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The objective of this study is to evaluate the effect of preoperative embolization on carotid body tumor resection. A systematic review and meta-analysis were conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. PubMed, Scopus, and Web of Science were screened for studies published between 2010 and 2022. Primary outcomes investigated were intraoperative blood loss, operative time, length of hospital stay, and perioperative complications such as transient ischemic attack (TIA)/stroke, vascular injury, and cranial nerve injury (CNI). A random effects model was used in cases where study heterogeneity was high. Overall, 25 studies were included in the systematic review, involving 1649 patients: 23 studies were eligible for meta-analysis. The incidence of vascular injury was significantly less in the preoperative embolization group (odds ratio (OR) = 0.60; 95% CI: 0.42–0.84; P =.003). There was no statistically significant difference between the two groups regarding intraoperative blood loss, operative time, length of hospital stay, incidence of TIA/stroke, and CNI. Subgroup analyses did not demonstrate significant difference between Shamblin I, II, and III subgroups regarding operative time. This meta-analysis found preoperative embolization to be significantly beneficial in reducing incidence of vascular injury. [ABSTRACT FROM AUTHOR]

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]

Carotid body tumors (CBTs) are rare, hypervascular neuroendocrine neoplasms that can lead to significant complications during surgical resection due to the high risk of bleeding. We present the case of a 64-year-old male with a palpable neck mass on the left side, diagnosed as a CBT through imaging studies. Given the tumor's increasing size and rich vascularization, percutaneous preoperative embolization with Onyx under ultrasound and angiographic guide, was performed to minimize intraoperative complications. Forty-eight hours later, the tumor was surgically resected with minimal blood loss and no injury to cranial nerves or the carotid bifurcation.

A carotid body tumor is an uncommon tumor that develops from the carotid body. Carotid body tumor, also called paraganglioma, is often benign in nature and mostly found in the neck. They make up 0.5% of all body tumors and resemble glomus jugulare, glomus tympanicum, and pheochromocytoma, which are paragangliomas of the body. We present a case of a 22-year-old male patient who presented to the medical outpatient department with complaints of swelling in the left carotid triangle for 1 month. The patient had hoarseness of voice with odynophagia and dysphagia. We found out the diagnosis of carotid body tumor when the patient came for ultrasound, and the diagnosis was later confirmed on magnetic resonance imaging. The gold standard treatment for carotid body tumors is surgery. The surgical categorization by Shamblin et al. marks a turning point in the evaluation of these tumors’ resectability and is still used to predict vascular morbidity, and according to it, our patient later underwent sub adventitial tumor excision.

Background: This study aims to determine the efficacy and safety of preoperative embolization in the management of Shamblin type III carotid body tumors (CBT). Method: In this retrospective study, patients with Shamblin type III CBT were included between January 2005 and January 2017. A total of 48 Patients were divided into preoperative embolization (SRE, n = 25) and non-preoperative embolization group (SR, n = 23). Result: Mean surgical time (145.24 ± 19.86 min vs 186.91 ± 17.808 min, P < 0.05) and intraoperative blood loss (271.4 ± 73.001 mL vs 380.36 ± 39.822 mL, P < 0.05) were markedly reduced in the SRE group compared with SR group. The preoperative tumor volume in the SRE group was larger than that in the SR group, but the volume was similar between the two groups after surgery. The number of tumor residual cases was higher in the SR group. The incidence of complications and duration of hospitalization were comparable between the two groups. Conclusion: This study demonstrates the efficacy of preoperative embolization in reducing the duration of surgery and volume of blood loss during the process of CBT resection. More prospective, well-designed studies are urgently needed to validate the current findings. [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]

Carotid body tumors (CBTs), or chemodectomas, are rare, especially in the pediatric population. They often present with minimal symptoms, making timely diagnosis challenging. This case report and systematic review highlights a distinctive presentation and summarize the current evidence on pediatric CBTs. We report a case of a 13-year-old girl presenting with neck pain and a left-sided neck mass. After extensive evaluation, a Shamblin type III tumor was identified and removed surgically. Postoperatively, the patient experienced transient hypertension and significant dysphagia, both of which resolved within a few weeks with no permanent sequelae. Histology confirmed a benign paraganglioma. A systematic literature review of PubMed identified 29 cases from 23 published studies spanning from 1968 to 2024. The average age at diagnosis was 12.6 ± 3.6 years. The most common symptom was a neck mass or swelling, reported in 75% of cases (n = 21). Tumor sizes ranged from 1.3 to 8.0 cm, with Shamblin III being the most frequent classification. Gross total resection (n = 25 [89.3%]) alone or in combination with preoperative embolization (n = 10 [35.7%]) were the most common methods of management. In 62.1% of cases, there were no permanent complication or sequelae. The proximity to vital neurovascular structures and high vascularity in pediatric patients necessitates careful perioperative interdisciplinary management. Owing to their rarity and nonspecific presentation, CBTs often remain undiagnosed for years. They respond well to treatment, but can be fatal if untreated, underscoring the importance of including CBTs in the differential diagnosis of pediatric neck masses.

