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Acta Medica Portuguesa Nov 2022Paragangliomas are usually benign slow-growing tumors, but they are locally invasive and can cause significant morbidity. The aim of this study was to characterize the...
INTRODUCTION
Paragangliomas are usually benign slow-growing tumors, but they are locally invasive and can cause significant morbidity. The aim of this study was to characterize the presenting symptoms, secretory status, genetics, imaging features, treatment modalities, post-treatment complications and survival of patients with head and neck paragangliomas treated at a single institution.
MATERIAL AND METHODS
We retrospectively reviewed the clinical records of patients managed at our center between 1997 and 2020.
RESULTS
Seventy-three patients were included in the study, encompassing 89 head and neck paragangliomas. Forty-eight patients (65.8%) were female and 15 (20.5%) had multiple tumor sites (including 10 patients with multicentric benign paragangliomas and five with disseminated malignant disease). Regarding location, our series encompassed 40 temporal bone paragangliomas (44.9%), 24 carotid body paragangliomas (27%), 22 vagal paragangliomas (24.7%), two laryngeal paragangliomas (2.2%) and one sinonasal paraganglioma (1.1%). Excessive catecholamine secretion was detected in 11 patients (15.1%). Sixty-four patients (87.7%) underwent genetic testing. Of those, 24 (37.5%) exhibited pathogenic succinate dehydrogenase complex germline mutations. Regarding patients who presented with untreated disease, 45 patients (66.2%), encompassing 55 tumors, underwent surgery as primary treatment modality, 20 (29.4%; 23 tumors) were initially treated with radiotherapy and three patients (4.4%, encompassing three solitary tumors) were kept solely under watchful waiting. Five-year overall survival was 94.9% and disease-free survival was 31.9%.
CONCLUSION
Head and neck paragangliomas are rare, slow-growing but locally aggressive tumors resulting in high morbidity but low mortality rates.
Topics: Humans; Female; Male; Retrospective Studies; Head and Neck Neoplasms; Paraganglioma; Paraganglioma, Extra-Adrenal; Carotid Body Tumor
PubMed: 35290759
DOI: 10.20344/amp.17185 -
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 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 -
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 -
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 -
The Laryngoscope Mar 2023Head & Neck Paragangliomas have been historically relying on surgery mostly, with worsened quality of life and major sequelae. Conventional external radiation therapy...
OBJECTIVES
Head & Neck Paragangliomas have been historically relying on surgery mostly, with worsened quality of life and major sequelae. Conventional external radiation therapy seems to offer an equivalent control rate with a low toxicity profile. The aim of this study was to assess the safety and efficiency of intensity-modulated radiation therapy in Head & Neck paragangliomas.
METHODS
This is a retrospective monocentric study conducted in a referral center, including all patients treated with IMRT, whether as an exclusive or post-operative treatment for a tympanic and jugular, carotid, or vagal paraganglioma. Data collection was performed through the manuscript and computerized medical files, including consultation, operative, imaging, pathological analyses, delineation, and treatment planning reports. Success was defined as the complete or partial regression or stabilization without progression, or relapse in accordance with the RECIST criteria. Acute toxicities and long-term sequelae were assessed.
RESULTS
Our cohort included 39 patients included between 2011 and 2021: 18 patients treated for a TJ PG (45.9%), 11 patients for a carotid PG (28.4%), and 9 for a vagal PG (23.1%). Twenty-nine patients had IMRT as an exclusive treatment (74.4%), whereas 10 patients had a post-operative complementary treatment (25.6%). Median follow-up in our cohort was 2318 days (average = 2200 days, 237-5690, sd = 1281.9). Among 39 patients, 37 were successfully controlled with IMRT (94.8%), and the toxicity profile was low without any major toxicity.
CONCLUSION
IMRT seems an ideal treatment, whether exclusive or post-operative for Head & Neck paragangliomas.
LEVEL OF EVIDENCE
3 Laryngoscope, 133:607-614, 2023.
Topics: Humans; Radiotherapy, Intensity-Modulated; Retrospective Studies; Quality of Life; Head and Neck Neoplasms; Neoplasm Recurrence, Local; Paraganglioma
PubMed: 35638238
DOI: 10.1002/lary.30226 -
European Journal of Nuclear Medicine... Jun 2024
PubMed: 38829375
DOI: 10.1007/s00259-024-06776-w -
Endocrine-related Cancer Mar 2022Head and neck paragangliomas (HNPGLs) are tumors of parasympathetic origin that occur at variable locations and are often secondary to germline mutations in succinate...
