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Polish Journal of Radiology 2016Hereditary head and neck paragangliomas (HNP) are very often associated with pheochromocytoma-paraganglioma syndromes, which are caused by mutations in genes encoding...
BACKGROUND
Hereditary head and neck paragangliomas (HNP) are very often associated with pheochromocytoma-paraganglioma syndromes, which are caused by mutations in genes encoding subunits of succinate dehydrogenase () complex. The aim of this study was to determine the frequency and location of HNP among SDHx carriers.
MATERIAL/METHODS
A total of 72 patients with mutations underwent computed tomography examinations of the head and neck. HNP were present in 44 (61.1%) out of 72 patients (31 , 11 , 2 ); 113 HNP were found; the most common were carotid paragangliomas (59) and vagal paragangliomas (27).
RESULTS
The HNP were statistically more frequent in carriers of mutations compared to carriers of mutations (72.1% 43.5%, p=0.033). Multiple tumors more often occurred in patients with mutations 26/31 (83.9%) than in patients with mutations 6/11 (54.5%) p=0.05. There was a significant difference in the prevalence of carotid paragangliomas between patients with and mutations (7/11 [63.6%] 30/31 [96.8%], respectively, p=0.004). Patients with mutations more often had carotid paragangliomas located on the left side than on the right side, as compared to mutations 25/31 (80.6%) 4/11 (36.4%), p=0.006.
CONCLUSIONS
mutations predispose to multifocal and bilateral HNP. Carotid and vagal paragangliomas occurred most often. Patients with mutations are characterized by higher frequency of HNP than patients with mutations, which is mainly driven by higher frequency of carotid body tumors in patients with mutations. No difference in the frequency of head and neck paragangliomas in other locations was found.
PubMed: 27867439
DOI: 10.12659/PJR.897490 -
Cancer Control : Journal of the Moffitt... Jul 2016Commonly occurring in the head and neck, paragangliomas are typically benign, highly vascular neoplasms embryologically originating from the extra-adrenal paraganglia of... (Review)
Review
BACKGROUND
Commonly occurring in the head and neck, paragangliomas are typically benign, highly vascular neoplasms embryologically originating from the extra-adrenal paraganglia of the neural crest. Frequently, these tumors are associated with the vagus or tympanic plexus nerve or the carotid artery, or jugular bulb. Their clinical presentation can vary across a wide spectrum of signs and symptoms.
METHODS
We reviewed and compared standard treatment approaches for paragangliomas of the head and neck.
RESULTS
In general, surgery is the first-line choice of therapy for carotid body tumors, whereas radiotherapy is the first-line option for jugular and vagal paragangliomas.
CONCLUSIONS
Because of the complexity of clinical scenarios and treatment options for paragangliomas, a multidisciplinary algorithmic approach should be used for treating paragangliomas. The approach should emphasize single-modality treatment that yields excellent rates of tumor control, low rates of severe, iatrogenic morbidity, and the preservation of long-term function in this patient population.
Topics: Female; Head and Neck Neoplasms; Humans; Male; Paraganglioma
PubMed: 27556663
DOI: 10.1177/107327481602300306 -
Endocrine-related Cancer Sep 2016The vagus nerve (cranial nerve X) is the main nerve of the parasympathetic division of the autonomic nervous system. Vagal paragangliomas (VPGLs) are a prime example of... (Review)
Review
The vagus nerve (cranial nerve X) is the main nerve of the parasympathetic division of the autonomic nervous system. Vagal paragangliomas (VPGLs) are a prime example of an endocrine tumor associated with the vagus nerve. This rare, neural crest tumor constitutes the second most common site of hereditary head and neck paragangliomas (HNPGLs), most often in relation to mutations in the succinate dehydrogenase complex subunit D (SDHD) gene. The treatment paradigm for VPGL has progressively shifted from surgery to abstention or therapeutic radiation with curative-like outcomes. Parathyroid tissue and parathyroid adenoma can also be found in close association with the vagus nerve in intra or paravagal situations. Vagal parathyroid adenoma can be identified with preoperative imaging or suspected intraoperatively by experienced surgeons. Vagal parathyroid adenomas located in the neck or superior mediastinum can be removed via initial cervicotomy, while those located in the aortopulmonary window require a thoracic approach. This review particularly emphasizes the embryology, molecular genetics, and modern imaging of these tumors.