Abstract Background Carotid body tumors are uncommon neuroendocrine growths near the carotid bifurcation. While some advocate preoperative embolization to minimize bleeding, others avoid it due to complications. This study shares the experience of a single center in managing patients with carotid body tumors without practicing preoperative embolization. Methods This was a cross-sectional study of patients with carotid body tumors managed between 2020 and 2024. Data were collected from the hospital’s registry. When necessary, routine blood tests, neck ultrasonography, and computed tomography scans were conducted. The tumors were categorized according to Shamblin’s classification. The average duration of follow-up was 20 months. Results The study involved 25 patients, 22 (88%) females and 3 (12%) males. Their ages ranged from 27 to 85 years old. Twenty (80%) cases presented with neck swelling, and six (24%) had a positive medical history. Tumors were mainly on the right side (52%), with 20 (80%) showing ill-defined neck masses. Tumor sizes ranged from 1.5 to 7 cm, with Shamblin type II tumors being discovered in the majority of cases (72%). Types of tumors were significantly associated with the tumor size (p-value

Background: Carotid body tumors (CBTs) are rare benign tumors that arise from the chemoreceptor tissue located at the carotid bifurcation that require excision if symptomatic. Depending on the size and location of the tumor, the carotid artery may need to be repaired after resection. This study aims to assess whether CBT excision with artery resection had higher rates of 30-day postoperative outcomes compared with CBT excision without artery resection. Methods: This is a retrospective cohort study. Patients were identified in the American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP) registry from 2005 to 2017. They were divided into two groups, based on Current Procedural Terminology (CPT®) codes: group A had CBT excision without carotid artery excision (CPT code 60600) and group B with carotid artery excision (CPT code 60605). Patient demographic characteristics, co-morbidities, and 30-days postoperative outcomes were compared between the two groups. Categorical data were analyzed using Pearson’s X2 or Fisher exact tests and presented as proportions (percentages). Continuous data were analyzed using parametric or non-parametric tests as appropriate. Statistical significance was defined as p < 0.05. Statistical analysis was performed using the SPSS statistical software package. Results: A total of 463 patients were identified, 410 (88.4%) in group A and 53 (11.4%) in group B. Overall, there were 291 (62.9%) women. A higher proportion of women underwent CBT excision only, compared to men (91.1% [265/291] vs. 84.3% [145/172], p < 0.0001). Demographics and comorbidities were similar between groups. There was no significant difference in the 30-day postoperative outcomes. The reoperation rate was higher in group B (3.8% vs. 1.5%, p = 0.334), while the readmission rate was higher in group A (3.2% vs. 0% p = 0.269), and both were not significantly different. Overall morbidity and serious morbidity were higher in group B (7.5% vs. 5.9%, p = 0.626) and lower in group A (5.7% vs. 3.9%, p = 0.544), respectively, but were not significantly different. Operative time (mean, SD) was higher in group B (187 ± 107 vs. 138 ± 66 min, p < 0.001). However, the median (IQR) of hospital length of stay (LOS) was similar (2 [1, 4] vs. 2 [1, 3] days, p = 0.134). Conclusions: Overall, no difference was noted in the 30-day postoperative outcome between the two surgical approaches of CBT. However, operative time was longer when artery resection was performed. Further research to determine the factors predicting the need for carotid artery resection among patient gender is needed.

Background: Carotid body tumors are uncommon neuroendocrine growths near the carotid bifurcation. While some advocate preoperative embolization to minimize bleeding, others avoid it due to complications. This study shares the experience of a single center in managing patients with carotid body tumors without practicing preoperative embolization. Methods: This was a cross-sectional study of patients with carotid body tumors managed between 2020 and 2024. Data were collected from the hospital's registry. When necessary, routine blood tests, neck ultrasonography, and computed tomography scans were conducted. The tumors were categorized according to Shamblin's classification. The average duration of follow-up was 20 months. Results: The study involved 25 patients, 22 (88%) females and 3 (12%) males. Their ages ranged from 27 to 85 years old. Twenty (80%) cases presented with neck swelling, and six (24%) had a positive medical history. Tumors were mainly on the right side (52%), with 20 (80%) showing ill-defined neck masses. Tumor sizes ranged from 1.5 to 7 cm, with Shamblin type II tumors being discovered in the majority of cases (72%). Types of tumors were significantly associated with the tumor size (p-value < 0.05). Blood transfusion was required in five cases (20%), three from type III and two from type II, with none from type I (p-value = 0.001). Temporary neurological deficits occurred in 3 cases (12%). No functional impairment or mortality was recorded. Conclusions: Carotid body tumors are rare tumors with an unknown etiology. Operation without practicing preoperative embolization may be feasible with an acceptable outcome. [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 tumors (CBTs) are neoplasms that occur at the bifurcation of the carotid artery and are pathologically classified as paragangliomas. In the 4th edition of the WHO classification, paragangliomas are categorized as neoplasms with malignant potential. Clinically, about 5% of CBTs present with malignant features such as metastasis. Currently, it is challenging to distinguish between tumors with benign courses and those that present malignantly. Recent advances in genetic testing have elucidated the genetic characteristics of paragangliomas, including carotid body tumors. Over 20 genes have been identified as being involved in tumor development. Particularly in head and neck paragangliomas, abnormalities in genes related to succinate dehydrogenase are frequently observed. Research is ongoing to understand the mechanisms by which these genes contribute to tumor development. The definitive treatment for CBTs is surgical resection. These tumors are prone to bleeding and often adhere firmly to the carotid artery, making intraoperative bleeding control challenging. The risk of lower cranial nerve paralysis is relatively high, and there is a risk of stroke because of manipulation of the carotid artery. Preoperative evaluation with angiography is essential, and a multi-disciplinary surgical team approach is necessary. In cases where the tumor is difficult to resect or has metastasized, radiation therapy or chemotherapy are employed. Clinical trials involving targeted molecular therapies and radiopharmaceuticals have recently been conducted, with some applied clinically. The development of various new treatments is anticipated, providing hope for therapeutic options in refractory cases. [ABSTRACT FROM AUTHOR]

