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Clinical Nuclear Medicine Dec 2021We present a case of a 75-year-old woman with clinical suspicion of stroke whose cranial CT showed ischemic changes in right precentral region, without visible vascular...
We present a case of a 75-year-old woman with clinical suspicion of stroke whose cranial CT showed ischemic changes in right precentral region, without visible vascular occlusion, and a right vagal lesion suggestive of paraganglioma. Two weeks later, 68Ga-DOTATOC PET/CT with IV iodine-based contrast administration (Biograph 6 True Point equipment; Siemens) was performed for characterization of this lesion and staging. Intraparenchymal 68Ga-DOTATOC uptake was visualized in right frontal lobe suggestive of hemorrhagic transformation of the previous stroke.
Topics: Aged; Female; Humans; Neuroendocrine Tumors; Octreotide; Organometallic Compounds; Paraganglioma; Positron Emission Tomography Computed Tomography; Stroke
PubMed: 34392278
DOI: 10.1097/RLU.0000000000003846 -
BMJ Case Reports Jul 2021Vagal paragangliomas (VPs) are a rare subset of neuroendocrine tumour derived from paraganglia located in the head/neck that are usually indolent in nature. Typical...
Vagal paragangliomas (VPs) are a rare subset of neuroendocrine tumour derived from paraganglia located in the head/neck that are usually indolent in nature. Typical clinical presentations include pulsatile tinnitus or hoarseness associated with a neck mass. Surgery and radiation therapy remain the primary treatment modalities but come with significant morbidity including cranial nerve damage. We describe a unique case of VP in which cough was the only presenting symptom, that was exacerbated by enlargement of the tumour. Symptoms improved following radiation treatment.
Topics: Cough; Cranial Nerve Neoplasms; Head and Neck Neoplasms; Humans; Paraganglioma; Paraganglioma, Extra-Adrenal
PubMed: 34330721
DOI: 10.1136/bcr-2021-241913 -
Operative Neurosurgery (Hagerstown, Md.) Oct 2021Paragangliomas (PGLs) are benign hypervascular tumors that can develop in head and neck at different locations, primarily in the carotid bifurcation, jugular bulb,...
Paragangliomas (PGLs) are benign hypervascular tumors that can develop in head and neck at different locations, primarily in the carotid bifurcation, jugular bulb, tympanic plexus, and vagal ganglia.1 Different gene mutations have been linked to the familial inherited forms, which can represent approximately 30% of all PGLs.1,2 These are classified into 5 different clinical syndromes: PGL 1 to 5.1 These patients have increased risk for synchronous and metachronous lesions requiring an extensive work-up for hormone secretion and other associated neoplasms, as well as attentive follow-up for lifelong management.1,3 Surgical resection is the best treatment option as it can be curative when the resection is total.2-4 Preservation of the lower cranial nerve function is central to the management of head and neck PGLs, given the gravity of bilateral injuries.3 Irradiation therapy should be considered if the risk for bilateral lower cranial nerve injuries is high.5 Surgically, intrabulbar resection with preservation of the medial wall of the jugular bulb protects the lower cranial nerve function.3 Other technical finesses, including maintaining the facial nerve in its bony fallopian canal (facial bridge), avoiding carotid artery sacrifice, preservation of the ear canal, and preoperative embolization, contributed markedly to outcome improvement.2,3 We report a case of a 34-yr-old male with PGL 3 with a left glomus jugulare tumor that recurred and a right carotid body tumor. Patient consented to surgery and photography. Image at 3:44 republished from Al-Mefty and Teixeira,3 with permission from JSNPG.
Topics: Cranial Nerves; Glomus Jugulare Tumor; Humans; Male; Neoplasm Recurrence, Local; Paraganglioma; Skull Base
PubMed: 34293168
DOI: 10.1093/ons/opab268 -
Annals of Medicine and Surgery (2012) Jun 2021Head and neck paragangliomas are rare vascular tumors derived from the paraganglionic system, located at the carotid body, jugular vein, tympanic cavity and vagal nerve....
Head and neck paragangliomas are rare vascular tumors derived from the paraganglionic system, located at the carotid body, jugular vein, tympanic cavity and vagal nerve. From 2010 to 2020, a cohort of 26 patients divided in two groups, 15 with cervical paragangliomas and 11 with temporal bone paragangliomas, was reviewed by analysing the medical history, the epidemiological and clinical parameters, the imaging results and classification, the modality of treatment and outcome. Cervical paragangliomas present as firm and pulsatile mass with the characteristic aspect of "salt and pepper" on MRI T1 weighted sequences. The most common type on Shamblin classification was the type II. Total surgical resection was performed in 93,33% of cases. The sensitivity of MRI in the diagnosis of vagal paragangliomas was up to 75%, with a specificity of 90,91% and the correlation of the MRI results and the findings of surgical exploration is significant with p ⩽ 0.02. Temporal bone paragangliomas appear as pulsatile mass behind the tympanic membrane, causing variable hearing loss in 90,90% of the cases. The facial nerve is the most frequently affected cranial nerve, in 36,36% of the cases. The main type according to FISH classification is the type B. Embolization was performed in all type C tumors. Surgery was the first line treatment while the inoperable patients were considered for radiotherapy. The aim of this study is to report the main clinical features of head and neck paragangliomas, the imaging tools and findings evaluating their sensitivity and specificity and the treatment protocol and outcome.
