-
The Journal of International Advanced... Nov 2022This study aimed to review tumor control and cranial nerve function outcomes in patients with complex jugular paragangliomas and to refine the surgical strategies for...
BACKGROUND
This study aimed to review tumor control and cranial nerve function outcomes in patients with complex jugular paragangliomas and to refine the surgical strategies for complex jugular paragangliomas.
METHODS
We describe our experience with 12 patients with complex jugular paragangliomas diagnosed in our institution from January 2013 to June 2020. The main outcomes included tumor control, complications, and function of facial nerve and lower cranial nerves, postoperatively.
RESULTS
Gross-total resection was achieved for 9 (75%) patients, and subtotal resection was achieved for 3 (25%) patients. The surgical tumor control rate was 100% after a mean follow-up of 45.5 months (range, 13-111 months). Postoperatively, 10 patients (83.3%) obtained unchanged or improved facial nerve function. However, new lower cranial nerve deficits occurred in 2 patients (16.7%) due to surgical removal of the concurrent vagal paraganglioma and scar tissue enclosing the IX and XII nerves.
CONCLUSION
Our refined surgical techniques, including tension-free anterior facial nerve rerouting, sigmoid sinus tunnel-packing, and pushpacking techniques, could be a choice for the treatment of complex jugular paragangliomas to achieve tumor control and cranial nerves preservation. A 2-stage surgery should be applied to minimize the risk of bilateral cranial neuropathies and the influence on cerebral circulation in patients with bilateral paragangliomas. The preoperative endovascular intervention such as coil embolization or internal carotid artery stenting can be employed for the management of paragangliomas with internal carotid artery-associated lesions.
Topics: Humans; Carotid Stenosis; Treatment Outcome; Retrospective Studies; Stents; Glomus Jugulare Tumor; Paraganglioma
PubMed: 36349670
DOI: 10.5152/iao.2022.22675 -
Annals of Vascular Surgery Nov 2017Neuroendocrine tumors of the neck are rare swellings, which constitute about 0.03% of all tumors. Most are sporadically arising paragangliomas. Over 50% arise from the... (Review)
Review
BACKGROUND
Neuroendocrine tumors of the neck are rare swellings, which constitute about 0.03% of all tumors. Most are sporadically arising paragangliomas. Over 50% arise from the carotid bodies and pose difficulties in diagnosis and surgical management, which may result in disabling complications.
OBJECTIVE
To determine the diagnostic, surgical, and postoperative challenges, and how to overcome them, in the management of neuroendocrine tumors of the neck.
STUDY DESIGN
Descriptive case series.
METHODS
A retrospective analysis of 19 patients who presented to our surgery clinics between 2010 and 2015 with neck swelling and suspicion of neurogenic tumors were included in the study. All patients received preoperative evaluation with Doppler studies and neck computed tomography scan, with the finding of suspicion of neurogenic tumor. Preoperative tissue diagnosis was not attempted as this usually required excisional biopsy. All tumors were surgically excised. Data regarding mean tumor size, neurovascular invasion, postoperative complications, and histopathological findings were recorded and statistically analyzed.
RESULTS
Nineteen patients (7 male) with median age of 45 years were included. Mean size of the tumor was 7.5 cm (range: 4.0-11.8 cm). Six of the tumors were right sided and 13 left sided. Clinical findings often were nonspecific and mimicked other conditions. Operative challenges were encountered in 11 (57.9%) patients, who had tumors of more than 5 cm. Vascular invasion was found in 2 (10.5%) patients, but bleeding requiring preoperative transfusion was not required in any patient. Neurological complications occurred in 11 (57.8%) patients; 3 had transit hypoglossal nerve palsy, with full recovery; 2 had complete nerve palsy, with reasonable rehabilitation without further surgical intervention; and 5 developed neurological deficits (hoarseness, dysphagia, or Horner's syndrome) but recovered and compensated well without further operation. Two patients who had vascular invasion required vascular reconstruction. Histopathology in 14 (73.7%) cases was carotid body tumor and 5 were Schwannomas (1 vagal and 4 sympathetic chain). All 4 patients with Schwannomas of cervical sympathetic chain developed Horner's syndrome; 2 of them have compensated well with reasonable recovery; 2 have had tarsorrhaphy for cosmetic reasons. The patient with vagal Schwannoma developed hoarseness and swallowing difficulties, which resolved without intervention. No malignancy was encountered in this series, and there was no mortality.
