-
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 -
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 -
Clinical Radiology Sep 2019To evaluate whether standard magnetic resonance imaging (MRI) could distinguish vagal paragangliomas (VPs) from carotid body tumours (CBTs) in the parapharyngeal space.
AIM
To evaluate whether standard magnetic resonance imaging (MRI) could distinguish vagal paragangliomas (VPs) from carotid body tumours (CBTs) in the parapharyngeal space.
METHODS AND MATERIALS
Thirteen VPs in 13 patients and 29 CBTs in 26 patients were included in this study. MRI features were evaluated independently by two head and neck radiologists with 10 and 16 years of experience (X.Wang and Y.Chen).
RESULTS
Significant differences were found in shape, direction of internal carotid artery (ICA) displacement, splaying of the carotid bifurcation, involvement of the jugular foramen, and maximum vertical diameters between VPs and CBTs (p=0.016, <0.001, <0.001, <0.001 and <0.001, respectively). Splaying of the carotid bifurcation was found in all the 29 CBTs for both observers, whereas only four VPs for observer 1 and two for observer 2 showed this feature. The ICA was displaced posteriorly in all the 29 patients with CBTs for both observers, and only three patients with VPs for observer 1 and two for observer 2. Involvement of the jugular foramen was found in seven patients with VPs for both observers, while none of patients with CBT showed this feature. With the combination of splaying of the carotid bifurcation and involvement of the jugular foramen, the multiple logistic regression model revealed the diagnostic accuracy was 95.2% for observer 1 and 97.6% for observer 2. With the combination of ICA displacement direction and involvement of the jugular foramen, the diagnostic accuracy was 97.6% for both observers.
CONCLUSION
MRI features can effectively differentiate VPs from CBTs in the parapharyngeal space.
Topics: Adult; Carotid Body Tumor; Contrast Media; Cranial Nerve Neoplasms; Diagnosis, Differential; Female; Gadolinium DTPA; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Paraganglioma; Parapharyngeal Space; Vagus Nerve
PubMed: 31122716
DOI: 10.1016/j.crad.2019.04.018 -
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 -
The Journal of International Advanced... Nov 2022This study aimed to review tumor control and cranial nerve function outcomes in patients with complex jugular paragangliomas and to refine the surgical strategies for...
BACKGROUND
This study aimed to review tumor control and cranial nerve function outcomes in patients with complex jugular paragangliomas and to refine the surgical strategies for complex jugular paragangliomas.
METHODS
We describe our experience with 12 patients with complex jugular paragangliomas diagnosed in our institution from January 2013 to June 2020. The main outcomes included tumor control, complications, and function of facial nerve and lower cranial nerves, postoperatively.
RESULTS
Gross-total resection was achieved for 9 (75%) patients, and subtotal resection was achieved for 3 (25%) patients. The surgical tumor control rate was 100% after a mean follow-up of 45.5 months (range, 13-111 months). Postoperatively, 10 patients (83.3%) obtained unchanged or improved facial nerve function. However, new lower cranial nerve deficits occurred in 2 patients (16.7%) due to surgical removal of the concurrent vagal paraganglioma and scar tissue enclosing the IX and XII nerves.
CONCLUSION
Our refined surgical techniques, including tension-free anterior facial nerve rerouting, sigmoid sinus tunnel-packing, and pushpacking techniques, could be a choice for the treatment of complex jugular paragangliomas to achieve tumor control and cranial nerves preservation. A 2-stage surgery should be applied to minimize the risk of bilateral cranial neuropathies and the influence on cerebral circulation in patients with bilateral paragangliomas. The preoperative endovascular intervention such as coil embolization or internal carotid artery stenting can be employed for the management of paragangliomas with internal carotid artery-associated lesions.
Topics: Humans; Carotid Stenosis; Treatment Outcome; Retrospective Studies; Stents; Glomus Jugulare Tumor; Paraganglioma
PubMed: 36349670
DOI: 10.5152/iao.2022.22675 -
Annals of Medicine and Surgery (2012) Oct 2021and importance: Head and neck paragangliomas are slowly growing benign tumors and they originate from specialized neural crest cells. We report an unusual combined...
INTRODUCTION
and importance: Head and neck paragangliomas are slowly growing benign tumors and they originate from specialized neural crest cells. We report an unusual combined glomus vagal and jugular tumor that was rarely described in the literature to the best of our knowledge.
CASE PRESENTATION
A 51 years old female with no pathological history was presented to our ENT department with 6 months' history of a right latero cervical swelling gradually increasing in size associated with a swallowing difficulties and hoarseness. Preoperatively clinical examination had found vagal and hypoglossal nerve paralysis. Cervical CT scan and MRI had shown glomus jugular tumor. The patient underwent a surgical excision with severe swallowing difficulties and facial palsy in the immediate postoperative period with a mild recovery afterwards.
CLINICAL DISCUSSION
Paragangliomas of the mesotympanum and jugular foramen most commonly present as a vascular middle ear mass. The most common presenting symptom is pulsatile tinnitus occurring in 80% followed by hearing loss (60%). Dysfunction of cranial nerves traversing the jugular foramen may be commonly encountered with resultant abnormalities of speech, swallowing and airway function. Vagal paragangliomas are the least common of the three primary craniocervical paragangliomas. The most common presenting sign is the presence of a painless neck mass accompanied occasionally by dysphagia and hoarseness. The association of both glomus vagal and jugular tumor is rarely described in the literature to the best of our knowledge.
CONCLUSION
Head and neck paragangliomas are slowly growing benign tumors and they originate from specialized neural crest cells. Vagal paragangliomas are the least common of the three primary craniocervical paragangliomas. The association of both glomus vagal and jugular tumor is rarely described in the literature to the best of our knowledge. The choice of treatment depends on the location, size, and also biologic activity of the tumor as well as the physical condition of the patient.
PubMed: 34691440
DOI: 10.1016/j.amsu.2021.102918