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International Journal of Surgery Case... Jun 2023Vagal paragangliomas of neck are rare tumours of neural crest origin usually arising in elderly age with female predominance. They have a vague clinical presentation...
INTRODUCTION AND IMPORTANCE
Vagal paragangliomas of neck are rare tumours of neural crest origin usually arising in elderly age with female predominance. They have a vague clinical presentation therefore difficult to diagnose preoperatively. We hope that this case report and literature review would add to the existing literature and help devise a comprehensive diagnostic and therapeutic plan for vagal paragangliomas.
CASE PRESENTATION
We report a case of vagal paraganglioma occurring in a 13-year-old male which is an extremely rare presentation in this age group. The patient presented with a large solitary painless progressively growing mass in posterior triangle of neck. External jugular vein was stretched and trachea was deviated medially. The mass was arising via a twig from vagus nerve and was surgically excised. Diagnosis was established post-operatively through histopathological analysis.
CLINICAL DISCUSSION
Vagal paraganglioma is a rare occurrence in male teenagers and may mimic schwannoma, neuroma, jugular meningioma, or other gangliomas. Surgical excision is mainstay of treatment but resultant vagal complications and neurological consequences are usually unavoidable. Nonetheless, the prognosis may be easily improved with sound surgical judgement, skill, and routine follow-up.
CONCLUSIONS
Vagal paraganglioma usually presents as a swelling in neck and cannot be diagnosed on simple clinical examination. CT scan and MRI are imaging modalities of choice and can be coupled with angiography to increase diagnostic accuracy. Although both radiation therapy and surgical excision have both been found to be successful treatment options, it is still unclear which is more beneficial.
PubMed: 37263007
DOI: 10.1016/j.ijscr.2023.108362 -
Current Opinion in Otolaryngology &... Apr 2023A vagal paraganglioma is a rare head and neck tumor arising from the paraganglionic tissue within the perineurium of the vagus nerve, anywhere along the course of the... (Review)
Review
PURPOSE OF REVIEW
A vagal paraganglioma is a rare head and neck tumor arising from the paraganglionic tissue within the perineurium of the vagus nerve, anywhere along the course of the nerve. Due to its proximity to the internal carotid artery, the internal jugular vein and the lower cranial nerves, this disorder poses significant diagnostic and therapeutic challenges. The diagnostic workup and management keep on evolving.
RECENT FINDINGS
This article gives a concise update of the clinical spectrum and the current state-of-the-art diagnostic workup and management of vagal paraganglioma.
SUMMARY
Every patient with suspected vagal paraganglioma needs to be evaluated by a multidisciplinary team. The management strategy is selected depending on the growth rate of the tumor, the age and fitness of the patient, the number of affected cranial nerves, the metabolic activity of the paraganglioma, and the eventual multicentricity. An algorithm guiding the clinician through the different treatment options is presented.
Topics: Humans; Paraganglioma, Extra-Adrenal; Paraganglioma; Head and Neck Neoplasms
PubMed: 36912227
DOI: 10.1097/MOO.0000000000000876 -
Current Opinion in Otolaryngology &... Apr 2023This review summarizes practical recommendations for screening, work-up, and management of hereditary head and neck paragangliomas based on the growing molecular and... (Review)
Review
PURPOSE OF REVIEW
This review summarizes practical recommendations for screening, work-up, and management of hereditary head and neck paragangliomas based on the growing molecular and empirical understanding of this disease.
RECENT FINDINGS
The proportion of hereditary cases among head and neck paragangliomas is significant (∼33 to 50%), and specific genetic alterations may increase the risk of malignancy. Genotyping should be performed for each case, and patients carrying a pathological mutation should be regularly screened for new tumors. Computed tomography (CT), magnetic resonance imaging (MRI), digital subtraction angiography (DSA), and functional positron emission tomography (PET) can provide a reliable preoperative diagnosis in the absence of histology. Comparative data on therapeutic outcome and morbidity now render radiation, stereotactic radiosurgery, and active surveillance preferable over surgery in highly advanced cases of jugulotympanic and vagal paragangliomas, whereas surgery remains the first choice for most carotid body paragangliomas.