Background: Carotid body tumors (CBTs) are rare benign tumors that arise from the chemoreceptor tissue located at the carotid bifurcation that require excision if symptomatic. Depending on the size and location of the tumor, the carotid artery may need to be repaired after resection. This study aims to assess whether CBT excision with artery resection had higher rates of 30-day postoperative outcomes compared with CBT excision without artery resection. Methods: This is a retrospective cohort study. Patients were identified in the American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP) registry from 2005 to 2017. They were divided into two groups, based on Current Procedural Terminology (CPT®) codes: group A had CBT excision without carotid artery excision (CPT code 60600) and group B with carotid artery excision (CPT code 60605). Patient demographic characteristics, co-morbidities, and 30-days postoperative outcomes were compared between the two groups. Categorical data were analyzed using Pearson's X2 or Fisher exact tests and presented as proportions (percentages). Continuous data were analyzed using parametric or non-parametric tests as appropriate. Statistical significance was defined as p < 0.05. Statistical analysis was performed using the SPSS statistical software package. Results: A total of 463 patients were identified, 410 (88.4%) in group A and 53 (11.4%) in group B. Overall, there were 291 (62.9%) women. A higher proportion of women underwent CBT excision only, compared to men (91.1% [265/291] vs. 84.3% [145/172], p < 0.0001). Demographics and comorbidities were similar between groups. There was no significant difference in the 30-day postoperative outcomes. The reoperation rate was higher in group B (3.8% vs. 1.5%, p = 0.334), while the readmission rate was higher in group A (3.2% vs. 0% p = 0.269), and both were not significantly different. Overall morbidity and serious morbidity were higher in group B (7.5% vs. 5.9%, p = 0.626) and lower in group A (5.7% vs. 3.9%, p = 0.544), respectively, but were not significantly different. Operative time (mean, SD) was higher in group B (187 ± 107 vs. 138 ± 66 min, p < 0.001). However, the median (IQR) of hospital length of stay (LOS) was similar (2 [1, 4] vs. 2 [1, 3] days, p = 0.134). Conclusions: Overall, no difference was noted in the 30-day postoperative outcome between the two surgical approaches of CBT. However, operative time was longer when artery resection was performed. Further research to determine the factors predicting the need for carotid artery resection among patient gender is needed. [ABSTRACT FROM AUTHOR]

Simultaneously diagnosing cervical sympathetic chain schwannoma, nodular goiter and parathyroid adenoma is a very rare event during clinical practice. We had the opportunity to find this unusual association on a female patient. While nodular goiter and parathyroid adenoma are more common diseases and easier to diagnose, identifying the etiology of a parapharyngeal space tumor remains a challenge and requires multiple imaging studies such as computed tomography scan, magnetic resonance imaging or angiography. A cervical sympathetic chain schwannoma, a carotid body tumor, a paraganglioma or a vagal schwannoma must be taken into account as possible diagnostic variants. Complaints such as Horner`s syndrome, hoarse voice or dysphagia may suggest a nerve originating tumor, but this is a rare situation. Only the surgical exploration is successful in detecting the tumor origin from the cervical sympathetic chain. Even so, the exact origin of the tumor cannot usually be detected without surgical exploration and removal of the piece or biopsies. Therefore, the pathological report of the specimen (adding or not immunohistochemistry tests) is mandatory to be able to confirm the diagnosis of schwannoma.

Background and purpose The classic Shamblin system fails to provide valuable guidance in many Shamblin’s III carotid body tumors (III-CBTs) due to the variable forms of carotid arteries and the complex anatomic relationships in parapharyngeal space. We proposed a modified classification to separately divide III-CBTs into different subgroups on the basis of arterial relevant features and anatomical relevant features. Materials and methods From 2020 to 2023, a total of 129 III-CBTs at a single institution were retrospectively analyzed. All cases were independently classified as arterial-relevant and anatomical-relevant subgroups. The pre-, peri- and postoperative data were summarized and compared accordingly. Results Among the 129 cases, 69 cases were identified as “Classical type”, 23 cases as “Medial type”, 27 cases as “Lateral type” and 10 cases as “Enveloped type” according to arterial morphologies. Besides, 76 cases were identified as “Common type”, 15 cases as “Pharynx- invasion type”, 18 cases as “Skull base-invasion type” and 20 cases as “Mixed type” according to anatomical relationships. “Enveloped type” of tumors in arterial-relevant classification and “Mixed type” of tumors in anatomical-relevant classification are the most challenging cases for surgeons with the lowest resection rate, highest incidence of carotid arteries injury and postoperative stroke. Conclusion The modified classifications provide comprehensive understanding of different III-CBTs which are applicable for individualized treatment in clinical practice. [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]

Introduction. Carotid body paragangliomas are rare, representing 0.6% of head and neck tumors, with average age of presentation in the fifth decade of life. The objective of this article is to describe a clinical case of carotid body paraganglioma in an adolescent. Clinical case. Review and analysis of the clinical case, reviewing its clinical history, study tests, surgical technique used and its corresponding evolution. Information obtained from the clinical record prior authorization by consent of the patient's parents. Results. Adolescent patient with paraganglioma of the right carotid body, 5x3x3 cm, Shamblin III. With complete surgical resection and inverted saphenous vein graft, favorable evolution, with complete permeability of the vascular bridge, without sequelae. Conclusion. This case could correspond to a tumor of familiar etiology, given its early age of presentation. It is necessary to complement the study with images and to objectively determine the associated vascular involvement for surgical planning. In these patients, the complexity of their location and vascular involvement of the tumor requires a multidisciplinary team with head and neck and vascular surgeons for a successful outcome. [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]

