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Background: Bilateral head and neck paragangliomas (HNPGLs) require nuanced management to balance tumor control with functional preservation. Methods: All patients seen at a single‐institution for bilateral paraganglioma between 1983 and 2023 were retrospectively reviewed. Demographics, genetic testing results, and tumor characteristics were analyzed and compared to treatment modality and cranial nerve outcomes. Results: There were 49 patients with 116 tumors (90 carotid body tumors [CBTs], 15 vagal paragangliomas [VPs], and 11 jugular paragangliomas [JPs]). Twenty‐six patients had SDH pathologic variants (PV). Surgical management was more commonly utilized in younger patients (OR: 0.97, 95% CI: 0.950–0.992) and for JPs (OR: 9, 95% CI: 1.386–58.443). In surgical cases, CBTs had a lower risk of postoperative cranial nerve deficits compared to JPs and VPs (OR: 0.095, 95% CI: 0.013–0.692). Conclusions: Younger patients with bilateral HNPGLs, especially those with JP and CBT, are more often treated with surgery. CBTs have lowest risk of cranial nerve deficits after surgery. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries, paragangliomas, and syringomyelia are uncommon diseases. Furthermore, in the absence of any genetic link and with less than five reported adult patients surviving unrepaired rare form of Tetralogy of Fallot, our case shows noteworthiness. The possibility of definitive treatment of these conditions is rendered unsafe due to this persistent defect. Thus, management and ongoing survival of this patient remains complex and challenging. [ABSTRACT FROM AUTHOR]

Background: Head and neck paragangliomas (HNPs) have been associated with gene mutations in the succinate dehydrogenase (SDH) complex, but the clinical significance remains unclear. We sought to explore the demographics, clinical characteristics, treatment methods, and outcomes of SDH‐mutated HNPs. Methods: Databases were systematically searched. Pooled event ratio and relative 95% confidence intervals were calculated for dichotomous outcomes. Meta‐regression was performed. Cochran's Q test and I2 test assessed heterogeneity. Funnel plot and Egger's regression test assessed publication bias. Results: Forty‐two studies with 8849 patients were included. Meta‐regression revealed a significant correlation between multifocality and SDHD mutations (0.03 ± 0.006, p < 0.0001) and between distant metastases and SDHB mutations (0.06 ± 0.023, p = 0.008). There was no correlation between sex, age, tumor size, or familial occurrences and SDH‐related mutations. Conclusion: Multifocality of HNPs correlates with the SDHD mutational subtype, and metastases correlate with the SDHB subtype. Knowledge of HNP phenotypes associated with SDH‐related mutations has the potential to influence the management approach to such HNPs. [ABSTRACT FROM AUTHOR]

Carotid body tumor (CBT) is a rare neck tumor located at the adventitia of the common carotid artery bifurcation. The prominent pathological features of CBT are high vascularization and abnormal proliferation. However, single‐cell transcriptome analysis of the microenvironment composition and molecular complexity in CBT has yet to be performed. In this study, we performed single‐cell RNA sequencing (scRNA‐seq) analysis on human CBT to define the cells that contribute to hypervascularization and chronic hyperplasia. Unbiased clustering analysis of transcriptional profiles identified 16 distinct cell populations including endothelial cells (ECs), smooth muscle cells (SMCs), neuron cells, macrophage cells, neutrophil cells, and T cells. Within the ECs population, we defined subsets with angiogenic capacity plus clear signs of later endothelial progenitor cells (EPCs) to normal ECs. Two populations of macrophages were detectable in CBT, macrophage1 showed enrichment in hypoxia‐inducible factor‐1 (HIF‐1) and as well as an early EPCs cell‐like population expressing CD14 and vascular endothelial growth factor. In addition to HIF‐1‐related transcriptional protein expression, macrophages1 also display a neovasculogenesis‐promoting phenotype. SMCs included three populations showing platelet‐derived growth factor receptor beta and vimentin expression, indicative of a cancer‐associated fibroblast phenotype. Finally, we identified three types of neuronal cells, including chief cells and sustentacular cells, and elucidated their distinct roles in the pathogenesis of CBT and abnormal proliferation of tumors. Overall, our study provided the first comprehensive characterization of the transcriptional landscape of CBT at scRNA‐seq profiles, providing novel insights into the mechanisms underlying its formation. [ABSTRACT FROM AUTHOR]