SUMMARY
Complete paraganglioma removal continues to be the primary therapeutic goal; however, this is sometimes impossible to accomplish with acceptable morbidity. In these cases, therapy selection should focus on preserving cranial nerve function and minimizing both tumor-associated and therapy-associated complications, particularly in genetically predisposed patients. An interdisciplinary approach to the management of hereditary head and neck paragangliomas is strongly recommended.
Topics: Humans; Head and Neck Neoplasms; Paraganglioma, Extra-Adrenal; Paraganglioma; Magnetic Resonance Imaging; Tomography, X-Ray Computed
PubMed: 36912223
DOI: 10.1097/MOO.0000000000000867 -
Indian Journal of Otolaryngology and... Dec 2022Paragangliomas of the head and neck can arise in many locations along the carotid sheath and middle ear. A hypervascular tumor in relation to the major cervical vessels...
Paragangliomas of the head and neck can arise in many locations along the carotid sheath and middle ear. A hypervascular tumor in relation to the major cervical vessels can be either a carotid body tumor or vagal paraganglioma. Carotid bifurcation separation is a characteristic feature of carotid body tumours. We present a case of vagal paraganglioma of the neck, causing carotid bifurcation separation similar to that of a carotid body tumor. In this case report, we highlight the imaging features that can differentiate these two paragangliomas in such a confusing situation.
PubMed: 36742569
DOI: 10.1007/s12070-021-03005-w -
American Journal of Otolaryngology 2023
Topics: Humans; Biopsy, Fine-Needle; Thyroid Neoplasms; Paraganglioma; Cranial Nerve Neoplasms; Vagus Nerve Diseases; Thyroid Nodule
PubMed: 36628912
DOI: 10.1016/j.amjoto.2022.103744 -
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 -
Annales D'endocrinologie Aug 2023Thoracic and cervical paragangliomas (PGLs) are rare neuroendocrine tumors arising from chromaffin cells of the neural crest progenitors located outside the adrenal... (Review)
Review
Thoracic and cervical paragangliomas (PGLs) are rare neuroendocrine tumors arising from chromaffin cells of the neural crest progenitors located outside the adrenal gland. We describe our current protocol as a multidisciplinary team for the management of cervical and thoracic PGLs. Surgery is generally considered the treatment of choice as it offers the best chance for cure. For resection of thoracic PGLs, video-assisted thoracoscopic surgery (VATS) is the main surgical approach, while open thoracotomy is preferred in case of tumors > 6cm, lacking confirmation of a plane of separation with adjacent structures, or with technical difficulties during VATS. In cervical PGLs, the surgical approach should be individualized according to location, mainly based on the Glasscock-Jackson and the Fisch-Mattox classifications. Surgery is the treatment of choice for most cervical and thoracic PGLs, but radiotherapy or observation could be more suitable options in unresectable cervical and thoracic PGLs or when resection has been incomplete.
Topics: Humans; Paraganglioma; Neuroendocrine Tumors; Adrenal Glands
PubMed: 36334803
DOI: 10.1016/j.ando.2022.10.013 -
Cureus Aug 2022Head and neck paragangliomas (PGLs) most commonly derive from the carotid body, jugulotympanic, vagal, and laryngeal paraganglia. Thyroid PGLs originate in the inferior...
Thyroid Paraganglioma With Medullary Carcinoma: A Unique Combination in a Patient in Association With Multiple Endocrine Neoplasia Type 2B Syndrome With Prolonged Survival.
Head and neck paragangliomas (PGLs) most commonly derive from the carotid body, jugulotympanic, vagal, and laryngeal paraganglia. Thyroid PGLs originate in the inferior laryngeal paraganglion, which may lie inside the thyroid parenchyma. Intrathyroid PGLs are rare with approximately 75 cases reported to date, mostly as solitary lesions. The coexistence of thyroid PGL with medullary thyroid carcinoma (MTC) has not been reported. Here, we report a unique case of intrathyroid PGL concomitant with MTC in the context of multiple endocrine neoplasia type 2B syndrome. Interestingly, the patient showed a prolonged survival with good clinical response to tyrosine kinase inhibitors, despite her advanced metastatic MTC. We discuss the challenges in pathology, differential diagnosis, and genetic background for the development of these thyroid lesions.
