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Der Chirurg; Zeitschrift Fur Alle... Jan 2019The therapies available for the rare tumor entity of cervical paraganglioma (PG) are currently undergoing a paradigm shift. The treatment of choice for small carotid... (Review)
Review
The therapies available for the rare tumor entity of cervical paraganglioma (PG) are currently undergoing a paradigm shift. The treatment of choice for small carotid body tumors, malignant and active endocrine tumors is surgical resection; however, for locally advanced carotid body tumors and vagal PG, surgical therapy should be critically evaluated. Due to the immediate proximity of these hypervascularized tumors to the caudal cranial nerves, there is a risk of severe nerve damage with a significant impairment of quality of life after resection, particularly for locally advanced cervical PG, emphasizing further the importance of a restrictive surgical strategy. External radiotherapy can provide an equivalent primary therapeutic option with respect to the rate of recurrence and is accompanied by a lower morbidity. The slow rate of tumor progression and the multifocality of the familial variant of cervical PG or significant comorbidities in older, asymptomatic patients warrant a less aggressive treatment strategy for these tumors. When a wait and scan approach is implemented, a closely monitored radiological and clinical re-evaluation is of upmost importance. In a multidisciplinary approach the following critical points require consideration before a therapy is implemented,: size and location of the tumor, progression rate, genetic background, patient age and general condition, relevant comorbidities, the presence of synchronous PG and/or vasoactive catecholamine-producing tumors. Although best practice algorithms for the treatment of cervical PG have already been devised, recent innovative developments have led to more patient-tailored, individualized treatment approaches.
Topics: Aged; Head and Neck Neoplasms; Humans; Neoplasm Recurrence, Local; Paraganglioma; Quality of Life
PubMed: 30242437
DOI: 10.1007/s00104-018-0734-y -
Journal of Clinical Medicine Sep 2018Paragangliomas are neuroendocrine neoplasms, derived from paraganglia of the sympathetic and parasympathetic nervous systems. They are most commonly identified in the... (Review)
Review
Paragangliomas are neuroendocrine neoplasms, derived from paraganglia of the sympathetic and parasympathetic nervous systems. They are most commonly identified in the head and neck, being most frequent in the carotid body, followed by jugulotympanic paraganglia, vagal nerve and ganglion nodosum, as well as laryngeal paraganglia. Abdominal sites include the well-known urinary bladder tumors that originate in the Organ of Zuckerkandl. However, other unusual sites of origin include peri-adrenal, para-aortic, inter-aortocaval, and paracaval retroperitoneal sites, as well as tumors in organs where they may not be expected in the differential diagnosis of neuroendocrine neoplasms, such as thyroid, parathyroid, pituitary, gut, pancreas, liver, mesentery, lung, heart and mediastinum. The distinction of these lesions from epithelial neuroendocrine neoplasms is critical for several reasons. Firstly, the determination of clinical and biochemical features is different from that used for epithelial neuroendocrine tumors. Secondly, the genetic implications are different, since paragangliomas/pheochromocytomas have the highest rate of germline susceptibility at almost 40%. Finally, the characterization of metastatic disease is unique in these highly syndromic lesions. In this review, we summarize updated concepts by outlining the spectrum of anatomic locations of paragangliomas, the importance of morphology in establishing the correct diagnosis, the clinical implications for management, and the impact of genetics on the distinction between multifocal primary tumors compared with malignant disease.
PubMed: 30217041
DOI: 10.3390/jcm7090280 -
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke... Jul 2020To study the clinical features, diagnosis and treatment of vagal paraganglioma in parapharyngeal space. Nine cases with vagal paraganglioma in parapharyngeal space...
To study the clinical features, diagnosis and treatment of vagal paraganglioma in parapharyngeal space. Nine cases with vagal paraganglioma in parapharyngeal space were retrospectively analyzed who were diagnosed and treated between January 2006 and December 2018 in Department of Otorhinolaryngology Head and Neck Surgery, Beijing Friendship Hospital and the First Medical Center, Chinese PLA General Hospital. There were 6 males and 3 females, aged from 24 to 50 years old. The main symptoms in the 9 patients were hoarseness and neck mass, and the secondary symptoms were irritating cough, cough on drinking and dysphagia. The main sign was a well-circumscribed round mass, tough in texture, with or without ipsilateral lateral oropharyngeal wall uplift and vocal cord paralysis. The tumors were located between the bifurcation of the carotid artery and the jugular foramen in 7 cases and intruded into jugular fossa in 2 cases. All the 9 patients underwent head and neck enhancement CT and MRI and 7 cases received digital subtraction angiography (DSA) examination and balloon occlusion test. The imaging features were tumors with rich blood supply in the parapharyngeal space of the upper neck, and the tumors were heterogeneous enhanced with contrast CT scan and enhanced MRI, which were closely related to the internal carotid artery, external carotid artery and jugular vein. Among these 9 patients, 8 underwent surgical resection of tumors, including complete tumor resection in 7 cases and partial tumor resection in 2 case. One patient underwent partial tumor resection after being transferred to vascular surgery. There was no recurrence in 7 patients with complete tumor resection and slow growth was shown in 2 patients with partial tumor resection. Posterior cranial nerve injury occurred in 2 patients and stroke in 1 patient due to intraoperative ligation of internal carotid artery. Vagal paraganglioma in the parapharyngeal space is rich in blood supply and closely related to the internal and external carotid arteries, internal jugular vein and posterior cranial nerves. Surgical resection is the first choice for treatments. Choosing a reasonable operative approach for fully exposing the operative field and completely removing the tumor while protecting the internal carotid artery are the keys to successful surgery.