Malignant carotid body tumors (MCBT) are rare and diagnosed after detection of nodal or distant metastases. This systematic review (SR) focuses on MCBT initially approached by surgery. Preferred Reporting Items for SR and Meta‐Analysis (MA) guided the articles search from 2000 to 2023 on PubMed, Scopus, and Web of Science. Among 3548 papers, 132 (337 patients) were considered for SR; of these, 20 (158 patients) for MA. Malignancy rate was 7.3%, succinate dehydrogenase (SDH) mutation 17%, age at diagnosis between 4th and 6th decades, with a higher prevalence of females. MCBTs were mostly Shamblin III, with nodal and distant metastasis in 79.7% and 44.7%, respectively. Malignancy should be suspected if CBT >4 cm, Shamblin III, painful or otherwise symptomatic, at the extremes of age, bilateral, with multifocal disease, and SDHx mutations. Levels II–III clearance should be performed to exclude nodal metastases and adjuvant treatments considered on a case‐by‐case basis. [ABSTRACT FROM AUTHOR]

Background: Collectively, germline pathogenic variants in succinate dehydrogenase (SDH) genes are the most common cause of hereditary paragangliomas. Loss of immunohistochemical expression of SDHB protein (termed SDH deficiency) occurs whenever there is biallelic inactivation of any SDH gene. We sought to estimate the prevalence of SDH deficiency in patients with carotid body paragangliomas. Methods: We identified all carotid body paragangliomas that had undergone surgical excision at our institution over the last 30 years. If SDHB immunohistochemistry was not performed at the time of excision, it was performed on archived material. Results: There were 64 carotid body paragangliomas identified in the 62 patients. Two‐thirds of the patients were female, and 43 (67%) were SDH‐deficient. Conclusion: Up to two‐thirds of all carotid body paragangliomas are associated with SDH deficiency. Therefore, genetic testing and counseling should be offered to all patients with carotid body paragangliomas, regardless of age or family history. [ABSTRACT FROM AUTHOR]

Paragangliomas are rare neuroendocrine tumors that can be found from the skull base to the pelvis. Head and neck paragangliomas have been historically treated with surgery. However, surgical resection adds risk of injury to vascular structures and cranial nerves that can lead to morbidity such as hoarseness, dysarthria, dysphagia, or aspiration. Recently, improved understanding of the behavior of these tumors and increasing experience in non-surgical treatments, such as observation and radiation therapy, have changed the paradigms of management of this entity. Multiple series now show a trend toward a more conservative management, with a higher percentage of patients being observed or treated with radiotherapy. Several factors should be taken into consideration when deciding the most appropriate treatment for head and neck paragangliomas, starting by differentiating carotid body tumors from non-carotid body tumors. In general, surgical resection is normally recommended for carotid body tumors as the complications from treatment are usually minimal. In contrast, for non-carotid body tumors, surgery is often associated with significant functional impairment due to cranial nerve paralysis. As such, non-surgical treatment is now usually recommended for this subset of head and neck paragangliomas. In young patients with no comorbidities and a small to medium carotid body tumors, surgery should be considered. Moreover, surgery should be offered for secreting tumors, malignant tumors, tumors with rapid growth or increase in symptomatology, and when radiotherapy cannot be performed. Conversely, conservative management with active surveillance or radiotherapy can be offered in the remaining cases in order to avoid unnecessary morbidity while still providing acceptable tumor control. [ABSTRACT FROM AUTHOR]