Objectives: This study aims to evaluate the role of pretherapy MRI in predicting outcomes in carotid body tumors and propose a grading system for high- and low-risk characteristics. Materials and Methods: A retrospective observational study of 44 patients with 51 lesions was carried out from year 2005 to 2020. MR images were reviewed for characteristics of carotid body tumor, and a score was given that was correlated with intra- and postoperative findings. The various other classifications and our proposed Mahajan classification were compared with Shamblin's classification. The area under the curve and ROC curves were used to present the accuracy of different predictive models. Results: Our scoring system allotted a score of 0 to 15 on the basis of MRI characteristics, with scores calculated for patients in our study ranging from 0 to 13. Lesions with scores of 0-6 were considered low risk (45%), and scores of 7-15 were regarded as high risk for surgery (55%). The Mahajan classification stages tumors into four grades: I (10%), II (20%), IIIa (8%), and IIIb (62%). The frequency of vascular injury was 50% in category I and 64% in category IIIb. The frequency of cranial nerve injury was 50%, 66%, and 27% in categories I, II, and IIIb. Conclusion: The Mahajan classification of CBTs evaluates high-risk factors like the distance of the tumor from the skull base and the angle of contact with ICA, which form the major predictors of neurovascular damage and morbidity associated with its surgery. Though the Shamblin classification of CBT is the most widely accepted classification, our proposed Mahajan classification system provides an imagingbased alternative to prognosticate surgical candidates preoperatively. [ABSTRACT FROM AUTHOR]

Simple Summary: Carotid body tumors (CBTs) are rare tumors and only appear in 1–2 individuals per 100,000. The etiology of CBTs remains unclear; however, SDH mutations and familial inheritance have been reported to be related to CBTs. SDH complexes play crucial roles in aerobic respiration, and SDH mutations in CBTs have been reported to be associated with hypoxia. Hypoxic signaling pathways, specifically hypoxic markers, have attracted more research attention in tumor exploration. However, the existing literature on these signaling and markers lacks a systematic review. Also, therapeutic approaches in CBTs based on hypoxic signaling are rarely used in clinics. In this review, we highlighted the role of hypoxic signaling pathways and markers and their potential implications in the initiation and progression of CBTs. Our findings underscore the involvement of the SDH family, the HIF family, VEGFs, and inflammatory cytokines in tumorigenesis and treatment based on them. Moreover, this review offers valuable insights for future research directions on understanding the relationship between hypoxia and CBTs. Carotid body tumors (CBTs) are rare tumors with a 1–2 incidence per 100,000 individuals. CBTs may initially present without apparent symptoms, and symptoms begin to arise since tumors grow bigger to compress surrounding tissue, such as recurrent laryngeal nerve and esophagus. Also, the etiology of CBTs remains unclear since it is more likely to occur in those who live in high-altitude areas or suffer from chronic hypoxic diseases such as COPD. SDH mutations and familial inheritance have been reported to be related to CBTs. SDH complexes play crucial roles in aerobic respiration, and SDH mutations in CBTs have been reported to be associated with hypoxia. Hypoxic signaling pathways, specifically hypoxic markers, have attracted more research attention in tumor exploration. However, the existing literature on these signaling and markers lacks a systematic review. Also, therapeutic approaches in CBTs based on hypoxic signaling are rarely used in clinics. In this review, we concluded the role of hypoxic signaling and markers and their potential implications in the initiation and progression of CBTs. Our findings underscore the involvement of the SDH family, the HIF family, VEGFs, and inflammatory cytokines (ICs) in tumorigenesis and treatment. Of particular interest is the role played by SDHx, which has recently been linked to oxygen sensing through mutations leading to hereditary CBTs. Among the SDH family, SDHB and SDHD exhibit remarkable characteristics associated with metastasis and multiple tumors. Besides SDH mutations in CBTs, the HIF family also plays crucial roles in CBTs via hypoxic signaling pathways. The HIF family regulates angiogenesis during mammalian development and tumor growth by gene expression in CBTs. HIF1α could induce the transcription of pyruvate dehydrogenase kinase 1 (PDK1) to inhibit pyruvate dehydrogenase kinase (PDH) by inhibiting the TCA cycle. Then, carotid body cells begin to hyperplasia and hypertrophy. At the same time, EPAS1 mutation, an activating mutation, could decrease the degradation of HIF2α and result in Pacak–Zhuang syndrome, which could result in paraganglioma. HIFs can also activate VEGF expression, and VEGFs act on Flk-1 to control the hyperplasia of type I cells and promote neovascularization. ICs also play a pivotal signaling role within the CB, as their expression is induced under hypoxic conditions to stimulate CB hyperplasia, ultimately leading to CBTs detecting hypoxic areas in tumors, and improving the hypoxic condition could enhance photon radiotherapy efficacy. Moreover, this review offers valuable insights for future research directions on understanding the relationship between hypoxic signaling pathways and CBTs. [ABSTRACT FROM AUTHOR]

Carotid body tumors are rare benign tumors that arise in the carotid space of neck typically presenting as soft to firm, painless swelling in the neck. While specific imaging characteristics have been previously described for carotid body tumors, we report a new imaging sign in three cases of carotid body tumors on computed tomography angiography.