CONCLUSIONS
Neuroendocrine tumors of the neck present diagnostic challenges because of nonspecific clinical findings mimicking other conditions. Preoperative tissue diagnosis is mostly unsuccessful, and exact diagnosis requires excisional biopsy. Surgical excision, which is the main treatment, often has associated technical difficulties and unavoidable complications requiring further intervention.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Carcinoma, Neuroendocrine; Diagnosis, Differential; Female; Head and Neck Neoplasms; Humans; Male; Middle Aged; Neoplasm Invasiveness; Postoperative Complications; Predictive Value of Tests; Retrospective Studies; Risk Factors; Tomography, X-Ray Computed; Treatment Outcome; Tumor Burden; Ultrasonography, Doppler; Young Adult
PubMed: 28688875
DOI: 10.1016/j.avsg.2017.06.128 -
Acta Otorrinolaringologica Espanola 2015Head and neck paragangliomas are rare tumours of a neuroendocrine nature. The aim of this study was to show our experience in the management of these kind of tumours.
INTRODUCTION
Head and neck paragangliomas are rare tumours of a neuroendocrine nature. The aim of this study was to show our experience in the management of these kind of tumours.
METHODS
This was a retrospective study of head and neck paragangliomas diagnosed between 1978 and 2014. A total of 126 patients with 162 tumours were analysed. The paragangliomas included 88 jugulotympanic tumours, 53 carotid tumours and 21 vagal paragangliomas.
RESULTS
Mean age at diagnosis was 53.1 years; 87 patients were female (69.1%) and 39, male (30.9%). Multifocality was present in 24 patients (19.1%). Germline mutations were found in 20 patients analysed; SDHD and SDHB were the most frequent. Surgery was performed on 72 isolated paragangliomas: these were 9 carotid, 21 tympanic, 37 jugular and 5 vagal paragangliomas. There were 25 isolated tumours that were observed periodically: 7 carotid, 3 tympanic, 9 jugular and 6 vagal paragangliomas; 5 jugular tumours were irradiated. Multifocal paragangliomas were individually treated, with a total of 26 surgical procedures and 36 tumours resected, 9 irradiated and 12 tumours periodically observed. Postoperative cranial nerve deficits in isolated carotid paragangliomas were lower (15%) compared with jugular tumours (45.5%, P=.04). Nerve deficit was found more frequently in tumours with intradural extension (100%) than in extradural tumours (37.5%, P=.007).
CONCLUSIONS
Management of head and neck paragangliomas include surgery, radiotherapy and wait and scan policies. A combination of all of them is usually needed in patients with multifocal paragangliomas.
Topics: Adult; Cranial Nerve Injuries; Dura Mater; Female; Genetic Predisposition to Disease; Germ-Line Mutation; Head and Neck Neoplasms; Humans; Male; Middle Aged; Neoplasm Invasiveness; Neoplastic Syndromes, Hereditary; Paraganglioma; Postoperative Complications; Retrospective Studies; Watchful Waiting
PubMed: 25638014
DOI: 10.1016/j.otorri.2014.11.002 -
Annals of Medicine and Surgery (2012) Oct 2021and importance: Head and neck paragangliomas are slowly growing benign tumors and they originate from specialized neural crest cells. We report an unusual combined...
INTRODUCTION
and importance: Head and neck paragangliomas are slowly growing benign tumors and they originate from specialized neural crest cells. We report an unusual combined glomus vagal and jugular tumor that was rarely described in the literature to the best of our knowledge.