PubMed: 34094530
DOI: 10.1016/j.amsu.2021.102412 -
Journal of Ultrasound Sep 2021Paragangliomas are a rare form of highly vascularized tumors that originate from paraganglia Baysal (J Med Genet 39: 617-622, 2002). In the head and neck PGL arise...
Paragangliomas are a rare form of highly vascularized tumors that originate from paraganglia Baysal (J Med Genet 39: 617-622, 2002). In the head and neck PGL arise primarily in four distinct areas: vagal, middle ear, and larynx and more frequently carotid bifurcation. Imaging evaluations include sonography, color Doppler, US-elastosonography and contrast-enhanced ultrasound (CEUS). Additionally, Computed Tomography, Magnetic Resonance Imaging (MRI) as well as digital subtraction angiography can be performed Stoeckli et al. (Laryngoscope 112: 143-146, 2002). We present herein a case of a rare bilateral carotid body tumor assessed with multiparametric ultrasound evaluation, including CEUS and US-elastography.
Topics: Angiography, Digital Subtraction; Carotid Body Tumor; Humans; Magnetic Resonance Imaging; Tomography, X-Ray Computed; Ultrasonography
PubMed: 33999368
DOI: 10.1007/s40477-021-00581-z -
Journal of Voice : Official Journal of... Jul 2023We present a prospective case series that aimed to report the functional (voice and swallowing) outcomes of delayed laryngeal reinnervation following vagal interruption...
PURPOSE
We present a prospective case series that aimed to report the functional (voice and swallowing) outcomes of delayed laryngeal reinnervation following vagal interruption by resection of vagal paraganglioma and schwannoma.
MATERIALS AND METHODS
A dedicated, anonymized database was established in 2012 with a minimum eighteen-month follow up set for this report. Internationally validated self- and observer-reported measures were recorded preoperatively and at six, 12 and, 18 months together with demographics, diagnoses, and operative details.
RESULTS
A total of eight patients with a median age of 46 (37-54) underwent excision of vagal paraganglioma (five) and schwannoma (three) with few mild complications. Three underwent selective and five non selective reinnervation. Seven out of eight patients underwent synchronous injection medialization. The voice handicap index (VHI-30) improved from a baseline median 83 (range 52-102) to 7.5 (5-58) at 18 months; maximum phonation time improved from median 8 (range 5-15) to 10.5 (8.5-11); voice grade ("G" in grade, roughness, breathiness, asthenia, and strain [GRBAS] scoring) improved from median three (severe impairment, range 0-3) to one (mild impairment, 0-2); Eating Assessment Tool (EAT-10) score improved from median 12 (range 3.5-27) preoperatively to one (0-16); and reflux symptom index (RSI) improved from median 25 (range 17-36) to 7 (0-36). One patient exhibited no discernible reinnervation, while the remainder exhibited good cord bulk and tone, though without purposive abduction.
CONCLUSION
Delayed laryngeal reinnervation for high vagal paralysis is a safe technique associated with good voice and swallowing outcomes by 12-18 months. Potential confounders in this small series and the absence of a control arm both limit conclusions, but this study suggests that further prospective, controlled studies, and/or case registration are merited.
Topics: Humans; Vocal Cord Paralysis; Treatment Outcome; Larynx; Paraganglioma; Neurilemmoma; Recurrent Laryngeal Nerve
PubMed: 33994255
DOI: 10.1016/j.jvoice.2021.03.021 -
Journal of Surgical Case Reports Apr 2021Paragangliomas (PGs) are extremely rare multicentric neoplasms. Hereditary or familial PGs are associated with germline mutations in succinate dehydrogenase genes, seen...
Paragangliomas (PGs) are extremely rare multicentric neoplasms. Hereditary or familial PGs are associated with germline mutations in succinate dehydrogenase genes, seen in one-third of cases. Primary PGs of the thyroid are uncommon neuroendocrine neoplasms that account for 0.012% of all head and neck lesions. Although majority of these tumors are solitary, familial PGs are associated with synchronous tumors (carotid/vagal). We report an interesting case of primary thyroid PG in a patient with a previous history of a right carotid body, right vagal PGs and positive familial history, confining the differential diagnosis to recurrent lesions, which is the most common occurrence or new primary or a metastatic lesion. However, long interval and surgical anatomy suggests the diagnosis to be a primary lesion. In conclusion, although these lesions present multicentrically present at varying intervals, their occurrence at anatomically distinct sites should raise the concern for a new primary PG.