Topics: Cranial Nerve Neoplasms; Humans; Paraganglioma; Parathyroid Neoplasms; Vagus Nerve; Vagus Nerve Diseases
PubMed: 27406876
DOI: 10.1530/ERC-16-0241 -
Journal of Clinical and Diagnostic... Jan 2016We report a large vagal neurilemmoma in the poststyloid compartment of the parapharyngeal space. A 52-year-old man was referred to our hospital with a feeling of...
We report a large vagal neurilemmoma in the poststyloid compartment of the parapharyngeal space. A 52-year-old man was referred to our hospital with a feeling of discomfort in the left upper neck. Computed tomography showed a 30mm x 30mm x 40mm mass with inhomogeneous internal enhancement in the left carotid space. Magnetic resonance imaging revealed a 30mm × 30mm × 40mm heterogeneous mass in the area of the bifurcation of the common carotid artery. We gave a provisional diagnosis of neurilemmoma or vagal paraganglioma in the parapharyngeal space preoperatively based on the results of physical examination and imaging. We selected a transcervical-transmandibular approach. Under general anaesthesia, a tumour originating from the vagus nerve was completely extirpated while protecting the internal and external carotid arteries. Although mild postvagotomy dysphagia and hoarseness were seem for 6 months postoperatively, symptoms resolved and the patient showed a satisfactory course without recurrence after 10 years. Histological examination of the excised specimen showed antoni A and antoni B pattern. Positive immunoreactivity for S-100 protein was identified, but negative results were obtained for neuron-specific enolase, chromogranin and neurofilament. The tumour was diagnosed as neurilemmoma of the vagus nerve.
PubMed: 26894190
DOI: 10.7860/JCDR/2016/13789.7141 -
BMJ Case Reports Jan 2016
Topics: Acetabulum; Bone Neoplasms; Female; Humans; Mutation; Neck; Neoplasm Staging; Organometallic Compounds; Paraganglioma; Positron-Emission Tomography; Radiopharmaceuticals; Succinate Dehydrogenase; Tomography, X-Ray Computed
PubMed: 26729832
DOI: 10.1136/bcr-2015-213438 -
Journal of Maxillofacial and Oral... Dec 2015A rare case of vagal paraganglioma is reported.
INTRODUCTION
A rare case of vagal paraganglioma is reported.
MATERIAL AND METHODS
The specific radiological features of this tumor are presented to the clinician in order to make presumptive diagnosis.
CONCLUSION
By CT-scan and/or MRI it must be suspected when a hypervascular tumor in relation to the major cervical vessels displaces the internal and external carotid arteries forward, does not open the carotid bifurcation, and displaces the internal jugular vein backwards.
PubMed: 26604479
DOI: 10.1007/s12663-015-0750-0 -
Head and Neck Pathology Jun 2016Paragangliomas are rare, typically benign neuroendocrine tumors that represent a small portion of head and neck tumors. A small percentage of these are known to have...
Paragangliomas are rare, typically benign neuroendocrine tumors that represent a small portion of head and neck tumors. A small percentage of these are known to have malignant potential. They arise from the carotid body, jugular bulb or vagus nerves. There is limited literature discussing the management of malignant vagal paragangliomas. We present a case of a 25 year old female with a left malignant vagal paraganglioma. The following case presentation will describe the presentation, classic radiologic findings, and management of a malignant vagal paraganglioma along with a review of the literature.