PubMed: 36176816
DOI: 10.7759/cureus.28423 -
Turkish Neurosurgery 2024Vagal paragangliomas (VPs) are rare tumors arising from paraganglionic tissue within the vagal nerve's perineurium. Usually, benign vascular tumors, VPs tend to invade...
Vagal paragangliomas (VPs) are rare tumors arising from paraganglionic tissue within the vagal nerve's perineurium. Usually, benign vascular tumors, VPs tend to invade the surrounding structures. Herein, we report the case of a VP presenting as a neck mass, which was evaluated as a glomus caroticum tumor preoperatively. A 65-year-old female complaining of a left-sided neck mass and intermittent hoarseness was assessed and operated on for possible glomus caroticum tumor. During the tumor excision, the vagal nerve was also involved, and hence, sacrificed. Histopathological examination revealed an encapsulated tumor associated with a nerve and ganglion and immunohistochemical staining tested positive for succinate dehydrogenase, confirming the diagnosis of VP. Postoperative residual hoarseness was corrected by vocal rehabilitation. While evaluating a retropharyngeal prestyloid neck mass, a VP should always be considered. Surgical excision involving vagal scarification, followed by vocal rehabilitation may be the appropriate treatment strategy.
Topics: Female; Humans; Aged; Hoarseness; Paraganglioma, Extra-Adrenal; Vagus Nerve; Immunohistochemistry; Paraganglioma
PubMed: 37846532
DOI: 10.5137/1019-5149.JTN.40702-22.2 -
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke... Jul 2022To investigate the clinical features, pathological types, imaging features, and surgical strategies of lateral skull base benign tumors with intracranial invasion....
To investigate the clinical features, pathological types, imaging features, and surgical strategies of lateral skull base benign tumors with intracranial invasion. From January 2011 to March 2021, 36 patients of lateral skull base benign tumors with intracranial invasion were included in this retrospective study. Among the 36 patients, 14 cases were male, 22 cases were female, the aged range from 20-67, with the median age of 48. The clinical manifestations, characteristic imaging findings, pathological types, surgical approach selection, and prognosis were analyzed. 36 cases of lateral skull base tumors with intracranial invasion were all accepted surgeries. 23 cases were neurogenic tumors, facial nerve tumors (=8), neurogenic tumors in jugular foramen with unknown origin(=6), hypoglossal schwannoma (=3), transotic intralabyrinthine schwannoma (=3), vestibular schwannoma involving the middle ear(=2), vagal nerve schwannoma(=1). Other types of tumors included meningioma (=10) and paraganglioma (Di 1 or 2,=3). Different pathological types of tumors had different clinical manifestations and imaging manifestations. Sixteen cases were subjected to primary resection, while, other 20 cases underwent staged operation. Among the patients with staged operation, 10 patients had completed the second stage operation, five patients were waiting for the second stage operation, the other five patient's residual intracranial tumor were significantly reduced and the space between tumor and brain tissues widened after the first stage operation, so, the following up with "wait and scan"policy was suggested. The total resection rate of tumors was related to the pathological nature, in which neurogenic tumors were 15/17, and meningiomas were 5/8. The main postoperative complications were cerebrospinal fluid leakage and infection in the operation area. There were two cases of postoperative intracranial infection, and three cases of cerebrospinal fluid leakage occurred in non staged operation cases. Lateral skull base tumors with intracranial invasion are rare. The most common pathological type is schwannoma, followed by meningioma and paraganglioma. For this type of tumor, if there is infection in the operation area and neck invasion is large, it is suggested to choose staged surgery, which can reduce the risk of intracranial infection and the incidence of cerebrospinal fluid leakage. Staged surgery strategy can also reduce the difficulty of second stage surgery, so the operation is much safer than non staged surgery.
Topics: Cerebrospinal Fluid Leak; Cranial Nerve Neoplasms; Female; Humans; Male; Meningeal Neoplasms; Meningioma; Neoplastic Processes; Neurilemmoma; Paraganglioma; Postoperative Complications; Retrospective Studies; Skull Base; Skull Base Neoplasms
PubMed: 35866273
DOI: 10.3760/cma.j.cn115330-20210630-00406