Paragangliomas are rare neuroendocrine tumors that are usually benign in nature. They may be either familial or sporadic in their occurrence. Numerous neuroendocrine tumors are collectively included under the umbrella of paragangliomas. Among them, carotid body tumors and glomus jugulare tumors are extremely rare. Thus, we present a rare case of 29-year-old male who was admitted with hearing difficulties and tinnitus in the left ear, with swelling on the left side of the neck. Based on clinical and radiological findings, a diagnosis of left-sided glomus jugulare with carotid body tumor was made. The patient underwent a twostage surgery with an interval of approximately 2 months. Histopathology revealed a paraganglioma. Herein, we present the clinical features, imaging findings, management, and a brief review of literature on the classification, evaluation, and management of carotid body and glomus jugulare tumors. Paraganglioma is a slow-growing tumor. The synchronous occurrence of carotid body and glomus jugulare tumors is infrequent. Microsurgical resection remains the primary treatment modality. Therefore, our patient underwent two-stage surgery. The rarity of occurrence and the proximity and adherence to vital neurovascular structures have resulted in the treatment of paragangliomas remaining a challenge. [ABSTRACT FROM AUTHOR]

Simultaneously diagnosing cervical sympathetic chain schwannoma, nodular goiter and parathyroid adenoma is a very rare event during clinical practice. We had the opportunity to find this unusual association on a female patient. While nodular goiter and parathyroid adenoma are more common diseases and easier to diagnose, identifying the etiology of a parapharyngeal space tumor remains a challenge and requires multiple imaging studies such as computed tomography scan, magnetic resonance imaging or angiography. A cervical sympathetic chain schwannoma, a carotid body tumor, a paraganglioma or a vagal schwannoma must be taken into account as possible diagnostic variants. Complaints such as Horner`s syndrome, hoarse voice or dysphagia may suggest a nerve originating tumor, but this is a rare situation. Only the surgical exploration is successful in detecting the tumor origin from the cervical sympathetic chain. Even so, the exact origin of the tumor cannot usually be detected without surgical exploration and removal of the piece or biopsies. Therefore, the pathological report of the specimen (adding or not immunohistochemistry tests) is mandatory to be able to confirm the diagnosis of schwannoma. [ABSTRACT FROM AUTHOR]

ObjectivesThis study aims to evaluate the role of pretherapy MRI in predicting outcomes in carotid body tumors and propose a grading system for high- and low-risk characteristics.Materials and methodsA retrospective observational study of 44 patients with 51 lesions was carried out from year 2005 to 2020. MR images were reviewed for characteristics of carotid body tumor, and a score was given that was correlated with intra- and postoperative findings. The various other classifications and our proposed Mahajan classification were compared with Shamblin’s classification. The area under the curve and ROC curves were used to present the accuracy of different predictive models.ResultsOur scoring system allotted a score of 0 to 15 on the basis of MRI characteristics, with scores calculated for patients in our study ranging from 0 to 13. Lesions with scores of 0–6 were considered low risk (45%), and scores of 7–15 were regarded as high risk for surgery (55%). The Mahajan classification stages tumors into four grades: I (10%), II (20%), IIIa (8%), and IIIb (62%). The frequency of vascular injury was 50% in category I and 64% in category IIIb. The frequency of cranial nerve injury was 50%, 66%, and 27% in categories I, II, and IIIb.ConclusionThe Mahajan classification of CBTs evaluates high-risk factors like the distance of the tumor from the skull base and the angle of contact with ICA, which form the major predictors of neurovascular damage and morbidity associated with its surgery. Though the Shamblin classification of CBT is the most widely accepted classification, our proposed Mahajan classification system provides an imaging-based alternative to prognosticate surgical candidates preoperatively.

Carotid body tumors are rare benign tumors that arise in the carotid space of neck typically presenting as soft to firm, painless swelling in the neck. While specific imaging characteristics have been previously described for carotid body tumors, we report a new imaging sign in three cases of carotid body tumors on computed tomography angiography. [ABSTRACT FROM AUTHOR]

Giant carotid body tumors, defined as those >8 cm in size, are extremely rare. Definitive surgical management is a complex undertaking because these large tumors tend to have grown to envelop cranial nerves and the carotid artery, and few data exist regarding the long-term outcomes for these patients. We present the case of a patient with bilateral giant carotid body tumors who underwent staged embolization and excision of a >10-cm carotid body tumor. After 5 years of follow-up, we demonstrated that elective open repair can provide long-term symptomatic relief. We describe and illustrate the crucial steps and considerations regarding the excision of complex Shamblin type III carotid body tumors.

Paragangliomas are rare neoplasms that originate from chemoreceptors and can be found in different locations. Their growth rate is slow, and they are usually benign tumors. The surgical approach may vary according to the location of the tumor. In this study, we report the case of a 56-year-old patient who underwent paraganglioma excision. The tumor was in close proximity to the aortic arch and main vascular origins and also extended into the retrosternal space. Despite the incision matching the carotid bifurcation tracing, the tumor could not be completely reached. Therefore, the tumor could be excised by performing a partial sternotomy. We wanted to share the case and surgery due to the rarity of a paraganglioma of this size, its atypical location, and the fact that it requires an extra anatomical incision. [ABSTRACT FROM AUTHOR]

Carotid body tumors (CBTs) are uncommon benign head and neck neoplasms. Surgical resection is the treatment of choice for CBTs. However, the anatomical structures adjacent to the tumor tissue may encounter serious injuries during the surgery. Shamblin grading system is a surgical intra-operative scoring system to determine the risks associated with the surgery. Therefore, we aimed to evaluate the correlation of pre-surgical imaging parameters with Shamblin grades and intra-operative complications. In this cross sectional study, we enrolled 36 patients with CBTs. Preoperative cervical CT angiography was acquired in each participant and following parameters were reported in each case: Tumor volume, tumor distance to the base of the skull (TDBS), tumor contact with the internal carotid artery (ICA), and external carotid artery (ECA) and tumor density. Finally, we assessed the relation of pre-surgical imaging parameters with Shamblin grades, and intra-operative complications. Only tumor volume was significantly correlated with Shamblin grades (P < 0.05). The tumor contact with ECA was marginally correlated with Shamblin grades (P = 0.103); however, other imaging parameters were not significantly correlating with Shamblin grades. There was a statistically significant correlation between ICA contact and tumor volume with ECA injury. In addition, the tumor density significantly correlated with cranial nerves injury. The results of STATA analysis were indicative for 69.44% accordance between radiologic typing and Shamblin grading system. We found that tumor volume correlates significantly with Shamblin grading system, and there is significant correlation between tumor ICA contact, and tumor density and intra-operative complications. [ABSTRACT FROM AUTHOR]

