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Introducing the diagnostic protocol and therapy of a rare disease reviewing the worldwide literature named as vagal paraganglioma is our main target. This type is specific for women of middle age, and it is appearing as a unilateral neck mass, with or without Fontaine's sign. Giving a heavy aspect on the ear, nose and throat examination, the most important diagnostic tool is CT-angiography. Carefully looking at the size and relationship with the narrowing arteries, veins, nerves, muscles and bony structures (skull base, cervical spine), in most of the cases we choose the surgical procedure. Orv Hetil. 2019; 160(34): 1358-1362.

Paragangliomas of the head and neck are rare vascular skull-base tumors derived from the paraganglionic system with an estimated incidence of 1:30,000 accounting for 3% of all paragangliomas. The most common paraganglioma locations of the head and neck in descending order are the carotid body, jugular, tympanic, and vagal paragangliomas. This article discusses the clinical characterics, normal anatamy, imaging findings and protocols, pathology, staging, and differential diagnosis for paragangliomas of the head and neck., (Copyright © 2016 Elsevier Inc. All rights reserved.)

There is emerging evidence linking cyanotic heart disease and pheochromocytoma-paraganglioma through a proposed hypoxia pathway. We present a 59-year-old woman with known Eisenmenger syndrome secondary to delayed diagnosis of ventricular septal defect, in whom F-FDG PET/CT provided comprehensive evaluation of both an F-FDG-avid left glomus vagale paraganglioma and also increased right ventricular myocardial glucose metabolism in response to severe pulmonary hypertension in the chronic shunting state.

Malignant paraganglioma of the glomus vagale is a rare tumor entity originating from paraganglia or glomus cells. It typically affects middle age. It differs from benign paraganglioma because of its rapid growth and more aggressive clinical behavior. We report the case of a 40-year-old man presenting with a 5 cm lesion in the upper right cervical region detected by computed tomography (CT) and magnetic resonance imaging (MRI), which also showed enlargement of ipsilateral spinal and jugulodigastric lymph nodes with contrast enhancement. Clinical manifestations at diagnosis included a partial neurological deficit involving the right cranial nerves X, XI and XII. Tumor vascularization was assessed by digital angiography. The tumor mass was entirely removed by a right cervical approach with en-bloc resection with the regional lymph nodes. Histopathological examination showed a paraganglioma with cellular pleomorphism, necrotic microfoci, perineural infiltration and angiogenesis. Massive metastases in two of three jugulodigastric and one spinal lymph nodes on the right side were also detected. Postoperative workup included MRI, positron emission tomography (PET)/CT, meta-iodine-benzyl-guanidine (MIBG) scan, liver ultrasound and chest radiography. Subsequently, the patient underwent conformal radiotherapy with concomitant cisplatin administration. At the last clinical and radiological follow-up examination 5 years after completion of treatment, the patient was free of tumor recurrence. The integrated treatment by surgery and chemoradiation was feasible and effective in the management of this rare case of malignant paraganglioma of the glomus vagale. Multicenter studies should be done to increase the knowledge of tumor presentation and natural history and to analyze the possible treatment options.

This case report demonstrates the resection of an aortic root paraganglioma incidentally discovered in a 32-year-old male with replacement of the root for an oncologically complete resection.

Malignant vagal paraganglioma is very uncommon and the diagnosis of malignancy is made on the basis of presence of distant metastasis rather than the histological findings. We report angiography findings of metastatic cervical lymph nodes in a case of malignant vagal paraganglioma.

Purpose: In this study, we describe the color Doppler imaging findings in carotid body tumors and vagal body tumors., Methods: B-mode and color Doppler imaging were performed on 17 patients who had a total of 25 previously diagnosed paragangliomas (14 carotid body tumors and 11 vagal body tumors)., Results: Nineteen of 25 tumors were depicted. Five small vagal body tumors in the region of the nodose ganglion and 1 carotid body tumor could not be depicted. With B-mode imaging, paragangliomas appeared as well-defined, solid, hypoechoic masses. With color Doppler imaging, hypervascularity with a low-resistance flow pattern was demonstrated in all but 1 of the 19 tumors., Conclusions: The use of color Doppler imaging in the workup of an ambiguous neck mass is advocated.

A patient with an aorticopulmonary paraganglioma was found to have normal plasma norepinephrine and epinephrine levels and elevated dopamine levels. Iodine-123-MIBG scintigraphy did not visualize this tumor. The same finding on a negative MIBG scan in two patients with exclusively dopamine-secreting chemodectomas has been previously reported. In our patient, [111In-DTPA-D-Phe1]-octreotide scintigraphy proved to be an effective imaging technique.

Two cases of vagal body tumours are reported for their surgical consequences of immediate post-operative airway obstruction in one case, and tachycardia hypotension and severe dysphagia requiring cricopharyngeal myotomy in another. An unusual case of carotid body tumour which had the same clinical picture was also reported for comparative surgical treatment. The surgical management of both tumours was reviewed and it was found that 28-43 per cent of carotid body tumour resection would have vascular operation while dissection of vagal body tumour usually did not. The angiographic diagnostic criteria for vagal body tumour were also reviewed and found to be inadequate. Additional angiographic criterion was proposed.