Topics: Adult; Female; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Paraganglioma; Parapharyngeal Space; Retrospective Studies; Treatment Outcome; Young Adult
PubMed: 32668877
DOI: 10.3760/cma.j.cn115330-20191209-00751 -
Journal of Voice : Official Journal of... Jul 2023We present a prospective case series that aimed to report the functional (voice and swallowing) outcomes of delayed laryngeal reinnervation following vagal interruption...
PURPOSE
We present a prospective case series that aimed to report the functional (voice and swallowing) outcomes of delayed laryngeal reinnervation following vagal interruption by resection of vagal paraganglioma and schwannoma.
MATERIALS AND METHODS
A dedicated, anonymized database was established in 2012 with a minimum eighteen-month follow up set for this report. Internationally validated self- and observer-reported measures were recorded preoperatively and at six, 12 and, 18 months together with demographics, diagnoses, and operative details.
RESULTS
A total of eight patients with a median age of 46 (37-54) underwent excision of vagal paraganglioma (five) and schwannoma (three) with few mild complications. Three underwent selective and five non selective reinnervation. Seven out of eight patients underwent synchronous injection medialization. The voice handicap index (VHI-30) improved from a baseline median 83 (range 52-102) to 7.5 (5-58) at 18 months; maximum phonation time improved from median 8 (range 5-15) to 10.5 (8.5-11); voice grade ("G" in grade, roughness, breathiness, asthenia, and strain [GRBAS] scoring) improved from median three (severe impairment, range 0-3) to one (mild impairment, 0-2); Eating Assessment Tool (EAT-10) score improved from median 12 (range 3.5-27) preoperatively to one (0-16); and reflux symptom index (RSI) improved from median 25 (range 17-36) to 7 (0-36). One patient exhibited no discernible reinnervation, while the remainder exhibited good cord bulk and tone, though without purposive abduction.
CONCLUSION
Delayed laryngeal reinnervation for high vagal paralysis is a safe technique associated with good voice and swallowing outcomes by 12-18 months. Potential confounders in this small series and the absence of a control arm both limit conclusions, but this study suggests that further prospective, controlled studies, and/or case registration are merited.
Topics: Humans; Vocal Cord Paralysis; Treatment Outcome; Larynx; Paraganglioma; Neurilemmoma; Recurrent Laryngeal Nerve
PubMed: 33994255
DOI: 10.1016/j.jvoice.2021.03.021 -
Journal of Neurological Surgery. Part... Dec 2017Treatment for head and neck paragangliomas (HNGPL) can be more harmful than the disease. After diagnosis, an initial period of surveillance is often indicated, and...
Treatment for head and neck paragangliomas (HNGPL) can be more harmful than the disease. After diagnosis, an initial period of surveillance is often indicated, and surgery or radiotherapy is reserved for progressive disease. With the aim to optimize this "wait and scan" strategy, we studied growth and possible predictors. A retrospective cohort study was conducted. This study was conducted at a tertiary referral center for patients with HNGPL. Tumor volume was estimated for 184 -related carotid and vagal body paragangliomas using sequential magnetic resonance imaging. Cox regression was used to study predictors of tumor growth. The estimated fraction of growing tumors ranged from 0.42 after 1 year of follow-up to 0.85 after 11 years. A median growth rate of 10.4 and 12.0% per year was observed for carotid and vagal body tumors, respectively. Tumor location, initial volume, and age ( < 0.05) were included in our prediction model. The probability of growth decreased with increasing age and volume, indicating a decelerating growth pattern. We created a prediction model (available online), enabling a more individualized "wait and scan" strategy. The favorable natural course of carotid and vagal body paragangliomas was confirmed; although with long follow-up growth will be observed in most cases.
PubMed: 29134169
DOI: 10.1055/s-0037-1604347 -
Journal of Cardiothoracic Surgery May 2020Paragangliomas are rare endocrine tumors that arise from the extra-adrenal autonomic paraganglia and sympathetic paragangliomas usually secret catecholamines and are...