Currently, there is no consensus on estimating the malignant potential of Carotid Body Tumor (CBT) and the only way to predict a metastatic CBT is through DOTANOC Positron Emission Tomography (PET) scan. There is a well-established correlation between CBT and superoxide anions inside tumor cells. The purpose of this pilot study was to measure superoxide anions inside CBT cells and find if these can be used as marker to predict malignant potential of CBT. The results were also co-related with findings of DOTANOC PET scan retrospectively. The CBT tissue from 10 patients was stained using a fluorogenic dye and superoxide anions were measured by analysis of fluorescent image. The patients were divided into two groups – First group with four patients having potentially malignant CBT based upon clinico-surgical characteristics and second group with the rest of the six patients. It was seen that the superoxide anions were highest in the first group which included patients with metastatic carotid body tumor, patients with multiple paragangliomas and patient with positive family history (p = 0.011). The same patients also had metastasis and multiple tumors detected on DOTANOC PET scan. It was concluded that measuring superoxide anions in excised tumor tissue can be used to estimate malignant potential of CBT and can identify patients who truly require DOTANOC PET scan; without affecting the treatment, as it is an expensive investigation involving ionizing radiation and may not be available in all centres. [ABSTRACT FROM AUTHOR]

Background: To investigate the impact of preoperative embolization (p-TAE) on CBT surgical resection and explore the optimal tumor volume for p-TAE of CBT resection. Methods: This retrospective study reviewed 139 surgically excised CBTs. According to Shamblin classification, tumor volumes, and whether to carry out the p-TAE, the patients were classified into different groups. The demographic, clinical features, and the intraoperative and post-operative information about the patients were retrieved and analyzed from the patient records. Results: A total of 139 CBTs was excised in 130 patients. According to the results of subgroup analysis, there were no significant differences in surgical time, blood loss, adverse events (AEs), and the revascularization when compared with non-embolization group (NEG) for type I, II, III, respectively (all p > 0.05) except for the surgical time in type I (p < 0.05). Then the X-tile program was employed and determine the cutoff point (tumor volume = 6670 mm3) for tumor volumes and blood loss. The average tumor volume was (29,782.37 vs. 31,345.10 mm3, p = 0.65) for embolization group (EG) and NEG. The mean surgical time (208.86 vs. 264.67 min, p > 0.05) and intraoperative blood loss (252.78 vs. 430.00 mL, p < 0.05) were less, and the incidence of revascularization required (35.56 vs. 52.38%, p > 0.05) and total complications (27.78 vs. 57.14%, p < 0.05) were lower in EG when compared with NEG (tumor volume ≥ 6670 mm3). However, the results were not statistically significant when the tumor size was less than 6670 mm3. No surgery-related mortality was observed during the follow-up. Conclusions: Preoperative selective embolization of CBT is an effective and safe adjunct for surgical resection, especially for Shamblin class II and III tumors (≥ 6670 mm3). [ABSTRACT FROM AUTHOR]

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Abstract Background Carotid body tumors (CBTs) are rare neuroendocrine neoplasms arising near the carotid bifurcation with a reported incidence of 1 to 2 cases in 100,000 patients. Most CBTs are sporadic, benign, slow-growing, and non-secreting, but untreated CBTs can grow locally to compress the nearby blood vessels, esophagus, and airway. Regional metastases can occur in 5% to 10% of cases, but distant metastases are exceedingly rare, occurring in roughly 1–2% of cases. As such, the optimal treatment for metastatic CBTs is not well-defined. We report a rare case of a patient with CBT distant metastases causing spinal cord compression. Case presentation A 40-year-old African American female presented with a right neck mass, headaches, vertigo, tinnitus, hoarseness, and dysphagia. Imaging demonstrated a Shamblin II right neck mass; subsequent transcervical resection and pathology showed a carotid body paraganglioma. The patient recurred locally near the carotid bifurcation, so she underwent Stereotactic Body Radiation Therapy to the recurrent right neck disease. She later re-presented with new onset bilateral lower extremity weakness, dysmetria, and numbness. She was found to have metastatic disease to the thoracic spine causing spinal cord compression. She underwent laminectomy, tumor resection, and posterior fixation followed by adjuvant radiation therapy. She was started on systemic therapy with sunitinib. She eventually progressed with metastatic disease to the right iliac bone, which was treated with palliative radiotherapy. Second line systemic therapy with capecitabine and temozolomide was started. At last follow up, the patient was asymptomatic with stable persistent disease. Conclusions Paragangliomas often exhibit a prolonged interval to the development of progression; locoregional recurrences or rare distant metastases have been reported to occur as many as 20 years from diagnosis. The natural course of CBTs in other cases as well as the present case call into question the idea that CBTs are truly benign; instead CBTs may be indolent tumors with metastatic potential. Treatment choices for CBTs include surgical resection, radiation therapy, and systemic therapy, though the optimal treatment regimen for metastatic CBTs is not well-defined. A more advanced understanding of CBT pathophysiology, disease classification, risk stratification, and treatment options is needed to improve outcomes for patients.