Background: To assess the efficacy and safety of preoperative embolization (PE) in patients with carotid body tumor (CBTs). Methods: In a single‐center retrospective cohort study, 127 patients underwent surgical resection of CBTs from January 2003 to December 2019. One‐to‐one propensity score matching was conducted between patients with or without PE. Results: Thirty‐two (25.2%) patients received PE. After propensity score matching, no statistically significant differences were found in the baseline characteristics of 28 patients in each group. Compared with NPE group, operative time and estimated blood loss (EBL) were significantly reduced in the PE group. The incidence of stroke, perioperative complications, intraoperative blood transfusion, vascular reconstruction, hospital stay, tumor recurrence, and all‐cause mortality were not different between the PE and NPE group. Conclusions: Preoperative embolization was efficient and safe with a reduction of intraoperative blood loss and operative time during CBT resection. [ABSTRACT FROM AUTHOR]

Background: This study aims to develop and validate a new classification system that better predicts combined risk of neurological and neurovascular complications following CBT surgery, crucial for treatment decision‐making. Methods: Multinational retrospective cohort study with 199 consecutive cases. A cohort of 132 CBT cases was used to develop the new classification. To undertake external validation, assessment was made between the actual complication rate and predicted risk by the model on an independent cohort (n = 67). Results: Univariate analyses showed statistically significant associations between developing a complication and the following factors: craniocaudal dimension, volume, Shamblin classification, and Mehanna types. In the multivariate prognostic model, only Mehanna type remained as a significant risk predictor. The risk of developing complications increases with increasing Mehanna type. Conclusions: We have developed and then validated a new classification and risk stratification system for CBTs, which demonstrated better prognostic power for the risk of developing neurovascular complications after surgery. [ABSTRACT FROM AUTHOR]

Objectives/hypothesis: A carotid body tumor (CBT) is a rare type of tumor that is divided among multiple surgical specialties. Individual surgeons may have limited experience in treating these tumors. We aim to compare different surgical specialties within a single healthcare system to detect variations in management and outcome.Study Design: Retrospective chart review.Methods: A chart review of all patients who underwent surgery for CBT at the University of Pittsburgh Medical Center (UPMC) from 2000 to 2019 was carried out. Univariate and multivariate analysis was used for descriptive statistics, comparison of outcomes, and identification of risk factors.Results: Fifty-eight CBT resection surgeries were performed at UPMC. Patients with advanced tumor were 6.7 (95% confidence interval [CI]: 1.36-32.7) times more likely to undergo preoperative embolization and 8.53 (95% CI: 2.011-36.19) times more likely to sustain carotid artery injury. Advanced-stage tumor resections were associated with greater blood loss (P = .03) and longer hospitalization (P = .02). Collaborative surgeries were associated with higher rates of carotid artery injury (P = .003), residual tumor (P < .001), and longer hospitalization (P = .003), as these combined cases were generally reserved for advanced-stage tumors (P = .02). There were no differences in outcomes between specialties. Of 22 surgeons, the median number of surgeries per surgeon was one (range = 1-12, 54.5%).Conclusions: Surgeons who completed only one surgery for CBT had a greater rate of hospital readmission and greater length of hospital stay. Collaborative surgeries had worse outcomes due to more advanced tumors requiring more complex surgeries.Level Of Evidence: 4 Laryngoscope, 131:E190-E195, 2021. [ABSTRACT FROM AUTHOR]

Background: Carotid paragangliomas are rare tumors. They are usually unique, non‐secreting, resectable, and benign. However, additional rare cases of complex tumors (bilateral, secretory, nonresectable, or malignant) complicate the management and final outcomes. Methods: Records of paragangliomas from our hospital are reviewed. Criteria defining complex paragangliomas have been previously defined. These are compared with those of the simple group. Results: Fifty patients, two groups: simple (n = 39) and complex (n = 11). The patients in the complex group were significantly younger (47.7 vs 63.8 years). Postoperative nerve complications (45.4% vs 6.3%) and mortality during follow‐up (27.3% vs 0%) were significantly more common in the complex group. Vascular complications (0% vs 3.1%) and early mortality (0%) were similarly in both groups. Conclusions: Patients with complex carotid paragangliomas are heterogeneous. The former are younger, exhibit a high degree of diagnostic and therapeutic complexity, and have poorer morbidity and mortality. Surgical experience and interdisciplinary collaboration are essential. [ABSTRACT FROM AUTHOR]

