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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 -
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 -
Medical Principles and Practice :... 2007The purpose of this study was to determine the clinical characteristics of carotid body tumors and to analyze the benefits of different treatment modalities.
OBJECTIVES
The purpose of this study was to determine the clinical characteristics of carotid body tumors and to analyze the benefits of different treatment modalities.
SUBJECTS AND METHODS
The clinical records of 13 patients with paragangliomas treated during the period of 1995-2005 were reviewed for age at diagnosis, sex, initial symptoms, duration of symptoms, evidence of secretory function, family history, radiographic studies, pre- and posttreatment cranial nerve deficits, multicentric tumors, angiography, operative procedures and findings, and complications. The diagnoses were established by computerized tomography and magnetic resonance imaging scanning. Angiography was performed on 3 patients with carotid body tumor.
RESULTS
All of the patients were followed up for a duration ranging from 16 months to 9 years. The median follow-up was 5 years. Of all 18 head and neck tumors confirmed in 13 patients, 16 were in the carotid body, 1 in the vagus nerve and 1 in the middle ear cleft. Four patients had multiple paragangliomas, 2 bilateral synchronous and 1 bilateral asynchronous paragangliomas. The remaining patient had 3 asynchronous paragangliomas, i.e. left jugulotympanic, right carotid body and left vagal paraganglioma. All patients underwent successful surgical resection of the tumor after appropriate preoperative investigation.
CONCLUSION
This study shows that surgical treatment is acceptably safe and effective in treating these neoplasms.
Topics: Adult; Angiography, Digital Subtraction; Female; Follow-Up Studies; Head and Neck Neoplasms; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Paraganglioma; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 17409756
DOI: 10.1159/000100392 -
Skull Base : Official Journal of North... Nov 2010We report a case of functional vagal paraganglioma to illustrate the biochemical and radiological imaging tests important in diagnosis and to highlight the importance of...
We report a case of functional vagal paraganglioma to illustrate the biochemical and radiological imaging tests important in diagnosis and to highlight the importance of a multidisciplinary team approach to manage the preoperative, perioperative, and postoperative effects of catecholamine secretion from these tumors.
PubMed: 21772811
DOI: 10.1055/s-0030-1266761 -
Cellular Oncology : the Official... Jan 2010Head and neck paraganglioma (PGL) are benign tumors that can cause important direct or surgery induced morbidity. Almost all familial and 11-29% of sporadic PGL are...
BACKGROUND
Head and neck paraganglioma (PGL) are benign tumors that can cause important direct or surgery induced morbidity. Almost all familial and 11-29% of sporadic PGL are caused by inactivating germline mutations in succinate dehydrogenase (SDH) genes. Our aim was to screen for such mutations and to evaluate clinical parameters as predictors of germline mutation.
METHODS
Seventy-four PGL patients were analyzed for germline mutations and large deletions in SDH genes, VHL and RET. Results were correlated to clinical characteristics including gender, age, tumor localization and multifocality. The surgical approach was evaluated in terms of tumor origin, sequelae and subsequent evolution.
RESULTS
Mutations in SDHB and SDHD were identified in equal proportion in 13/13 (100%) of familial and in 15/61 (25%) of sporadic cases. Familiarity, age < or =50 years and male gender were predictors of any germline mutation, while multifocality and carotid/vagal localization were indicative of SDHD mutation in particular.
CONCLUSION
In contrast to other series, this cohort of Spanish patients showed many SDHB mutations. Sporadic cases with germline mutation are frequent and underline the importance of mutational screening of all PGL patients, allowing the identification of relatives at risk and the early diagnosis of the disease, reducing or avoiding morbidity.
Topics: Adolescent; Adult; Age Factors; Aged; DNA Mutational Analysis; Early Detection of Cancer; Female; Genetic Predisposition to Disease; Germ-Line Mutation; Head and Neck Neoplasms; Humans; Male; Middle Aged; Paraganglioma; Pedigree; Predictive Value of Tests; Sex Factors; Spain; Succinate Dehydrogenase
PubMed: 20208144
DOI: 10.3233/CLO-2009-0498 -
Skull Base : Official Journal of North... Nov 2006To report long-term functional results of the surgical treatment of cervical paragangliomas.
