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Journal of Clinical Neuroscience :... Feb 2024Head and Neck Paragangliomas are characterized by having a rich blood supply. Presurgical embolization with Onyx as a neoadjuvant treatment is not a consensus regarding... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Head and Neck Paragangliomas are characterized by having a rich blood supply. Presurgical embolization with Onyx as a neoadjuvant treatment is not a consensus regarding its efficacy and safety. Our study aimed to answer this matter through a single-arm meta-analysis.
METHODS
We systematically reviewed 4 databases. Sixteen studies were described and suitable papers were selected for meta-analysis of estimated intraoperative blood loss (EBL), percentage of tumor devascularization, and complications associated with embolization.
RESULTS
The study identified 198 patients with 203 tumors, aged between 8 and 70 years. Commonly reported symptoms included neck mass perception and cranial nerve impairment. Carotid Body Tumors were most prevalent (127, 62.5 %), followed by jugular (48, 23.6 %), or vagal (29, 14.2 %) tumors. Eight studies reported estimated intraoperative blood loss (EBL) averaging 261.89 ml (95 %CI: 128.96 to 394.81 ml). In an analysis of 9 studies, 99 % (95 %CI: 96 to 100 %) achieved 70 % or more devascularization, and 79 % (95 %CI: 58 to 100 %) achieved 90 % or more devascularization. Complications from endovascular procedures were observed in 3 % (95 %CI: 0 to 8 %) of 96 patients across 10 studies, including 4 facial nerve deficits. Eighteen postoperative neurological deficits were reported across 15 articles.
CONCLUSION
Despite acknowledged limitations, with refined indications, EVOH, especially Onyx embolization may significantly bolster patient safety, decreasing EBL and easing surgical resection. Further research with larger studies will refine criteria, optimize techniques, and improve patient care and treatment outcomes in the management of head and neck paragangliomas.
Topics: Humans; Child; Adolescent; Young Adult; Adult; Middle Aged; Aged; Blood Loss, Surgical; Paraganglioma; Carotid Body Tumor; Embolization, Therapeutic; Head and Neck Neoplasms; Treatment Outcome; Retrospective Studies
PubMed: 38244529
DOI: 10.1016/j.jocn.2024.01.017 -
Cardiovascular and Interventional... Apr 2024This study aims to provide a comprehensive review of the clinical benefits, complications, and safety profile associated with preoperative embolization in Glomus... (Meta-Analysis)
Meta-Analysis Review
PURPOSE
This study aims to provide a comprehensive review of the clinical benefits, complications, and safety profile associated with preoperative embolization in Glomus jugulare tumors (GJTs).
MATERIALS AND METHODS
A comprehensive search in PubMed, Embase, and Web of Science was conducted for English articles published up to March 2023, focusing on GJTs and preoperative embolization. Included studies involved patients over 18 with GJTs. We excluded studies that explored embolization methods other than the standard endovascular approach, as well as studies involving paragangliomas that did not provide specific data related to GJTs. Key variables such as hemorrhage volume and surgical time, as well as clinical outcomes, were analyzed. Data were analyzed using a random-effects model meta-analysis, assessing heterogeneity with the I statistic.
RESULTS
This review encompasses 19 studies with a total of 328 patients. The studies incorporated into our meta-analysis display considerable differences and inconsistencies in their data. The findings of the meta-analysis show a mean hemorrhage volume of 636 ml (95% confidence interval (CI) 473-799) following preoperative embolization, and a mean surgical duration of 487 min (95% CI 350-624). The study also notes potential complications: facial nerve deficits occurred in 20% of cases (95% CI 11-32%), and vagal nerve deficits in 22% (95% CI 13-31%).
CONCLUSION
This study suggests that preoperative embolization could decrease surgery duration and blood loss, but emphasizes the importance of evaluating risks like nerve damage. However, the generalizability of these findings is restricted due to the diversity of available data.
Topics: Humans; Glomus Jugulare Tumor; Embolization, Therapeutic; Hemorrhage; Treatment Outcome; Retrospective Studies
PubMed: 38528173
DOI: 10.1007/s00270-024-03687-z -
Frontiers in Endocrinology 2024Vagal paraganglioma (VPGL) is a rare neuroendocrine tumor that originates from the paraganglion associated with the vagus nerve. VPGLs present challenges in terms of...