Background: We aimed to review our experience and the changing trends in the management of head and neck paragangliomas (HNPG) over the last three decades. Methods: We retrospectively reviewed 103 patients with HNPG treated at our center (1986‐2017). We included patients treated with surgery, radiotherapy, and patients maintained under active surveillance. Results: Of the surgically treated patients (n = 79), 20% (12/59) of the carotid body tumors (CBT) had a cranial nerve deficit as sequela compared to 95% (19/20) of the non‐CBT. Radiotherapy controlled growth in all tumors treated with this modality (n = 10). Of the initially observed patients, 70% (14/20) remained stable and did not require additional treatment. Stratifying by decades, there was a progressive increase in patients initially attempted to be observed and a decrease in upfront surgery. No deaths attributable to the HNPG were encountered. Conclusions: Surgery is an effective treatment for CBT. Nonsurgical treatment should be considered for non‐CBT. [ABSTRACT FROM AUTHOR]

Backgrounds: Carotid body tumors (CBTs) are rare neoplasms and some of them produce catecholamine. Although operations for catecholamine‐producing CBTs are safe, the relationship between prognosis and endocrine function has not been analyzed before. Methods: Patients diagnosed with CBTs in our department between 2009 and 2018 were analyzed. Plasma catecholamine was examined as a variable of surgical outcomes and prognosis by using statistical methods. Results: Patients who suffered CBTs and underwent operations were divided into two groups according to their plasma catecholamine. Patients in the normal group had more or heavier surgical complications, such as neurological complications (P =.008) and blood loss (P =.03), than those in the high group. However, overall survival, local recurrence, and remote metastasis were not varied significantly in both groups. Conclusions: A high level of plasma catecholamine was a predictor for the improved operative outcomes of CBTs. Hence, nonfunctional CBTs had further short‐term surgical complications. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to evaluate the efficacy of the procedure by analyzing the blood loss and duration of carotid body tumor (CBT) surgery following same‐day preoperative embolization. Methods: We reviewed the medical records of subjects retrospectively. Fifteen patients with 16 CBTs were enrolled in this study. Our same‐day procedure comprises preoperative embolization of the feeding arteries in the morning followed by surgery within 3 hours after the embolization is completed. Results: The mean operative time and the mean amount of blood loss were 138 minutes and 29.3 mL, respectively. The tumor volume after embolization was markedly reduced and the mean reduction rate was 50%. We found that 13 CBTs had more than three feeding arteries. Almost all the postoperative complications, mainly cranial nerve paralyzes, resolved within months after surgery. Conclusion: Our same‐day procedure is a safer and superior alternative to traditional CBT surgery, having good outcomes. [ABSTRACT FROM AUTHOR]

ANI can be used for early detection of parasympathetic signals before the occurrence of bradycardia or to locate unidentified carotid structures in the head and neck region. [ABSTRACT FROM AUTHOR]

Background: The purpose of this study was to investigate the outcomes of surgical resection of carotid body tumors with and without preoperative embolization. Methods: There were 31 patients who underwent surgical resection combined with preoperative embolization (SRE group), and 27 patients who underwent conventional surgical resection (SR group); all clinical data were included and reviewed. Results: There was no difference in the approach for carotid reconstruction in either group (P >.05). The mean surgical time (110.65 ± 35.77 minutes vs 188.33 ± 66.44 minutes) and intraoperative blood loss (140.32 ± 57.12 mL vs 396.43 ± 272.82 mL) were significantly less in the SRE group (P <.05). The volume of blood transfusions required (475 ± 301.18 mL vs 0 mL) and incidence rate of total complications (33.3% vs 9.7%) were higher in the SR group (P <.05). However, the length of hospital stay was similar in both groups (P >.05). Conclusion: Our results demonstrate that preoperative embolization of a carotid body tumor can reduce blood loss and complications and improve tumor excision. [ABSTRACT FROM AUTHOR]