Objective: To investigate the value of CT spectral imaging in the study of carotid body tumors (CBT). Methods: Thirty patients with CBT who underwent CT energy spectrum examination and were confirmed by operation were included. Subsequently, 60 keV and 40 keV single energy images were reconstructed through the GSI viewer. The CT value, background noise (SD), contrast noise ratio (CNR), signal-to-noise ratio (SNR), and subjective score of CBT feeding arteries of the 40 keV, 60 keV single energy images and 120 kVp like images were statistically analyzed to evaluate the relationship between CBT energy spectrum parameters and imaging features with surgical results. Results: There was no correlation between energy spectrum parameters in the arterial and venous phases of CBT and the surgical results. However, CBT transverse diameter, longitudinal diameter, and Shamblin classification were strongly correlated with intraoperative bleeding, and the number of feeding arteries was moderately correlated with intraoperative bleeding. Additionally, the transverse diameter, longitudinal diameter, and Shamblin classification were moderately correlated with cranial nerve injury, and the number of feeding arteries was strongly correlated with cranial nerve injury. We also found that 40 keV was the best energy level for the CBT feeding artery display. Furthermore, the CT value, SD, CNR, and SNR of the 60 keV group were significantly higher than those of the 60 keV and 120 kVp like groups, and the CT value and noise of the 60 keV group were significantly higher than those of the 120 kVp like group. However, there was no significant difference in CNR and SNR between the two groups. The subjective evaluation score of the 40 keV group was the highest, and the subjective evaluation of the two radiologists had good consistency. Conclusion: (1) There was no correlation between the energy spectrum parameters in the arterial and venous phases of CBT and GAPP score. (2) The number of CBT feeding arteries is one of the important parameters for evaluating surgical complications and 40 keV single energy imaging can significantly optimize the display of CBT feeding arteries.

Introduction: Carotid body tumors (CBTs), also known as paragangliomas or chemodectomas, are rare neuroendocrine neoplasms that arise near the carotid bifurcation within glomus cells derived from the embryonic neural crest. The reported incidence of CBTs is 1–2 cases per 1 lakh cases. Surgery is the mainstay of treatment. Objectives: The objective of this study was to report our experience and describe the presentation, surgical approach, postoperative complications, and outcomes of surgical treatment. Materials and Methods: It is a single-institution experience where a retrospective analysis of 6 cases with CBTs of patients who were candidates for surgery from June 2018 to June 2021 with a mean follow-up period of 24 months was done. Results: The mean age was 37 years, with a female predominance of 66%. The mean size of the tumor was 4.4 ± 1 cm. Four (66%) cases were presented with pain and pulsating neck swelling, and 2 (33%) cases were incidentally detected. All the cases were unilateral tumors. Neck ultrasound was done in all cases as a primary investigation. Magnetic resonance imaging was performed in 5 (87.5%) cases, which revealed tumors having near circumferentially encasing the internal carotid artery (ICA). No cases were malignant. All cases were surgically approached through transcervical vertical incision. Four cases (66%) and 2 (33%) cases were classified as Shamblin's type II and III, respectively. Two (33%) cases needed special maneuvers such as division of the posterior belly of digastric and nasal intubation for the extra length of ICA. No major complications occurred. One patient (16.6%), as the tumor could not be dissected from the adventitia of ICA so, had to resect it, and primary end-to-end anastomosis was done. External carotid artery ligation was performed in 1 (16.6%) case. Minor postoperative complications such as hoarseness of voice occurred in 2 (33%) patients. No operative mortality occurred. Conclusion: We found a predominance of women and pain as the most common presenting complaint. Resection of CBTs by surgeons with experience in vascular reconstruction is recommended. Early surgical management is recommended to avoid neurological deficit or vascular reconstruction due to a Shamblin's class III tumor. Preferred approach was caudo–cranial during dissection. Cranial nerve injury continues to be the most common complication.

Abstract Background Arising from chemoreceptor cells, carotid body tumors (CBTs) are rare neoplasms associated with hemodynamics. Perioperative changes in blood pressure (BP) and heart rate (HR) are not completely understood. Methods This retrospective, observational, controlled study included all CBT patients from 2013 to 2018 in Peking Union Medical College Hospital. Perioperative changes in BP/HR within or between unilateral/bilateral/control groups were investigated. Perioperative details across Shamblin types were also assessed. Results This study included 108 patients (116 excised CBTs). The postoperative systolic BP and HR increased in both unilateral (mean difference of systolic BP = 5.9mmHg, 95% CI 3.1 ~ 8.6; mean difference of HR = 3.7 bpm, 95% CI 2.6 ~ 4.9) and bilateral (mean difference of systolic BP = 10.3mmHg, 95% CI 0.6 ~ 19.9; mean difference of HR = 8.4 bpm, 95% CI 0.5 ~ 16.2) CBT patients compared with the preoperative measures. Compared with control group, the postoperative systolic BP increased (difference in the alteration = 6.3mmHg, 95% CI 3.5 ~ 9.0) in unilateral CBT patients; both systolic BP (difference in the alteration = 9.2mmHg, 95% CI 1.1 ~ 17.3) and HR (difference in the alteration = 5.3 bpm, 95% CI 1.0 ~ 9.6) increased in bilateral CBT patients. More CBT patients required extra antihypertensive therapy after surgery than controls (OR = 2.5, 95% CI 1.14 ~ 5.5). Maximum tumor diameter, intraoperative vascular injury, continuous vasoactive agent requirement, total fluid volume, transfusion, estimated blood loss, operation duration, postoperative pathology, overall complications, and intensive care unit/hospital lengths of stay significantly varied among Shamblin types. Conclusion CBT excision may be associated with subtle perioperative hemodynamic changes. Perioperative management of CBT patients necessitates careful assessment, full preparation and close postoperative monitoring.