PubMed: 33936587
DOI: 10.1093/jscr/rjab102 -
Ear, Nose, & Throat Journal Jun 2023Extirpation of multiple head and neck paragangliomas carries challenge due to close anatomic relationships with critical neurovascular bundles.
INTRODUCTION
Extirpation of multiple head and neck paragangliomas carries challenge due to close anatomic relationships with critical neurovascular bundles.
OBJECTIVES
This study aims to assess whether the application of 3-D models can assist with surgical planning and treatment of these paragangliomas, decrease surgically related morbidity and mortality.
METHODS
Fourteen patients undergoing surgical resection of multiple head and neck paragangliomas were enrolled in this study. A preoperative 3-D model was created based on radiologic data, and relevant critical anatomic relationships were preoperatively assessed and intraoperatively validated.
RESULTS
All 14 patients presented with multiple head and neck paragangliomas, including bilateral carotid body tumors (CBT, n = 9), concurrent CBT with glomus jugulare tumors (GJT, n = 4), and multiple vagal paragangliomas (n = 1). Ten patients underwent genomic analysis and all harbored succinate dehydrogenase complex subunit D (SDHD) mutations. Under guidance of the 3-D model, the internal carotid artery (ICA) was circumferentially encased by tumor on 5 of the operated sides, in 4 (80%) of which the tumor was successfully dissected out from the ICA, whereas ICA reconstruction was required on one side (20%). Following removal of CBT, anterior rerouting of the facial nerve was avoided in 3 (75%) of 4 patients during the extirpation of GJT with assistance of a 3-D model. Two patients developed permanent postoperative vocal cord paralysis. There was no vessel rupture or mortality in this study cohort.
CONCLUSION
The 3-D model is beneficial for establishment of a preoperative strategy, as well as planning and guiding the intraoperative procedure for resection of multiple head and neck paragangliomas.
Topics: Humans; Head and Neck Neoplasms; Paraganglioma, Extra-Adrenal; Paraganglioma; Carotid Body Tumor; Glomus Jugulare Tumor
PubMed: 33829883
DOI: 10.1177/01455613211009441 -
Journal of Neurosciences in Rural... Jan 2021The aim of the study is to determine the surgical candidacy and nuances of skull base paraganglioma surgery in the era of radiotherapy. This was a retrospective...
The aim of the study is to determine the surgical candidacy and nuances of skull base paraganglioma surgery in the era of radiotherapy. This was a retrospective observational study conducted in patients who presented with skull base paragangliomas between January 2017 and December 2019. Primary data, including indication for surgery, the approach used, the extent of resection, complications, and postoperative lower cranial nerve status were studied. A total of 21 cases of skull base paragangliomas were analyzed, including seven cases of tympanic paraganglioma, 10 cases of jugular foramen paraganglioma, three cases of multiple paragangliomas, and one case of vagal paraganglioma. Indications for surgery were young age, bleeding from ear, neck mass with upper aerodigestive pressure symptoms, lower cranial nerve paralysis, and patients with intracranial pressure symptoms. Total excision was done in 11 patients, near-total excision in five patients, subtotal in three patients, and surgery was not done in two patients. Facial nerve paralysis was the most common complication observed, followed by bleeding and flap necrosis. Radiotherapy was considered as adjuvant treatment wherever indicated. A thorough knowledge and understanding of the pathophysiology of the skull base paragangliomas and its management strategies can help to achieve excellent results in terms of tumor clearance and reduction in complications. A multidisciplinary team approach and meticulous skull base surgical techniques have a significant role to play in the management of paragangliomas, especially in developing countries where availability of radiosurgery is still a challenge.
PubMed: 33531769
DOI: 10.1055/s-0040-1721201 -
Frontiers in Genetics 2020Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumors that have a high degree of heritability and are predominantly associated with mutations in ten...
Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumors that have a high degree of heritability and are predominantly associated with mutations in ten genes, such as , and . Elucidating the mutation prevalence is crucial for the development of genetic testing. In this study, we identified pathogenic/likely pathogenic variants in the main susceptibility genes in 102 Russian patients with HNPGLs (82 carotid and 23 vagal paragangliomas) using whole exome sequencing. Pathogenic/likely pathogenic variants were detected in 43% (44/102) of patients. We identified the following variant distribution of the tested genes: (1%), (10%), (5%), (24.5%), and (5%). variants were observed in the majority of the patients with bilateral/multiple paragangliomas. Thus, among Russian patients with HNPGLs the most frequently mutated gene was followed by , and .
PubMed: 33391357
DOI: 10.3389/fgene.2020.614908