Background: The most common head and neck paraganglioma is the carotid body paraganglioma. Treatment of carotid body tumors is primarily surgical, and uncontrolled growth leads to cranial nerve deficits and more morbid resection. Methods: A 60‐year‐old man was referred for evaluation of carotid body tumor, and workup revealed an internal carotid artery (ICA) aneurysm within the known mass. Results: Interventional Radiology performed angiogram and stenting across aneurysm with interval dramatic reduction in size of mass, and surgery was avoided altogether. Conclusions: Surgical resection is indicated for carotid body paragangliomas when the patient can tolerate the surgery and when the tumor was not very advanced. This patient had a small tumor that initially appeared easily resectable. Failure to detect the ICA aneurysm before resection may have resulted in devastating vascular injury and possible stroke or death. Identification of underlying vascular pathology is essential for safe treatment and should be prioritized, especially considering this case. [ABSTRACT FROM AUTHOR]

Objective: There is no definitive consensus on the impact of preoperative embolization on carotid body paraganglioma management. The purpose of this study was to assess the effects of preoperative embolization on carotid body paraganglioma excision.Data Sources: A systematic search was conducted without limits, and it included studies published on or before July 2013 from PubMed, CINAHL, Web of Knowledge, and the Cochrane Library. Relevant synonyms for the search terms "paraganglioma,""carotid body tumor," and "embolization" were applied.Review Methods: Studies evaluating patients undergoing surgical intervention with embolization for carotid body tumors were included. Two reviewers independently assessed the titles and abstracts for inclusion and extracted the data. The guidelines set forth by the Cochrane Collaboration were followed in the process of data extraction. Data were pooled with a fixed effects model, and standardized mean difference (SMD) and 95% confidence intervals (95% CIs) are reported.Results: A total of 22 studies (15 nonrandomized studies with a comparator, 7 single-arm studies) were included, enrolling 578 patients with 607 tumors. Patients undergoing preoperative embolization had significantly less estimated blood loss compared with those of surgical excision only (12 studies; 295 tumors; SMD: -0.52; 95% CI: -0.77, -0.28). Patients undergoing preoperative embolization had less operative time compared with that of surgical excision only (6 studies; 174 tumors; SMD: -0.46; 95% CI: -0.77, -0.14).Conclusion: Surgical excision with preoperative embolization appears to decrease estimated blood loss and operative time when compared with that without preoperative embolization for carotid body paragangliomas. [ABSTRACT FROM AUTHOR]

Carotid body tumors (paragangliomas) arise from chemoreceptors located at the carotid bifurcation. In imaging studies, this neoplasm may be confused with other neck neoplasms such as thyroid carcinoma. The purpose of this retrospective, cross-sectional study was to describe computed tomographic (CT) and magnetic resonance imaging (MRI) characteristics of confirmed carotid body tumors in a multi-institutional sample of dogs. A total of 16 dogs met inclusion criteria (14 examined using CT and two with MRI). The most common reason for imaging was a palpable cervical mass or respiratory signs (i.e., dyspnea or increased respiratory noises). The most commonly affected breed was Boston terrier ( n = 5). Dogs were predominantly male castrated ( n = 10) and the median age was 9 years [range 3-14.5]. Most tumors appeared as a large mass centered at the carotid bifurcation, with poor margination in six dogs and discrete margins in ten dogs. Masses were iso- to hypoattenuating to adjacent muscles in CT images and hyperintense to muscles in T1- and T2-weighted MRI. For both CT and MRI, masses typically showed strong and heterogeneous contrast enhancement. There was invasion into the adjacent structures in 9/16 dogs. In six of these nine dogs, the basilar portion of the skull was affected. The external carotid artery was entrapped in seven dogs. There was invasion into the internal jugular vein in three dogs, and into the external jugular, maxillary, and linguo-facial veins in one dog. Imaging characteristics helped explain some clinical presentations such as breathing difficulties, Horner's syndrome, head tilt, or facial nerve paralysis. [ABSTRACT FROM AUTHOR]