Background: The gold standard for patients with carotid body tumors (CBT) is surgical resection; nevertheless, some patients are unfit for surgery or, for other reasons, could not be operated on. Active surveillance has been known to be a reasonable strategy for these cases. This study aimed to evaluate tumor growth in unoperated patients with CBTs. Methods: A retrospective review of all unoperated patients with CBT from a single academic hospital diagnosed between 2014 and 2021 was performed. Results of nonparametric testing were presented using the median and ranges for Mann-Whitney-U or Kruskal-Wallis. Significance was defined as a 2-tailed P <.05. Results: The cohort included a total of 31 patients, with a median age of 60 years (range: 37-80 years), of which 27 (87.1%) were females. The patients live at a median altitude of 2800 meters (range: 2756-2980 meters) above sea level. Twenty (64.5%) patients had Shamblin I tumors, eight (25.8%) patients had Shamblin II tumors, and three (9.7%) patients had Shamblin III tumors. Median CBT volume at diagnosis was 14.1 cm3 (range:.9 - 213.3 cm3). Median volume at diagnosis of symptomatic tumors was substantially larger than asymptomatic tumors, 49.2 cm3 vs 7.9 cm3, respectively (P =.03). Median growth of the tumors during a median 15-month follow-up (range: 3-43 months) was 3.3 cm3 (range: 0-199.9 cm3). Overall, 77% (n = 24) of the CBTs grew at least 1 cm3. Conclusion: Most patients in the present study had tumor growth by at least 1 cm3, with a median tumor growth of 3.3 cm.3 In the present study tumor growth was shown to be greater than other low altitude CBT active surveillance studies; therefore, surgical resection should be recommended in patients with CBT living at high altitudes. [ABSTRACT FROM AUTHOR]

Thyroid nodule is the most common presentation of thyroid carcinoma. Considering the indolent course of the disease, indeed, the papillary thyroid cancer (PTC) microcarcinoma puts much debate regarding large-scale cohorts on patient follow-up over several decades to detect differences in aggressiveness and outcome. Lymph node metastases might be the salient manifestation of the disease. The nodal metastases usually appear in the central and to a lesser degree in the lateral neck mostly as a solid nodule and rarely in form of a cystic mass. In this study, we describe a case of lymph node metastasis from thyroid papillary carcinoma that clinically and radiologically mimicked a carotid body tumor and despite all pre-surgical evaluations; papillary thyroid carcinoma was detected in the final histopathology report. We intend to describe the initial clinical evaluation, radiological survey and pathology report. [ABSTRACT FROM AUTHOR]

Carotid body tumors are slow growing neck masses that arise from the neural crest cells at the carotid bifurcation. Majority are asymptomatic and are diagnosed incidentally. Surgical excision is accepted as the treatment of choice to reduce complications. In the present series, we report 10 cases of carotid body tumors and our institutional experience. All patients underwent radiological evaluation with an ultrasonography with Doppler, contrast enhanced computed tomography and MR angiography. 6 cases were operated by a transcervical excision. The tumor was excised in tototranscervically. One of the cases required saphenous vein graft intraoperatively due to vascular injury and also had postoperative vocal cord palsy. The rest had an uneventful recovery. Carotid body tumors although rare and seemingly indolent can cause substantial symptoms if left untreated. A prompt multi modality approach is needed for both diagnosis and treatment to avoid major complications. [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]

There is no definitive consensus on the impact of preoperative embolization on carotid body tumor (CBT) treatment. The objective of this study was to compare surgical outcomes of patients who underwent preoperative embolization before CBT resection vs patients who underwent resection alone. The CAPACITY registry included 1432 patients with CBT from 11 medical centers in four different countries. The group of patients undergoing CBT resection with preoperative embolization was matched in a 1:6 ratio from a pool of patients from the CAPACITY database, using a generated propensity score with patients who did not underwent preoperative embolization. A total of 553 patients were included for analysis. Mean patient age was 56.23 ± 12.22 years. Patients were mostly female (n = 469; 84.8%). Bilateral CBT was registered in 60 patients (10.8%). Seventy-nine patients (14.3%) underwent preoperative embolization. Embolized patients had larger CBT sizes than non-embolized patients (33.8 mm vs 18.4 mm; P =.0001). Operative blood loss was lower in the embolized group compared with the non-embolized group (200 mL vs 250 mL; P =.031). Hematomas were more frequent in the non-embolized group (0% vs 2.7%; P =.044). Operative time, rates of stroke, cranial nerve injuries, and death were not statistically significant between groups. Embolization before CBT resection was associated with significantly lower blood loss and lower neck hematomas than patients who underwent resection alone. Operative time, stroke, cranial nerve injuries, and death were similar between groups. [ABSTRACT FROM AUTHOR]

In the present case, a 41-year-old male patient had reported a large swelling on the right side of his neck that had been radiographically diagnosed as a carotid body tumor. The tumor extended toward the right thyroid lobe, deep to the parapharyngeal space and cranially to the base of the skull. The clinical and medical imaging findings confirmed the diagnosis. Because of the large size of the tumor (50 mm × 48 mm × 85 mm), extent of involvement, and hypervascularity, surgical excision by midline mandibulotomy was chosen as the treatment approach. Midline mandibulotomy is a very good approach for large tumors with extensive involvement of the surrounding tissues, especially tumors with great medial and cephalic extension.