Vagal paraganglioma (VPGL) is a rare neuroendocrine tumor that originates from the paraganglion associated with the vagus nerve. VPGLs present challenges in terms of diagnostics and treatment. VPGL can occur as a hereditary tumor and, like other head and neck paragangliomas, is most frequently associated with mutations in the genes. However, data regarding the genetics of VPGL are limited. Herein, we report a rare case of a 41-year-old woman with VPGL carrying a germline variant in the gene. Using whole-exome sequencing, a variant, p.S249R, was identified; no variants were found in other PPGL susceptibility and candidate genes. Loss of heterozygosity analysis revealed the loss of the wild-type allele of the gene in the tumor. The pathogenic effect of the p.S249R variant on FH activity was confirmed by immunohistochemistry for S-(2-succino)cysteine (2SC). Potentially deleterious somatic variants were found in three genes, , , and . The latter two encode transcriptional regulators that can impact gene expression deregulation and are involved in tumor development and progression. Moreover, -mutated VPGL was characterized by a molecular phenotype different from -mutated PPGLs. In conclusion, the association of genetic changes in the gene with the development of VPGL was demonstrated. The germline variant : p.S249R and somatic deletion of the second allele can lead to biallelic gene damage that promotes tumor initiation. These results expand the clinical and mutation spectra of -related disorders and improve our understanding of the molecular genetic mechanisms underlying the pathogenesis of VPGL.
Topics: Adult; Female; Humans; Acid Anhydride Hydrolases; Cranial Nerve Neoplasms; Exome Sequencing; Germ-Line Mutation; Paraganglioma; Vagus Nerve Diseases
PubMed: 38721148
DOI: 10.3389/fendo.2024.1381093 -
OTO Open 2024High-altitude natives have a high incidence of parangangliomas (PGL) of the head and neck, especially the carotid body tumor. The aim of this study is to describe the...
OBJECTIVES
High-altitude natives have a high incidence of parangangliomas (PGL) of the head and neck, especially the carotid body tumor. The aim of this study is to describe the clinical presentation, pattern, altitude of residence, distribution, management, and follow-up of head and neck paragangliomas (HNPGL) in our sub-Himalayan population.
STUDY DESIGN
Retrospective cohort study.
SETTING
Academic tertiary care hospital.
METHODS
Hospital records of 20 patients of HNPGL diagnosed from December 2017 to December 2021 were retrieved for analysis.
RESULTS
Twenty patients with 23 HNPGL, with a mean age of 41.74 years were managed in our institute. The female-to-male ratio was 2.3: 1 and the mean follow-up was 29.95 months. Nine had carotid body (CBPGL), 7 had tympanic (TPGL), 2 had jugular (JPGL), and 2 had vagal paragangliomas (VPGL). Multiple PGL were seen in 4 patients (20%). Majority of cases (all CBPGL and 57.14% of TPGL) were residents of the high altitude, and the rest were from the low altitude. Fifteen patients (8 CBPGL, 7 TPGL) were operated. There were no major complications except in a patient with large carotid body tumor required anastomosis of carotid artery. Five patients received stereotactic radiotherapy, and 1 malignant PGL received chemoradiotherapy.
CONCLUSION
In this study, JPGL and VPGL are common at low altitudes, whereas carotid body and tympanic PGL were the most common tumor at high altitudes. Being a retrospective and study small sample size, a definite conclusion is not established, however, a genetic analysis and inclusion of a wider population in a future prospective study may establish the hypothesis.
PubMed: 38357702
DOI: 10.1002/oto2.112 -
International Journal of Surgery Case... Dec 2023Paragangliomas are rare tumors that most commonly occur in the head and neck. They are typically slow-growing tumors that are often associated with genetic syndromes and...
INTRODUCTION AND SIGNIFICANCE
Paragangliomas are rare tumors that most commonly occur in the head and neck. They are typically slow-growing tumors that are often associated with genetic syndromes and mutations in the enzyme succinate dehydrogenase.
CASE PRESENTATION
We describe a case of a male patient who presented with ear pain and dysphagia. On physical examination, there was a left neck mass and swalling in the left oropharynx. The mass was excised through a cervical approach and it was found to be a vagal paraganglioma.
CLINICAL DISCUSSION
A few cases of head and neck paragangliomas (HNPGLs) have been reported in the medical literature. These tumors often present as asymptomatic masses, but this case, the patient presented with ear pain and dysphagia the location of the mass in the parapharyngeal space presents a surgical challenge. HNPGLs should be considered in the differential diagnosis of any neck mass.
CONCLUSION
Paragangliomas are generally considered to have a good prognosis when they are completely excised. They rarely metastasize to adjacent or distant tissues. Cervical approach is a valid option for complete excision of cervical paraganglioma.