ABSTRACT Background Recent advances in genetics revealed that 25% to 30% of head and neck paragangliomas (PGLs) are inherited tumors associated with germline mutation, mainly in the succinate dehydrogenase ( SDH) gene. Methods DNA was isolated from whole blood and polymerase chain reaction (PCR) products were sequenced with an ABI3730 × 1 Genetic Analyzer. Results A 30-year-old Korean woman underwent resection of a carotid PGL. Fourteen years later, she was readmitted for a cervical mass. 18F-fluorodeoxyglucose (FDG)-positron emission tomography (PET)/CT revealed a hot spot in the adrenal gland, besides the carotid mass. Surgical pathology confirmed recurrence of the carotid PGL and a concomitant pheochromocytoma. Genetic analysis revealed SDHD c.119del T (p.I40TfsX46) mutation. One daughter has been identified as a carrier. Conclusion We found a novel SDHD mutation from a Korean family that shows similar clinical features to those in other SDHD mutations, mostly from Western countries. Further studies are needed to determine whether similar genotype-phenotype correlations exist in the Asian patients with familial PGLs. © 2014 Wiley Periodicals, Inc. Head Neck 36: E131-E135, 2014 [ABSTRACT FROM AUTHOR]

Background Carotid body tumors are uncommon neoplasms with unique epidemiology and management demands. Cervical embryology is complex. Developmental abnormalities can result in ectopic displacement of native tissues. Methods We present the case of a 21-year-old female with bilateral carotid body paragangliomas who presented to our clinic seeking excision of the symptomatic right-sided tumor. Results The patient was successfully treated with surgical excision. Two rare anatomic variants were identified in her surgical specimen: a carotid sheath parathyroid gland and ectopic thymus tissue. Conclusions This patient represents an unreported combination of pathologic and anatomic phenomena. The paraganglioma resulted from a familial genetic mutation that is well studied in this patient's ethnic population (Dutch), and the literature on this topic is reviewed herein. The 2 anatomic variants likely represent a single, embryologic glitch that will carry no physiologic sequelae. The clinical application of this ectopic anatomy and the common embryologic origins are discussed. © 2011 Wiley Periodicals, Inc. Head Neck, 2013 [ABSTRACT FROM AUTHOR]

Background This study was carried out to explore the use of an intraoperative shunt during surgical resection of complicated carotid body tumors (CBTs). Methods Of the 47 patients who underwent surgical resection for CBT, an intraoperative shunt was performed in 10 patients with complicated CBTs involving the carotid artery wall between January 2005 and August 2010, and their clinical materials were respectively reviewed. Results No severe complications occurred intraoperatively and postoperatively in all the 10 patients with complicated CBTs involving the carotid artery wall. No recurrence and metastasis occurred during follow-up period for a mean of 35.3 months (range, 12-60 months). Conclusions An intraoperative shunt maintained cerebral circulation, decreased the size of tumor by excluding the vascular supply of the external carotid artery, and guided the resection of CBT. The intraoperative shunt was a safe and effective way during surgical resection of complicated CBTs. © 2012 Wiley Periodicals, Inc. Head Neck, 2013 [ABSTRACT FROM AUTHOR]

