-
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 -
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 -
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 -
The American Journal of Case Reports Oct 2023BACKGROUND Paragangliomas are neuroendocrine neoplasms derived from paraganglia of the sympathetic and parasympathetic nervous systems. Parasympathetic ganglia-derived...
BACKGROUND Paragangliomas are neuroendocrine neoplasms derived from paraganglia of the sympathetic and parasympathetic nervous systems. Parasympathetic ganglia-derived tumors, also called non-chromaffin, are located almost exclusively in the neck and skull base and are usually non-secretory and inactive. A case of malignant glomus jugulare with a metastatic cervical lymph node is described here. CASE REPORT A 24-year-old woman was referred to an otolaryngology clinic for concern of voice change for 1 month, which was associated with right progressive hearing loss, pulsating tinnitus, and right facial weakness. A clinical examination revealed a reddish mass in the right ear behind an intact tympanic membrane with right facial weakness of House-Brackmann grade VI. A bedside flexible nasopharyngoscopy revealed an immobile right vocal fold. A computed tomography scan of the brain revealed a destructive lesion within the right jugular foramen. The patient underwent embolization followed by glomus tumor resection via infra-temporal fossa with Fisch type A approach. Pathology revealed that the tumor was an infiltrative epithelioid tumor with a spindle and nesting pattern separated by fibrovascular stroma. The submitted lateral neck lymph node revealed a metastatic tumor. CONCLUSIONS Glomus jugulare tumors are uncommon paragangliomas, and malignant behavior with metastasis is extremely rare. Metastatic tumors are often associated with facial and vagal nerves palsy. There are no histological features that distinguish malignant glomus jugulare tumors. Malignant neoplasms are characterized by the presence of metastases. Tumors of the glomus jugulare that are malignant are treated with surgery, radiotherapy, or both. However, our search of the literature revealed no clear guidelines, given the scarcity of cases. Moreover, the presence of metastasis increases the risk of death.
Topics: Female; Humans; Young Adult; Adult; Glomus Jugulare Tumor; Glomus Tumor; Paraganglioma; Facial Paralysis; Skull Base; Neoplasms, Second Primary; Sarcoma
PubMed: 37814445
DOI: 10.12659/AJCR.940138 -
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 Endocrinologica (Bucharest,... 2023Paragangliomas are rare neuroendocrine slow-growing tumors, often asymptomatic, that originate from embryonic neural crest cell. In the head and neck area, the most...
UNLABELLED
Paragangliomas are rare neuroendocrine slow-growing tumors, often asymptomatic, that originate from embryonic neural crest cell. In the head and neck area, the most common location is the carotid body, followed, with decreasing frequency, in jugular, tympanic and vagal sites. Bilateral carotid body tumors are extremely rare.
AIM
To present the most important features of carotid body paragangliomas, illustrating the clinical characteristics, associated with a thorough analysis of the diagnostic imaging elements, but also the current therapeutic strategies, with respective anatomical, surgical considerations and potential complications that can occur. Surgical resection is the main line of treatment. The complex anatomy of the cervical region and the close relationships of carotid body paragangliomas with carotid vessels and cranial nerves, as well as its intense vascularization makes the surgical intervention a real challenge even for an experienced surgeon.
DISCUSSION
Starts from a bilateral carotid paraganglioma in a 35-year-old male, with painless lateral neck swelling, accidentally discovered by his barber about two years ago. Diagnosis was suspected on the basis of history, clinical and radiological findings. "Wait and scan" strategy plus endocrinologic assessment for MEN syndromes were considered the optimal therapeutic approach in this case.
PubMed: 37601710
DOI: 10.4183/aeb.2023.133 -
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 -
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