PubMed: 37984258
DOI: 10.1016/j.ijscr.2023.109025 -
Clinical Endocrinology May 2024Head and neck paragangliomas (HNPGLs) are rare, usually benign, slow-growing tumours arising from neural crest-derived tissue. Definitive management pathways for HNPGLs...
CONTEXT
Head and neck paragangliomas (HNPGLs) are rare, usually benign, slow-growing tumours arising from neural crest-derived tissue. Definitive management pathways for HNPGLs have yet to be clearly defined.
OBJECTIVE
To review our experience of the clinical features and management of these tumours and to analyse outcomes of different treatment modalities.
METHODS
Demographic and clinical data were obtained from The Northern Ireland Electronic Care Record (NIECR) as well from a prospectively maintained HNPGL database between January 2011 through December 2023.
RESULTS
There were 87 patients; 50 females: 37 males with a mean age of 52.3 ± 14.2 years old (range 17-91 years old). 58.6% (n = 51) of patients had carotid body tumours, 25.2% (n = 22) glomus vagal tumours, 6.8% (n = 6) tumours in the middle ear, 2.2% (n = 2) in the parapharyngeal space and 1.1% (n = 1) in the sphenoid sinus. 5.7% (n = 5) of patients had multifocal disease. The mean tumour size at presentation was 3.2 ± 1.4 cm (range 0.5-6.9 cm). Pathogenic SDHD mutations were identified in 41.3% (n = 36), SDHB in 12.6% (n = 11), SDHC in 2.2% (n = 2) and SDHA in 1.1% (n = 1) of the patients. Overall treatment modalities included surgery alone in 51.7% (n = 45) of patients, radiotherapy in 14.9% (n = 13), observation in 28.7% (n = 25), and somatostatin analogue therapy with octreotide in 4.5% (n = 4) of patients. Factors associated with a significantly higher risk of recurrence included age over 60 years (p = .04), tumour size exceeding 2 cm (p = .03), positive SDHx variants (p = .01), and vagal and jugular tumours (p = .04).
CONCLUSION
The majority of our patients underwent initial surgical intervention and achieved disease stability. Our results suggest that carefully selected asymptomatic or medically unfit patients can be safely observed provided lifelong surveillance is maintained. We advocate for the establishment of a UK and Ireland national HNPGL registry, to delineate optimal management strategies for these rare tumours and improve long term outcomes.
PubMed: 38696538
DOI: 10.1111/cen.15070 -
Cureus Mar 2024Paragangliomas (PGLs) are tumors that are rarely malignant; the majority of them are benign. Similar to pheochromocytoma, they develop from the autonomic nerve system....
Paragangliomas (PGLs) are tumors that are rarely malignant; the majority of them are benign. Similar to pheochromocytoma, they develop from the autonomic nerve system. This system originates from neural crest cells and can undergo neoplastic transformation. PGLs can arise either inside or outside the adrenal glands. Head and neck PGLs are very scarce. The primary locations where this tumor commonly originates within this region are the carotid body, jugular bulb, and vagal body. Hence, in our case report, we attempt to highlight the uncommon presentation of this disease in a 46-year-old female, who initially presented with hypertension and persistent dysphonia. The patient underwent successful external radiotherapy. This case report aims to raise awareness of the characteristics of these rare malignancies.
PubMed: 38586626
DOI: 10.7759/cureus.55720 -
AJNR. American Journal of Neuroradiology May 2024The impact of therapeutic embolization as a stand-alone treatment of head and neck paragangliomas considered surgically high-risk remains insufficiently understood. The...
BACKGROUND AND PURPOSE
The impact of therapeutic embolization as a stand-alone treatment of head and neck paragangliomas considered surgically high-risk remains insufficiently understood. The aim of this study was to investigate the procedural risks and long-term volumetric development in head and neck paragangliomas with high surgical risk following therapeutic endovascular embolization as stand-alone treatment.
MATERIALS AND METHODS
A retrospective database review of patients who underwent endovascular embolization as primary treatment for head and neck paragangliomas lacking appropriate curative treatment options at our institution (from January 2000 to February 2023) was conducted. Tumor volumetric analyses were performed before embolization and during follow-up. To assess the changes in tumor volume over time, the measurements were performed after embolization, first at 6 months and then on a yearly basis up to 6 years (mean follow-up time was: 33.7 ± 24.4 months). Subgroup analyses were conducted for vagal and jugular/jugulotympanic paragangliomas.