This study compares clinical, ultrasound, computed tomography ( CT), magnetic resonance imaging ( MRI), and pathology findings in 16 prospectively, and seven retrospectively recruited dogs presented for suspected thyroid carcinoma. Of these, 17 were confirmed thyroid carcinoma, while six were initially misdiagnosed. These included four carotid body tumors, one para-esophageal abscess, and one undifferentiated squamous cell carcinoma. Thyroid carcinomas occurred in older dogs without evidence of sex predilection, and were more often unilateral. All were large, heterogeneous, moderately to strongly vascularized, and most commonly contained areas of dystrophic mineralization and/or fluid accumulations. On MRI, thyroid carcinomas appeared hyperintense compared to surrounding musculature in all imaging sequences used, while on CT they had a lower attenuation value than normal thyroid gland tissue. Histologically confirmed tumor capsule disruption with invasion of the surrounding structures was most commonly detected with MRI. Palpation was not an accurate predictor of locally invasive vs. well-encapsulated masses. Computed tomography had the highest specificity (100%) and MRI had the highest sensitivity (93%) in diagnosing thyroid carcinoma, while ultrasound had considerably lower results. We conclude that ultrasound is adequate for use as a screening tool for dogs with suspected thyroid carcinoma, but recommend either CT or MRI for preoperative diagnosis and staging. [ABSTRACT FROM AUTHOR]

The carotid body is a small mass of tissue inside the carotid bifurcation that reacts to the body's level of oxygen. In rare cases, the carotid body may develop a tumor known as a chemodectoma or paraganglioma. These tumors can vary in size and, typically, they are benign. Although carotid body tumors usually are painless and slow growing, they may cause a compression syndrome that results in symptoms such as dysphagia. The treatment of choice for many carotid body tumors is surgical removal, but there are risks involved with resecting these tumors because of their close location to the carotid vessels and cranial nerves. The use of newer imaging modalities to allow earlier detection of carotid body tumors and careful surgical technique can decrease the complications associated with this challenging surgical procedure. [ABSTRACT FROM AUTHOR]

Background Inflammatory myofibroblastic tumor rarely occurs in the carotid artery, whereas syncope is infrequently associated with neck mass. Methods and Results We reby present a case in which a 56-year-old man with inflammatory myofibroblastic tumor at the left carotid bifurcation and is accompanied by recurrent syncope and falls. He underwent surgery, after which corticosteroids and antibiotics were administered for a short term. No recurrence was observed during the 2-year follow-up. Conclusion To our knowledge, this is the first case of inflammatory myofibroblastic tumor in which recurrent syncope is the only clinical manifestation. © 2016 Wiley Periodicals, Inc. Head Neck 38: E2461-E2463, 2016 [ABSTRACT FROM AUTHOR]

Background Carotid body tumors of Shamblin class III without free internal carotid, between the tumor and skull base, are considered nonresectable. The objective of this work is to describe a surgical technique that combines traditional and endovascular strategies to achieve a safe surgical resection of the carotid body tumor of this class. Methods and Results A female patient with a nonresectable carotid body tumor underwent placement of an endoprosthesis going from the common carotid artery to the internal carotid beyond the skull base (to secure cerebral circulation) excluding the external carotid artery. Forty-five days later, the tumor was resected without vascular or cranial nerves injury, and bleeding amounted to 50 cm3. At 6 months, the patient is tumor free and asymptomatic. Conclusion This is the first known reported case that has been successfully resolved by combining endovascular and traditional strategies. The endoprosthesis maintained cerebral circulation, which otherwisewould have been impossible; besides, the vascular supply from the external carotid artery was excluded leading to a decrease in tumor size and a recovery of the subadventitial dissection plane, allowing for a successful and safe tumor resection. © 2008 Wiley Periodicals, Inc. Head Neck, 2008 [ABSTRACT FROM AUTHOR]

