Review
Glomus tumors (paragangliomas) are rare, usually benign, neuroendocrine tumors that arise from nonchromaffin cells and can be multifocal or familial. They grow slowly, often infiltratively and rarely form metastases. A distinction is made between carotid, jugular, tympanicum and vagal tumors. Depending on the location, clinical symptoms include neck swelling, hearing loss with pulse-synchronous ear noise or cranial nerve failures (IX-XI). Diagnosis is often delayed because of nonspecific symptoms. Diagnostically, tumor expansion and perfusion can be well visualized by color Doppler sonography. In the T2-weighted MRI, the tumor is hyperintense, after contrast agent administration a strong enhancement occurs due to the strong vascularization. In DSA (digital subtraction angiography), even small glomus tumors can be detected. Therapeutically, operative tumor resection after embolization with, e.g., polyvinyl alcohol particles or liquid embolization can be performed. If the patient is in poor general condition or if the tumor is inoperable, different treatment techniques (intensity-modulated radiotherapy, gamma or cyber-knife) may be considered as a conservative therapeutic alternative.
Topics: Angiography, Digital Subtraction; Embolization, Therapeutic; Glomus Tumor; Head and Neck Neoplasms; Humans; Skull Base
PubMed: 31701165
DOI: 10.1007/s00117-019-00605-0 -
Medicina (Kaunas, Lithuania) May 2024: Paragangliomas of the head and neck are rare, slow-growing neuroendocrine tumors, benign in their vast majority, but with a possibility of developing distant... (Review)
Review
: Paragangliomas of the head and neck are rare, slow-growing neuroendocrine tumors, benign in their vast majority, but with a possibility of developing distant metastases. They show great inheritable character, and their behavior has proven to be unpredictable; therefore, they are considered malignant. This article aims to offer a more comprehensive presentation of the pathogenesis, epidemiology, diagnostic methods, imaging development, and treatment guidelines. We tried to bring together all the necessary data that, in our opinion, a head and neck practitioner should know when managing this type of tumor. Our main focus is on the most recent studies, with the purpose of a homogenous presentation of all current guidelines and approaches to this pathology. : Paragangliomas of the head and neck are still a disputed topic. One of the main reasons for that is their low incidence of 0.3 to 1 per 100,000 every year. The most frequent locations are the carotid body, the temporal bone, the jugular and mastoid foramen, and the vagal nerve. Their clinical presentation usually involves a painless lateral mass associated with symptoms such as hoarseness, hearing loss, tinnitus, and cranial nerve deficits. Up to 40% of them are inherited, mostly linked with mutations of succinate dehydrogenase complex. Imaging evaluation consists of CT and MRI, and new functional explorations such as F-FDA and F-FDG PET/CT, F-DOPA PET, I-MIBG, and Ga-DOTATE PET/CT. Measuring the catecholamine levels in the plasma and urine is mandatory, even though paragangliomas of the head and neck rarely display secretory behavior. Treatment mainly consists of surgery, with different approaches and techniques, but conservative management methods such as wait and scan, radiotherapy, proton therapy, and chemotherapy have proven their efficiency. The therapeutical decision lacks consensus, and current studies tend to recommend an individualized approach. Guidelines regarding long-term follow-up are still a matter of debate.
Topics: Humans; Head and Neck Neoplasms; Paraganglioma
PubMed: 38929531
DOI: 10.3390/medicina60060914 -
Auris, Nasus, Larynx Jun 2024The patient, a 40-year-old woman, was diagnosed as having a functional right vagal paraganglioma (PGL) 15 years after undergoing resection for a retroperitoneal PGL....