INTRODUCTION
Paragangliomas are rare endocrine tumors that arise from the extra-adrenal autonomic paraganglia and sympathetic paragangliomas usually secret catecholamines and are located in the sympathetic paravertebral ganglia of thorax, abdomen, and pelvis. In contrast, most parasympathetic paragangliomas are nonfunctional and located along the glossopharyngeal and vagal nerves in the neck and at the base of the skull. Such neoplasms, although rare, are clinically important because they may recur after surgical resection and 10% of them give rise to metastases causing death with the lymphatic nodes, bones, liver, and lungs being the most common locations.
CASE PRESENTATION
We present a case of a 26-year-old male patient that was diagnosed with paraganglioma of the right-frontal lobe infiltrating the falx and frontal bone which was diagnosed after suffering from a headache and abnormal vision. On initial work-up he was found to have right pulmonary nodules that increased in size after follow up and other nodules appeared in the contralateral lung. He underwent subtotal resection of the brain tumor and complete resection of the bilateral pulmonary nodules.
CONCLUSION
To our knowledge, paraganglioma is considered to be a rare entity in the central nervous system with very few cases being reported in the supratentorial region and no cases were reported of metastatic such paraganglioma to the lung.
Topics: Adult; Brain; Humans; Lung Neoplasms; Magnetic Resonance Imaging; Male; Multiple Pulmonary Nodules; Neoplasm Metastasis; Neoplasm Recurrence, Local; Paraganglioma; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 32393294
DOI: 10.1186/s13019-020-01113-2 -
Journal of Neurosciences in Rural... Jan 2021The aim of the study is to determine the surgical candidacy and nuances of skull base paraganglioma surgery in the era of radiotherapy. This was a retrospective...
The aim of the study is to determine the surgical candidacy and nuances of skull base paraganglioma surgery in the era of radiotherapy. This was a retrospective observational study conducted in patients who presented with skull base paragangliomas between January 2017 and December 2019. Primary data, including indication for surgery, the approach used, the extent of resection, complications, and postoperative lower cranial nerve status were studied. A total of 21 cases of skull base paragangliomas were analyzed, including seven cases of tympanic paraganglioma, 10 cases of jugular foramen paraganglioma, three cases of multiple paragangliomas, and one case of vagal paraganglioma. Indications for surgery were young age, bleeding from ear, neck mass with upper aerodigestive pressure symptoms, lower cranial nerve paralysis, and patients with intracranial pressure symptoms. Total excision was done in 11 patients, near-total excision in five patients, subtotal in three patients, and surgery was not done in two patients. Facial nerve paralysis was the most common complication observed, followed by bleeding and flap necrosis. Radiotherapy was considered as adjuvant treatment wherever indicated. A thorough knowledge and understanding of the pathophysiology of the skull base paragangliomas and its management strategies can help to achieve excellent results in terms of tumor clearance and reduction in complications. A multidisciplinary team approach and meticulous skull base surgical techniques have a significant role to play in the management of paragangliomas, especially in developing countries where availability of radiosurgery is still a challenge.
PubMed: 33531769
DOI: 10.1055/s-0040-1721201 -
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 -
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 -
International Journal of Surgery Case... 2018Paraganglioma of head and neck is a rare tumor and vagal schwannoma is even rarer. The majority of patients with vagal schwannoma presents with a lateral neck mass. Its...
BACKGROUND
Paraganglioma of head and neck is a rare tumor and vagal schwannoma is even rarer. The majority of patients with vagal schwannoma presents with a lateral neck mass. Its management is delicate as the need to confirm the diagnosis by histopathology via a biopsy is contraindicated. Here, is a case of a young female with vagal schwannoma complicated with hoarseness after the biopsy of the mass, which persists after extirpation of the tumor.
CASE DESCRIPTION
A 22-year old lady presented with a history of a right neck mass for a 5-months duration. Clinical examination revealed a mass at level II neck region which measures 3.0 cm × 2.0 cm and it was mobile, non-pulsatile and had smooth surfaced. CT scan and angiogram showed that the mass arose between the carotid artery and vagal nerve and it was a highly vascular lesion. A CT scan-guided biopsy performed but complicated with neck hematoma and patient developed hoarseness. On follow up, her hoarseness persists and her tissue biopsy came back as schwannoma. She was counseled regarding surgery versus radiation for her treatment and she agreeable for surgery. Hence, surgical excision was performed and intraoperatively the mass visualized arising from the vagal nerve. Postoperatively however, her voice did not improve.
CONCLUSION
Vagal schwannoma is a rare paraganglioma of head and neck and the best treatment is still controversial. Unnecessary investigation and procedure should be avoided in order to reduce morbidity as well as improves patient's quality of life.
PubMed: 30366175
DOI: 10.1016/j.ijscr.2018.10.025