Topics: Adult; Female; Humans; Lymphatic Metastasis; Paraganglioma, Extra-Adrenal; Vagus Nerve Diseases
PubMed: 25712400
DOI: 10.1007/s12105-015-0621-5 -
Acta Otorhinolaryngologica Italica :... Oct 2014The aim of this study was to describe our experience with benign parapharyngeal space tumours resected via a transcervical route without mandibulotomy and to investigate...
The aim of this study was to describe our experience with benign parapharyngeal space tumours resected via a transcervical route without mandibulotomy and to investigate associated postoperative sequelae and complications. The study investigated and analysed the retrospective charts of 44 patients who underwent surgery for benign parapharyngeal space tumours over a 10-year period. The diagnosis was reached in all patients with clinical and radiologic findings; preoperative fine-needle aspiration biopsy was not performed in any case. The preferred means of accessing the parapharyngeal space in all patients was a transcervical route. In 5 of these patients, transparotid extension was performed due to the position of the tumour. Tumours were classified radiologically as poststyloid in 27 cases and prestyloid in 17 cases. The final histopathologic diagnosis was vagal paraganglioma in 16 cases, pleomorphic adenoma in 13 cases, schwannoma in 10 cases and comparatively rarer tumours in the remaining 5 cases. In three patients, cranial nerve paralysis was observed during preoperative evaluation. Permanent cranial nerve paralysis occurred in 19 cases (43.2%) in the postoperative period, the majority of which were neurogenic tumours such as vagal paraganglioma (n = 16) and schwannoma (n = 2), and one case of non-neurogenic parapharyngeal tumour. The median duration of follow-up was 61 ± 33 months. There was no local recurrence in any patient during the follow-up period. A transcervical approach should be the first choice for excision of parapharyngeal space tumours, except for recurrent or malignant tumours, considering its advantages of providing direct access to the neoplasm, adequate control of neurovascular structures from the neck and optimal aesthetic outcomes due to preservation of mandibular continuity with minimal morbidity and hospitalisation time.
Topics: Adenoma, Pleomorphic; Adult; Aged; Female; Follow-Up Studies; Head and Neck Neoplasms; Humans; Male; Middle Aged; Neurilemmoma; Paraganglioma; Pharynx; Retrospective Studies; Surgical Procedures, Operative
PubMed: 25709146
DOI: No ID Found -
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 -
Journal of Surgical Technique and Case... 2015Schwannomas are benign, rare peripheral nerve sheath tumors that occur in the head and neck region. Some physicians opt to closely observe cases of schwannoma of the...
Schwannomas are benign, rare peripheral nerve sheath tumors that occur in the head and neck region. Some physicians opt to closely observe cases of schwannoma of the neck on an outpatient basis rather than to perform radical surgery. However, there is a possibility, albeit rare, of malignant transformation of a benign schwannoma. Here, we are reporting the first case from the Indian subcontinent which was transformed into the angiosarcoma from benign vagal schwannoma over a long period. A 47-year-old male patient complaining of left sided neck swelling since last 12 years, swelling was insidious in onset, gradually progressive very slowly. In last 2 months, the size of the swelling was suddenly increased. On examination, there was an approximately 6 cm × 6 cm of size, firm, nodular, well-defined, nontender swelling in the left lateral part of the neck. Fine-needle aspiration cytology (FNAC) revealed paraganglioma and magnetic resonance imaging demonstrated very clearly a tumor, its morphology, and its relation to the surrounding structures, the tumor was thought to be a vagal schwannoma. Surgery was done, and the whole of the tumor was removed in toto. On final histopathological diagnosis, the tumor was proved to be angiosarcoma developed from vagal schwannoma. Postoperative chemotherapy was given but due to distant metastasis, the patient died. Long standing neck masses can convert into malignancy as in our case, therefore, work up of the patient should be done properly. Multiple FNAC should be done because single FNAC can give the false negative result as in our case. This was our diagnostic drawback not to do multiple computed tomography guided FNAC.
PubMed: 27512546
DOI: 10.4103/2006-8808.184941