The patient, a 40-year-old woman, was diagnosed as having a functional right vagal paraganglioma (PGL) 15 years after undergoing resection for a retroperitoneal PGL. I-MIBG scintigraphy showed no accumulation, but as the blood noradrenaline and urinary normetanephrine concentrations were elevated, the tumor was judged as being functional, and surgery was scheduled. The patient was started on doxazosin infusion and embolization of the tumor feeding vessel was performed before the surgery. Intraoperative examination showed that the tumor was contiguous with the vagal nerve, necessitating combined resection of the vagal nerve with the tumor. Postoperatively, the catecholamine levels returned to normal range. Histopathologically, the tumor was diagnosed as a moderately differentiated, intermediate-malignant-grade PGL, with a GAPP score of 4 to 6. No non-chromaffin tissue was observed in the tumor background, so that the functional vagal PGL was considered as a sporadic metachronous tumor rather than as a metastasis from the retroperitoneal PGL. More than half of head and neck paragangliomas (HNPGLs) are reported to arise in the carotid body, and about 5% from the vagal nerve. In addition, HNPGLs rarely produce catecholamines. Herein, we consider the relationship with the previously resected retroperitoneal PGL based on a review of the literature.
Topics: Humans; Female; Adult; Retroperitoneal Neoplasms; Paraganglioma; Paraganglioma, Extra-Adrenal; Normetanephrine; Neoplasms, Second Primary; Cranial Nerve Neoplasms; Vagus Nerve Diseases; Embolization, Therapeutic; Norepinephrine; Vagus Nerve
PubMed: 38520971
DOI: 10.1016/j.anl.2023.12.002 -
Head & Neck Oct 2017The main goal of head and neck paraganglioma (PGL) management is reduction of treatment-induced and tumor-induced complications. In the current study, tumor growth rates...
BACKGROUND
The main goal of head and neck paraganglioma (PGL) management is reduction of treatment-induced and tumor-induced complications. In the current study, tumor growth rates and tumor-induced complications during a wait-and-scan period are evaluated.
METHODS
This was a retrospective cohort study. Tumor growth was measured in axial plane diameter and tumor volume.
RESULTS
Of 59 jugulotympanic tumors, 71 carotid body tumors, and 29 vagal body tumors, 44% were growing (median follow-up of 63.6 months). Median growth rates were 0.41 mm/year (range 0-439 mm), 1.6 mm/year (range 0-23.68 mm), and 1.6 mm/year (range 0-23.68 mm) respectively. Growth was significantly correlated to age at presentation (odds ratio [OR] = 0.974; P < .05). Seventeen tumors induced 20 complications. Six of these tumors were growing, and growth rates were higher than in tumors not inducing complications (P = .016; F = 6.496).
CONCLUSION
These results illustrate the feasibility of a wait-and-scan strategy for head and neck PGL. The management strategy could not prevent tumor-induced complications in 16% of nongrowing tumors.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Cohort Studies; Feasibility Studies; Female; Follow-Up Studies; Head and Neck Neoplasms; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Mutation; Paraganglioma; Retrospective Studies; Succinate Dehydrogenase; Watchful Waiting; Young Adult
PubMed: 28691354
DOI: 10.1002/hed.24871 -
Indian Journal of Otolaryngology and... Oct 2019Vagal paragangliomas are rare tumors. Advances in the imaging have aided the diagnosis and assessment of this disease. Surgery is the mainstay of the treatment however...
Vagal paragangliomas are rare tumors. Advances in the imaging have aided the diagnosis and assessment of this disease. Surgery is the mainstay of the treatment however radiotherapy has been tried in the selected cases. Surgery is associated with some mortality but with a lot of morbidity in view of cranial nerve deficit. The necessity to prevent additional cranial nerve deficits needs thorough preoperative evaluation and influences the therapeutic approach. This study involves the retrograde evaluation of five patients of vagal paraganglioma histopathologically confirmed, all the patients were operated via trancervical approach. Preoperatively three of five had 9, 11 and 12th cranial nerve involvement, one had horner's syndrome. Postoperative vocal rehabilitation was done in all the patient by medialisation thyroplasty Issiki type 1. Though vagal paragangliomas are associated with mortality and morbidity. Surgical treatment should aim at reducing mortality. Vagal nerve morbidity can be taken care as a planned and staged procedure mainly involving restoring voice as rest of concerns are taken care by compensation from opposite nerves.
PubMed: 31741999
DOI: 10.1007/s12070-018-1340-5