CASE PRESENTATION
A 51 years old female with no pathological history was presented to our ENT department with 6 months' history of a right latero cervical swelling gradually increasing in size associated with a swallowing difficulties and hoarseness. Preoperatively clinical examination had found vagal and hypoglossal nerve paralysis. Cervical CT scan and MRI had shown glomus jugular tumor. The patient underwent a surgical excision with severe swallowing difficulties and facial palsy in the immediate postoperative period with a mild recovery afterwards.
CLINICAL DISCUSSION
Paragangliomas of the mesotympanum and jugular foramen most commonly present as a vascular middle ear mass. The most common presenting symptom is pulsatile tinnitus occurring in 80% followed by hearing loss (60%). Dysfunction of cranial nerves traversing the jugular foramen may be commonly encountered with resultant abnormalities of speech, swallowing and airway function. Vagal paragangliomas are the least common of the three primary craniocervical paragangliomas. The most common presenting sign is the presence of a painless neck mass accompanied occasionally by dysphagia and hoarseness. The association of both glomus vagal and jugular tumor is rarely described in the literature to the best of our knowledge.
CONCLUSION
Head and neck paragangliomas are slowly growing benign tumors and they originate from specialized neural crest cells. Vagal paragangliomas are the least common of the three primary craniocervical paragangliomas. The association of both glomus vagal and jugular tumor is rarely described in the literature to the best of our knowledge. The choice of treatment depends on the location, size, and also biologic activity of the tumor as well as the physical condition of the patient.
PubMed: 34691440
DOI: 10.1016/j.amsu.2021.102918 -
Journal of Neurointerventional Surgery Jun 2016To report the morbidity and long term results in the treatment of paragangliomas by transarterial embolization with ethylene vinyl alcohol (Onyx), either as preoperative...
OBJECT
To report the morbidity and long term results in the treatment of paragangliomas by transarterial embolization with ethylene vinyl alcohol (Onyx), either as preoperative or palliative treatment.
METHODS
Between September 2005 and 2012, 18 jugulotympanic, 7 vagal, and 4 carotid body paragangliomas (CBPs) underwent Onyx embolization, accordingly to our head and neck multidisciplinary team's decision. CBPs were embolized preoperatively. Jugulotympanic and vagal paragangliomas underwent surgery when feasible, otherwise palliative embolization was carried out alone, or in combination with radiotherapy or tympanic surgery in the case of skull base or tympanic extension. Treatment results, and clinical and MRI follow-up data were recorded.
RESULTS
In all cases, devascularization of at least 60% of the initial tumor blush was obtained; 6 patients underwent two embolizations. Post-embolization, 8 patients presented with cranial nerve palsy, with partial or complete regression at follow-up (mean 31 months, range 3-86 months), except for 2 vagal and 1 hypoglossal palsy. 10 patients were embolized preoperatively; 70% were cured after surgery and 30% showed residual tumor. 19 patients received palliative embolization, of whom 5 underwent radiotherapy and 3 received tympanic surgery post-embolization. Long term follow-up of palliative embolization resulted in tumor volume stability (75%) or extension in intracranial or tympanic compartments. Onyx embolization of CBPs resulted in more difficult surgical dissection in 2 of 4 cases.
CONCLUSIONS
Onyx embolization is a valuable alternative to surgery in the treatment of jugulotympanic and vagal paragangliomas; tympanic surgery or radiosurgery of the skull base should be considered in selected cases. Preoperative Onyx embolization of CBPs is not recommended.
Topics: Adult; Aged; Biocompatible Materials; Dimethyl Sulfoxide; Embolization, Therapeutic; Female; Head and Neck Neoplasms; Humans; Male; Middle Aged; Paraganglioma, Extra-Adrenal; Polyvinyls; Preoperative Care; Retrospective Studies; Treatment Outcome
PubMed: 25935924
DOI: 10.1136/neurintsurg-2014-011582 -
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 -
European Archives of... Sep 2015Vagal paragangliomas are very rare benign vascular tumors of neuroendocrine nature, and are much less frequent than carotid and jugulo-tympanic tumors. The goal of this...
