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Journal of Neurological Surgery. Part... Feb 2019To improve our understanding of the natural course of head and neck paragangliomas (HNPGL) and ultimately differentiate between cases that benefit from early...
To improve our understanding of the natural course of head and neck paragangliomas (HNPGL) and ultimately differentiate between cases that benefit from early treatment and those that are best left untreated, we studied the growth dynamics of 77 HNPGL managed with primary observation. Using digitally available magnetic resonance images, tumor volume was estimated at three time points. Subsequently, nonlinear least squares regression was used to fit seven mathematical models to the observed growth data. Goodness of fit was assessed with the coefficient of determination ( ) and root-mean-squared error. The models were compared with Kruskal-Wallis one-way analysis of variance and subsequent post-hoc tests. In addition, the credibility of predictions (age at onset of neoplastic growth and estimated volume at age 90) was evaluated. Equations generating sigmoidal-shaped growth curves (Gompertz, logistic, Spratt and Bertalanffy) provided a good fit (median : 0.996-1.00) and better described the observed data compared with the linear, exponential, and Mendelsohn equations ( < 0.001). Although there was no statistically significant difference between the sigmoidal-shaped growth curves regarding the goodness of fit, a realistic age at onset and estimated volume at age 90 were most often predicted by the Bertalanffy model. Growth of HNPGL is best described by decelerating tumor growth laws, with a preference for the Bertalanffy model. To the best of our knowledge, this is the first time that this often-neglected model has been successfully fitted to clinically obtained growth data.
PubMed: 30733904
DOI: 10.1055/s-0038-1667148 -
Head & Neck Jun 2019F-FDOPA PET/CT was proved to be a highly sensitive imaging method for detecting head and neck paraganglioma (HNPGL). The primary aim of the study was to evaluate the...
BACKGROUND
F-FDOPA PET/CT was proved to be a highly sensitive imaging method for detecting head and neck paraganglioma (HNPGL). The primary aim of the study was to evaluate the relationship between tumor characteristics and the SDHx-mutational status in a large series of patients with HNPGL evaluated by F-FDOPA PET/CT.
METHODS
A total of 104 patients with HNPGL (65 sporadic/39 SDHx-mutated) were included.
RESULTS
In comparison to SDHB/SDC/SDHx-negative cases, patients with SDHD were younger at diagnosis and had a higher rate of multifocal, vagal, and carotid paraganglioma. In patients with SDHD, vagal paraganglia represented the primary site of tumor origin. Multicentric involvement of the vagus nerve alone or in association with other locations was found to be a typical feature of SDHD cases compared to other cases (odds ratio = 59.4).
CONCLUSION
The present study shows that tumor multifocality within the vagus nerve is a phenotypic marker of SDHD mutation. This information is essential in the choice of the therapeutic strategy.
Topics: Age Factors; Cranial Nerve Neoplasms; Dihydroxyphenylalanine; Female; Fluorine Radioisotopes; Head and Neck Neoplasms; Heterozygote; Humans; Male; Middle Aged; Mutation; Neoplasms, Multiple Primary; Paraganglioma, Extra-Adrenal; Phenotype; Positron Emission Tomography Computed Tomography; Retrospective Studies; Succinate Dehydrogenase; Vagus Nerve; Vagus Nerve Diseases
PubMed: 30584686
DOI: 10.1002/hed.25604 -
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 -
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 -
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 -
Case Reports in Otolaryngology 2017A 70-year-old female presented with a neck mass and sporadic dry cough, often leading to fits of coughing severe enough to cause vomiting. The patient reported that...
A 70-year-old female presented with a neck mass and sporadic dry cough, often leading to fits of coughing severe enough to cause vomiting. The patient reported that touching the mass triggered the cough. On examination, a 2.5 cm right-sided level two neck mass deep to the sternocleidomastoid was present. Palpation of the mass immediately triggered coughing. Cross-sectional imaging proposed vagal paraganglioma as the chief differential, which was confirmed following surgical excision. The patient reported complete resolution of her severe dry cough after surgery. Vagal paragangliomas are rare neuroendocrine tumors arising from the neural crest-derived paraganglionic tissue surrounding the vagus nerve, typically presenting as a neck mass associated with hoarseness or pulsatile tinnitus. To the best of our knowledge this is a unique description in the English literature. This case is presented to aid physicians should they encounter a neck mass associated with cough. Vagal paraganglioma, although rare, should be part of the differential in such a presentation.
PubMed: 28717527
DOI: 10.1155/2017/7603814 -
European Annals of Otorhinolaryngology,... Dec 2017Vagal paraganglioma are rare tumors that are mostly asymptomatic. We report a case of vagal paraganglioma associated with paraneoplastic polymyalgia rheumatica and...