Background Chemodectomas of the head and neck are tumors that originate from the neural crest. The authors report a case of carotid body tumor with chronic hypoxia secondary to congenital cyanogenic cardiac malformation, radiologically and surgically treated. Methods and Results A 37-year-old woman presented 1-year history of a slow-growing right lateral-cervical swelling. Radiologic examination led to a suspicion of carotid body tumor. Her history revealed thoracic situs solitus and a complex congenital heart disease. She was hospitalized for a selective angiography of the cervical vessels. Twenty-four hours later, the tumor was completely removed under local anesthesia. One year later, there were no signs of recurrence, and the patient showed a correct hemodynamic compensation. Conclusions The choice of surgical resection with selective preoperative embolization, which induced the obliteration of the feeder vessels, devascularizing the tumor and avoiding significant intraoperative bleeding, was successful. © 2008 Wiley Periodicals, Inc. Head Neck, 2008 [ABSTRACT FROM AUTHOR]

Familial paragangliomas of the neck are often bilateral and more aggressive than spontaneous forms. Tumors appear earlier (2nd–4th decade) often with diffuse, multifocal involvement. Without treatment, these tumors can lead to significant morbidity. Three families with succinate dehydrogenase subunit D (SDHD) germline mutations underwent clinical and genetic evaluation. Patients were screened using ultrasound and evaluated further with conventional and functional imaging. Tumors with a diameter >1.5 cm were surgically removed. Multicentric and bilateral tumors were detected in 9/13 (69%) and 8/13 (62%) patients, respectively. Surgical morbidity occurred in 64% of patients. Local recurrence was 57%, although this was lower in tumors with a diameter <2 cm. We recommend an algorithm for a systematic approach to the diagnosis, monitoring, and treatment of familial head and neck paragangliomas. Operative treatment in advanced stages often leads to unwanted morbidity, such that earlier detection and treatment of smaller tumors seems to be of benefit. © 2007 Wiley Periodicals, Inc. Head Neck 2007 [ABSTRACT FROM AUTHOR]

Objective/Hypothesis To determine the heritable proportion of paraganglioma (PGL) and identify clinical features associated with heritable PGL. Study Design Patients diagnosed with head and neck PGLs, identified retrospectively through clinical otolaryngology practices and/or participation in previous PGL research studies, were given a medical and family history questionnaire. Methods Questionnaire information was used to classify participants as having 'heritable' or 'non-heritable' cases of PGL. Classification of the participants identified through otolaryngology clinics was used to estimate the heritable proportion of PGL. Statistical analysis was performed to identify significant differences in the clinical characteristics of the heritable versus non-heritable groups. Results Among the otolaryngology clinic population, 35% were classified as having heritable PGL. Individuals with heritable PGL were younger on average than those with non-heritable PGL. The majority of non-heritable participants were female, but there was an equal gender ratio among the heritable participants. Individuals diagnosed with a carotid body tumor (CBT) were 5.8 times more likely to be classified as heritable than those diagnosed with PGL at other anatomic locations. Conclusions Approximately 35% of individuals who present to an otolaryngologist with a head and neck PGL have inherited a predisposition for this growth. Among individuals diagnosed with head and neck PGL, those diagnosed with CBT are 5.8 times more likely to have an inherited predisposition than those diagnosed with PGL at other anatomic locations. [ABSTRACT FROM AUTHOR]

Carotid body tumors are rare neoplasms that have to be considered in the evaluation of all lateral neck mass. Early surgical removal has been recommended to avoid possible cranial nerve injury, the most common perioperative complication. Computed tomography (CT) and magnetic resonance imaging (MRA) angiographies are the preferred pre-operative diagnostic imaging investigations, as well as the 111 In-pentetreotide scintigraphic scan, whereas the standard ultrasound investigations have poor sensitivity in characterizing of the blood flows of the parenchimal structure of the carotid body tumors. We describe a case of a patient with a carotid body tumor assessed with contrast ultrasonography that clearly improved the quality of the standard color Duplex. This technique may represent a non-invasive method, easy to use and to repeat, and able to achieve high diagnostic accuracy. [ABSTRACT FROM AUTHOR]