OBJECTIVE
To report long-term functional results of the surgical treatment of cervical paragangliomas.
PATIENTS AND METHODS
A retrospective review of 22 patients with 34 head and neck paragangliomas of which 27 were resected between 1981 and 2004. Of these, 16 were carotid body tumors and 11 were vagal paragangliomas. There were 13 women and 9 men with an average age of 48.6 years (range, 26 to 75 years; median, 49 years) and the mean follow-up period was 82 months (range, 3 to 184 months; median, 61 months).
RESULTS
There were 13 solitary tumors of which 5 were carotid body tumors and 8 vagal paragangliomas. Multiple head and neck paragangliomas were seen in 9 patients (41%). The incidence of associated multiple tumors was 64.3% for carotid body tumors and 38.5% for vagal paragangliomas. Complete tumor resection was achieved in all but 1 patient in whom a small intradural residual vagal paraganglioma had to be left. The internal carotid artery was preserved in all carotid body tumor resections. Lower cranial nerve deficits were sustained in 1 carotid body tumor resection only, but in all cases with multiple tumors. All patients with vagal paragangliomas had or developed a vagal nerve paralysis. In 4 cases minor complications developed postoperatively. No recurrent tumors were seen during the follow-up period.
CONCLUSIONS
Even in large head and neck paragangliomas surgical treatment provides excellent tumor control with low postoperative morbidity. A wait-and-scan policy may be more appropriate for those patients with multiple tumors, advanced age, or high operative risk and for those whose tumors have recurred following radiotherapy.
PubMed: 17471317
DOI: 10.1055/s-2006-950386 -
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 -
Proceedings (Baylor University. Medical... Apr 2012
PubMed: 22481846
DOI: 10.1080/08998280.2012.11928813 -
Cancer Dec 2014Paragangliomas of the head and neck are rare, slow-growing, generally benign tumors of neuroendocrine cells associated with the peripheral nervous system that commonly...
BACKGROUND
Paragangliomas of the head and neck are rare, slow-growing, generally benign tumors of neuroendocrine cells associated with the peripheral nervous system that commonly involve the carotid body, jugular bulb, vagal ganglia, and temporal bone. Treatment options include surgery, radiotherapy (RT), stereotactic radiosurgery (SRS), and observation. This article briefly reviews our 45-year institutional experience treating this neoplasm with RT.
METHODS
From January 1968 through March 2011, 131 patients with 156 benign paragangliomas of the temporal bone, carotid body, jugular bulb, or glomus vagale were treated with RT at a median dose of 45 Gy in 25 fractions. The mean and median follow-up times were 11.5 years and 8.7 years, respectively.
RESULTS
Five tumors (3.2%) recurred locally after RT, all within 10 years of treatment. The overall local control rates at 5 and 10 years were 99% and 96%, respectively. The cause-specific survival rates at 5 and 10 years were 98% and 97%, respectively. The distant-metastasis free survival rates at 5 and 10 years were 99% and 99%, respectively. The overall survival rates at 5 and 10 years were 91% and 72%, respectively. There were no severe complications.
CONCLUSION
RT for benign head and neck paragangliomas is a safe and efficacious treatment associated with minimal morbidity. Surgery is reserved for patients in good health whose risk of associated morbidity is low. SRS may be suitable for patients with skull base tumors <3 cm where RT is logistically unsuitable. Observation is a reasonable option for asymptomatic patients with a limited life expectancy.
Topics: Adult; Aged; Aged, 80 and over; Aortic Bodies; Carotid Body Tumor; Cohort Studies; Disease-Free Survival; Female; Glomus Jugulare Tumor; Head and Neck Neoplasms; Humans; Male; Middle Aged; Paraganglioma, Extra-Adrenal; Radiotherapy, Intensity-Modulated; Retrospective Studies; Temporal Bone; Treatment Outcome; Young Adult
PubMed: 25060724
DOI: 10.1002/cncr.28923