INTRODUCTION
Vagal paraganglioma are rare tumors that are mostly asymptomatic. We report a case of vagal paraganglioma associated with paraneoplastic polymyalgia rheumatica and review the literature on benign paragangliomas of the head and neck associated with paraneoplastic syndrome.
CASE REPORT
A 53-year-old man presented with atypical polymyalgia rheumatica. MRI revealed a tumor that was then surgically excised. Histological examination confirmed the diagnosis of benign vagal paraganglioma. Rapid, complete and permanent resolution of all rheumatological symptoms were observed postoperatively, confirming the diagnosis of paraneoplastic polymyalgia rheumatica.
CONCLUSION
Paraganglioma of the neck associated with paraneoplastic syndrome remains exceptional. A predisposing gene mutation must be systematically investigated. Long-term surveillance must be ensured due to the risk of local recurrence, second tumors or metastasis.
Topics: Cranial Nerve Neoplasms; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Paraganglioma, Extra-Adrenal; Polymyalgia Rheumatica; Treatment Outcome; Vagus Nerve Diseases
PubMed: 28412079
DOI: 10.1016/j.anorl.2017.03.005 -
Head and Neck Pathology Sep 2017Paragangliomas (PGL) develop from the parasympathetic system in the head and neck (HN) and arise primarily in four distinct areas: Carotid body, vagal, middle ear, and... (Review)
Review
Paragangliomas (PGL) develop from the parasympathetic system in the head and neck (HN) and arise primarily in four distinct areas: Carotid body, vagal, middle ear, and larynx. Globally, the diagnosis and morphologic features are the same regardless of anatomic site, however the incidence, frequency of genetic alterations/syndromes and differential diagnosis vary. It is now recognized that nearly 40% of all HN PGLs are hereditary, including a significant subset without a known family history. Now pathologists are central to the evaluation for diagnosis and further management of patients with HNPGLs. Specifically, SDHB immunohistochemical evaluation is an excellent screening tool to detect tumors with alterations in the SDH family of genes that represent the majority of hereditary cases in HNPGL. Similarly, SDHB immunohistochemical analysis allows for screening of PGL syndrome associated tumors (gastrointestinal stromal tumor (GIST), renal cell carcinoma (RCC), and pituitary adenomas) that have now been linked by their overlapping gene alterations. Awareness of the spectrum of these syndromes, and their associated tumors, positions the pathologist to augment patient care and surveillance.
Topics: Head and Neck Neoplasms; Humans; Paraganglioma, Extra-Adrenal
PubMed: 28321772
DOI: 10.1007/s12105-017-0803-4 -
Journal of Ultrasound Mar 2017Vagal paragangliomas are rare benign tumors accounting for only 3% of head and neck paragangliomas and arise from vagal ganglions, predominately the nodose ganglion in...
Vagal paragangliomas are rare benign tumors accounting for only 3% of head and neck paragangliomas and arise from vagal ganglions, predominately the nodose ganglion in the carotid sheath. Imaging plays an important role in their diagnosis, differentiation from more common lesions presenting in the carotid space, and management. MRI is the method of choice for investigation when paragangliomas are clinically suspected. The typical salt-and-pepper appearance in T1-weighted images is well known but not specific to paragangliomas. Demonstration of the continuity of the lesion with the vagus nerve on magnetic resonance imaging (MRI) further asserts the diagnosis of glomus vagale. Although this tumor can be diagnosed on a contrast-enhanced computed tomography (CECT) examination, it is limited by the non-demonstration of its neural origin. Sonography is considered only a preliminary investigation in patients, and a CECT and/or MRI study of the neck is conducted before treatment planning. This case is unique as a confident diagnosis of glomus vagale was made on high-resolution sonography of the neck. A middle-aged male patient presenting with a neck swelling was diagnosed with a glomus vagale tumor on USG by the direct demonstration of its continuity with the vagus nerve, and Doppler examination confirmed its vascularity. The diagnosis was confirmed on histopathology after surgical excision. The sonographic appearance, Doppler characteristics, and imaging differential diagnosis of glomus vagale are discussed in detail.
Topics: Diagnosis, Differential; Glomus Tumor; Head and Neck Neoplasms; Humans; Immunohistochemistry; Male; Middle Aged; Neck; Paraganglioma, Extra-Adrenal; Tomography, X-Ray Computed; Ultrasonography, Doppler; Vagus Nerve
PubMed: 28298947
DOI: 10.1007/s40477-016-0237-y