Head and neck paragangliomas (HNPGLs) are tumors of parasympathetic origin that occur at variable locations and are often secondary to germline mutations in succinate dehydrogenase (SDH) subunit genes. Occasionally, these tumors produce catecholamines. Here, we assessed whether different locations of HNPGLs relate to the presence of SDHx mutations, catecholamine production and other presentations. In this multicenter study, we collected clinical and biochemical data from 244 patients with HNPGLs and 71 patients without HNPGLs. We clarified that jugulotympanic HNPGLs have distinct features. In particular, 88% of jugulotympanic HNPGLs arose in women, among whom only 24% occurred due to SDHx mutations compared to 55% in men. Jugulotympanic HNPGLs were also rarely bilateral, were of a smaller size and were less often metastatic compared to carotid body and vagal HNPGLs. Furthermore, we showed that plasma concentrations of methoxytyramine (MTY) were higher (P < 0.0001) in patients with HNPGL than without HNPGL, whereas plasma normetanephrine did not differ. Only 3.7% of patients showed strong increases in plasma normetanephrine. Plasma MTY was positively related to tumor size but did not relate to the presence of SDHx mutations or tumor location. Our findings confirm that increases in plasma MTY represent the main catecholamine-related biochemical feature of patients with HNPGLs. We expect that more sensitive analytical methods will make biochemical testing of HNPGLs more practical in the future and enable more than the current 30% of patients to be identified with dopamine-producing HNPGLs. The sex-dependent differences in the development of HNPGLs may have relevance to the diagnosis, management and outcomes of these tumors.
Topics: Catecholamines; Dopamine; Female; Head and Neck Neoplasms; Humans; Male; Mutation; Normetanephrine; Paraganglioma; Succinate Dehydrogenase
PubMed: 35171114
DOI: 10.1530/ERC-21-0359 -
Diagnostics (Basel, Switzerland) Dec 2021Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumors, comprising only 3% of all head and neck tumors. Early diagnosis forms an integral part of the...
Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumors, comprising only 3% of all head and neck tumors. Early diagnosis forms an integral part of the management of these tumors. The two main aims of any treatment approach are long-term tumor control and minimal cranial nerve morbidity. The scope of this article is to present our case series of HNPGLs to stress most important clinical aspects of their presentation as well as critical issues of their complex management. Thirty patients with suspected HNPGLs were referred to our otorhinolaryngology clinic for surgical consultation between 2016-2020. We assessed the demographical pattern, clinicoradiological correlation, as well as type and outcome of treatment. A total of 42 non-secretory tumors were diagnosed-16.7% were incidental findings and 97% patients had benign tumors. Six patients had multiple tumors. Jugular paragangliomas were the most commonly treated tumors. Tumor control was achieved in nearly 96% of operated patients with minimal cranial nerve morbidity. Surgery is curative in most cases and should be considered as frontline treatment modality in experienced hands for younger patients, hereditary and secretory tumors. Cranial nerve dysfunction associated with tumor encasement is a negative prognostic factor for both surgery and radiotherapy. Multifocal tumors and metastasis are difficult to treat, even with early detection using genetic analysis. Detecting malignancy in HNPGLs is challenging due to the lack of histomorphological criteria; therefore, limited lymph node dissection should be considered, even in the absence of clinical and radiological signs of metastasis in carotid body, vagal, and jugular paragangliomas.
PubMed: 35054195
DOI: 10.3390/diagnostics12010028 -
Otolaryngology--head and Neck Surgery :... Jun 2020To provide the first description of hypofractionated stereotactic radiosurgery (SRS) and evaluate tumor control and safety for vagal paragangliomas (VPs), which begin at...
OBJECTIVE
To provide the first description of hypofractionated stereotactic radiosurgery (SRS) and evaluate tumor control and safety for vagal paragangliomas (VPs), which begin at the skull base but often have significant extracranial extension.
STUDY DESIGN
Retrospective chart review.
SETTING
Tertiary-referral neurotology and neurosurgery practice.
SUBJECTS AND METHODS
Five VPs in 4 patients (all male, ages 15-56 years) underwent SRS between 2010 and 2018. Outcome measures included tumor dimensions on serial imaging, cranial nerve function, and radiation side effects.
RESULTS
CyberKnife hypofractionated SRS was performed. The prescription dose was 24 or 27 Gy (maximum dose 33.4 Gy; range, 29.3-35.5 Gy) delivered in 3 equal fractions. The mean isodose line was 79% (range, 76%-82%). Four VPs were treated primarily, and 1 tumor underwent SRS to treat regrowth 2 years after microsurgical subtotal resection via the modified infratemporal fossa approach. The treatment volume ranged from 8.81 to 86.3 cm (mean, 35.7 cm). All demonstrated stable size (n = 3) or regression (n = 2) at last follow-up, 63 to 85 months after SRS (mean, 76 months). One patient had stable premorbid vocal fold paralysis from a prior ipsilateral glomus jugulare tumor resection. All others demonstrated normal vagal function following SRS. Treatment-related side effects, including dysgeusia (n = 1), mucositis (n = 1), and neck soft-tissue edema (n = 2), were self-limited.
CONCLUSIONS
Hypofractionated SRS appears to be both safe and effective for treating VPs, including large-volume and predominantly extracranial tumors, while preserving vagal function. SRS should be considered as a cranial nerve preservation option, especially in settings of contralateral lower cranial nerve deficits or in those with multiple paragangliomas risking both vagal nerves.
Topics: Adolescent; Adult; Cranial Nerve Neoplasms; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neoplasm Staging; Paraganglioma; Radiosurgery; Retrospective Studies; Robotics; Tomography, X-Ray Computed; Treatment Outcome; Vagus Nerve; Young Adult
PubMed: 32125943
DOI: 10.1177/0194599820910150