Vagal paragangliomas are very rare benign vascular tumors of neuroendocrine nature, and are much less frequent than carotid and jugulo-tympanic tumors. The goal of this retrospective study is to review the clinical and genetic findings, surgical treatment, and complications of vagal paragangliomas, as well as to discuss the management options. During the period 1990-2013, 17 patients with vagal paragangliomas were referred to our institution. There were ten patients with isolated tumors, and seven with multicentric paragangliomas. There were nine women and eight men. Mean age of patients was 51.4 years. Five cases had a positive family history of paraganglioma (29.4 %). Germline mutations of SDH genes were found in six of our patients (35.3 %). Many options were considered in the management of vagal paragangliomas. Surgical treatment was performed in 11 young patients (64.7 %) using different approaches: in 4 patients the tumor was resected through a transcervical approach; in 3 through a transcervical-transmandibular approach; in 1 it was resected using a transcervical-transmastoid approach, and in 3 a type A infratemporal fossa approach was performed. In all operated cases, the removal of the tumor led to sacrificing of the vagus nerve. Postoperative hypoglossal nerve deficit was reported in 4 cases (36.3 %). In six elderly patients (35.3 %), we decided to "wait-and-scan" in order to avoid creating greater morbidity than that of the tumor itself. Many factors should be considered in the treatment of vagal paragangliomas: the age and general condition of the patient, the biological behavior of the tumor, tumor size, genetic results, bilaterality, multicentricity, lower cranial nerve function, and of course the potential morbidity of the surgical treatment itself. Rehabilitation and, possibly surgery, are necessary to treat postoperative lower cranial nerve deficits.
Topics: Aged; Cranial Nerve Neoplasms; Female; Humans; Male; Middle Aged; Paraganglioma, Extra-Adrenal; Retrospective Studies; Vagus Nerve Diseases
PubMed: 24973966
DOI: 10.1007/s00405-014-3141-0 -
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 -
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 -
Clinical Radiology Sep 2019To evaluate whether standard magnetic resonance imaging (MRI) could distinguish vagal paragangliomas (VPs) from carotid body tumours (CBTs) in the parapharyngeal space.
AIM
To evaluate whether standard magnetic resonance imaging (MRI) could distinguish vagal paragangliomas (VPs) from carotid body tumours (CBTs) in the parapharyngeal space.
METHODS AND MATERIALS
Thirteen VPs in 13 patients and 29 CBTs in 26 patients were included in this study. MRI features were evaluated independently by two head and neck radiologists with 10 and 16 years of experience (X.Wang and Y.Chen).
RESULTS
Significant differences were found in shape, direction of internal carotid artery (ICA) displacement, splaying of the carotid bifurcation, involvement of the jugular foramen, and maximum vertical diameters between VPs and CBTs (p=0.016, <0.001, <0.001, <0.001 and <0.001, respectively). Splaying of the carotid bifurcation was found in all the 29 CBTs for both observers, whereas only four VPs for observer 1 and two for observer 2 showed this feature. The ICA was displaced posteriorly in all the 29 patients with CBTs for both observers, and only three patients with VPs for observer 1 and two for observer 2. Involvement of the jugular foramen was found in seven patients with VPs for both observers, while none of patients with CBT showed this feature. With the combination of splaying of the carotid bifurcation and involvement of the jugular foramen, the multiple logistic regression model revealed the diagnostic accuracy was 95.2% for observer 1 and 97.6% for observer 2. With the combination of ICA displacement direction and involvement of the jugular foramen, the diagnostic accuracy was 97.6% for both observers.
CONCLUSION
MRI features can effectively differentiate VPs from CBTs in the parapharyngeal space.
Topics: Adult; Carotid Body Tumor; Contrast Media; Cranial Nerve Neoplasms; Diagnosis, Differential; Female; Gadolinium DTPA; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Paraganglioma; Parapharyngeal Space; Vagus Nerve
PubMed: 31122716
DOI: 10.1016/j.crad.2019.04.018