RESULTS
A total of 32 head and neck paragangliomas in 28 patients (mean age, 56.1 years ± 16.5 [standard deviation]; 18 female) with therapeutic embolization as stand-alone treatment were evaluated, of which 11 were vagal paragangliomas, 15 jugular/jugulotympanic paragangliomas and 6 carotid body tumors. After a mean follow-up duration of 33.7 ± 24.4 months tumor control was achieved in 75%, with significant median tumor volume reduction at 6 months (p = .02, n = 21). Vagal paragangliomas responded the most to embolization with a significantly decreased median volume from 22.32 cm; to 19.09 cm (p = .008, n = 8). Transient complications occurred in 3.4%.
CONCLUSIONS
Therapeutic embolization as a stand-alone treatment offers a low-risk control of tumor growth in surgically high-risk lesions, with a significant reduction in tumor volume after treatment. Among the different subtypes, vagal paragangliomas exhibited the strongest and longest regression of the tumor volume. HNPGL = head and neck paragangliomas; IQR = interquartile range; JTP = jugular/jugulotympanic paragangliomas; PVA = polyvinyl alcohol; VP = vagal paragangliomas.
PubMed: 38719606
DOI: 10.3174/ajnr.A8328 -
WMJ : Official Publication of the State... Sep 2023Radiation therapy may result in complications, including fibrosis, which can result in pain and difficulty with movement - especially in the neck.
BACKGROUND
Radiation therapy may result in complications, including fibrosis, which can result in pain and difficulty with movement - especially in the neck.
CASE PRESENTATION
A 52-year-old man with right-sided vague neck pain unresponsive to conservative management had a computed tomography scan that showed a vagal paraganglioma in the carotid sheath surrounding the right carotid arteries and internal jugular vein. Following radiation therapy, he noticed a new pain in his right jaw and neck worse with certain movements of the neck. Nonsurgical conservative measures including physical therapy and pharmacological management were unsuccessful. An ultrasound evaluation demonstrated fibrosis beneath the sternocleidomastoid muscle and in proximity to the carotid sheath. After careful trajectory planning using ultrasound imaging, a 25 G needle was introduced real time in proximity to the fibrosis. Using a dexamethasone/saline mixture under real-time ultrasound guidance, adhesions were released. After 3 injections, the patient reported greater than 90% pain relief, which lasted 4 months. Subsequently, he required similar injections approximately every 3 months to achieve greater than 75% pain relief.
CONCLUSIONS
This is a successful demonstration of the utility of ultrasound evaluation and guidance for adhesiolysis following radiation therapy.
Topics: Male; Humans; Middle Aged; Neck Pain; Radiation Fibrosis Syndrome; Ultrasonography; Pain Management; Ultrasonography, Interventional
PubMed: 37768775
DOI: No ID Found -
Acta Neurochirurgica Oct 2023Drilling injuries of the inner ear are an underreported complication of lateral skull base (LSB) surgery. Inner ear breaches can cause hearing loss, vestibular...
PURPOSE
Drilling injuries of the inner ear are an underreported complication of lateral skull base (LSB) surgery. Inner ear breaches can cause hearing loss, vestibular dysfunction, and third window phenomenon. This study aims to elucidate primary factors causing iatrogenic inner ear dehiscences (IED) in 9 patients who presented to a tertiary care center with postoperative symptoms of IED following LSB surgery for vestibular schwannoma, endolymphatic sac tumor, Meniere's disease, paraganglioma jugulare, and vagal schwannoma.
METHODS
Utilizing 3D Slicer image processing software, geometric and volumetric analysis was applied to both preoperative and postoperative imaging to identify causal factors iatrogenic inner ear breaches. Segmentation analyses, craniotomy analyses, and drilling trajectory analyses were performed. Cases of retrosigmoid approaches for vestibular schwannoma resection were compared to matched controls.
RESULTS
Excessive lateral drilling and breach of a single inner ear structure occurred in 3 cases undergoing transjugular (n=2) and transmastoid (n=1) approaches. Inadequate drilling trajectory breaching ≥1 inner ear structure occurred in 6 cases undergoing retrosigmoid (n=4), transmastoid (n=1), and middle cranial fossa approaches (n=1). In retrosigmoid approaches the 2-cm visualization window and craniotomy limits did not provide drilling angles to the entire tumor without causing IED in comparison to matched controls.
CONCLUSIONS
Inappropriate drill depth, errant lateral drilling, inadequate drill trajectory, or a combination of these led to iatrogenic IED. Image-based segmentation, individualized 3D anatomical model generation, and geometric and volumetric analyses can optimize operative plans and possibly reduce inner ear breaches from lateral skull base surgery.
Topics: Humans; Neuroma, Acoustic; Ear, Inner; Neurosurgical Procedures; Skull Base; Iatrogenic Disease
PubMed: 37430067
DOI: 10.1007/s00701-023-05695-3