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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.

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A 47-year-old male presented with a right-sided Shamblin type 2 carotid body tumor measuring 5*5 cm. After preoperative embolization, a sub adventitial resection of the tumor was done. He was discharged after postoperative day 5 and presented again to emergency 10 days later with a bleeding pseudoaneurysm at the surgical site causing dysphagia and dyspnea. He was taken for emergency exploration of the surgical wound and, intraoperatively, it was observed that the proximal ends of the internal carotid artery and external carotid artery close to the bifurcation were forming a pseudoaneurysm, 1 cm distal to the common carotid artery. The external carotid artery was ligated and a common carotid to internal carotid artery bypass was done with a reversed saphenous vein graft. He recovered well in the postoperative period and was discharged on day 7. Pseudoaneurysm formation following carotid body tumor resection is extremely rare and has only been reported thrice in the literature.

Um homem de 47 anos apresentou tumor carotídeo Shamblin tipo 2 no lado direito, medindo 5 x 5 cm. Após embolização pré-operatória, foi realizada ressecção subadventicial do tumor. O paciente teve alta no quinto dia pós-operatório e voltou à emergência 10 dias depois, com pseudoaneurisma hemorrágico no sítio operatório causando disfagia e dispneia. Foi levado para exploração emergencial da ferida cirúrgica, e, no intraoperatório, 1 cm distalmente à artéria carótida comum, as extremidades proximais da artéria carótida interna e da artéria carótida externa próximas à bifurcação formavam um pseudoaneurisma. A artéria carótida externa foi ligada, e foi realizada uma ponte de safena de carótida comum para a artéria carótida interna com a veia safena invertida. O paciente se recuperou bem no pós-operatório e recebeu alta no sétimo dia. A formação de pseudoaneurisma após ressecção de tumor do corpo carotídeo é extremamente rara, tendo sido relatada apenas três vezes na literatura.

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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.

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Objective:To introduce the surgical experience of carotid body tumor（CBT） resection with preservation of internal carotid artery. Methods:The clinical data of 109 patients with CBT were retrospectively analyzed. The key points of surgical techniques were summarized, the imaging and pathological results were comprehensively analyzed, and the postoperative complications were observed. Results:Of the 109 patients, 28 were Shamblin Ⅰ, 46 were Shamblin Ⅱ, and 35 were Shamblin Ⅲ. Synaptophysin（SYN） and soluble protein-100（S-100） were positive in all cases. There was a positive correlation between the average expression area percentage of S-100 and SYN in pathological tissue of 17 patients（r=0.48）, and the difference was statistically significant（P<0.05）. The average operation time was（148.4±46.2） minutes, the average intraoperative blood loss was（64.7±22.8） mL, and the average hospital stay was（15.2±2.6） days. Three patients underwent tumor resection combined with external carotid artery ligation, 1 patient underwent tumor resection combined with internal carotid artery ligation, and the remaining patients underwent tumor resection alone. The overall rate of intraoperative vascular ligation was 3.7% and the rate of nerve injury was 6.4%. According to preoperative CTA, intraoperative situation and postoperative pathological results, a new classification of CBT was proposed, which could intuitively reflect the gap between the tumor and the carotid artery and the nature of the tumor. Conclusion:Surgical resection of CBT is recommended after diagnosis. The potential gap between the tumor and the blood vessels was found under the microscope. Low energy bipolar electrocoagulation was used to coagulate and cut off the fibrous connective tissue between the tumor and gradually separated along the adventitia of the artery. The carotid artery could be preserved in most cases while the tumor was completely removed, and the amount of intraoperative bleeding and the incidence of complications were reduced. It is particularly important to identify the difficult cases before operation.

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Protection of cranial nerves is one of the major challenges in the resection of paragangliomas of head and neck, especially in complex paragangliomas. We report a case of bilateral jugular tumor with unilateral carotid body tumor. Baroreflex failure syndrome（BFS） occurred after staged resection of bilateral lesions. There is still a lack of effective treatment for this complication. More prudent and reasonable treatment strategy is important to reduce the incidence of BFS.

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INTRODUCTION AND IMPORTANCE: This case study presents the clinical details of a 37-year-old woman who presented with an asymptomatic swelling in the right supraclavicular region, ultimately diagnosed as a carotid body tumor (CBT). CASE PRESENTATION: The patient's medical background, which included her brother's CBT, prompted further investigation. Upon clinical examination and imaging studies, a tissue mass surrounding the right internal carotid artery was identified. Subsequent surgical resection of the tumor was performed, and histological analysis confirmed a neoplastic proliferation consistent with paraganglioma. CLINICAL DISCUSSION: This case highlights the significance of taking into account familial history and conducting comprehensive diagnostic assessments for patients exhibiting similar symptoms. The successful multidisciplinary management of this rare tumor underscores the importance of early detection and appropriate therapeutic interventions. CONCLUSION: This report offers valuable insights into the clinical presentation, diagnostic process, and treatment of CBTs, emphasizing the necessity for a comprehensive approach to managing this uncommon neoplasm.

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Carotid body paraganglioma is a slow growing tumor of head and neck region. It can rarely be malignant in nature which is characterized by distant metastases on anatomical imaging. We share an interesting presentation of a malignant carotid body on F-18 FDG PET/CT in form of liver and skeletal metastases.

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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.

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OBJECTIVE: Carotid body tumours (CBTs) and baroreceptor failure (BRF) are two distinct but interrelated conditions, affecting the carotid body and its regulatory mechanisms. We aim to describe and quantify BRF after unilateral and bilateral CBT resections. METHODS: Prospective cohort study. We included all patients with unilateral or bilateral CBT undergoing resection from April 2021 to January 2023. Demographics and CBTs characteristics were analysed; baroreceptor sensitivity assessment was conducted using the Composite Autonomic Severity Score (CASS). Statistical analyses were performed using R. Significance level was set at a 2-tailed α = 0.05. RESULTS: A total of 30 patients with CBT underwent surgical resection, twenty-three were included in the study (18 unilateral and 5 bilateral CBTs). All 23 (100 %) were females, median age of 60 years. Regarding patients with unilateral CBT; preoperatively, 13 had BRF, the most common dysfunction subtype was mixed. Postoperatively, the most common dysfunction subtype was sympathetic failure. With regards to bilateral CBTs; 2 patients did not have autonomic dysfunction preoperatively. After bilateral surgical resection one patient remained without autonomic dysfunction; however, all other patients persisted with BRF. CONCLUSION: BRF was present in 13 patients with unilateral CBT and 3 patients with bilateral tumours preoperatively; most will remain with BRF and will only change the characteristics postoperatively. No associations were found between type, severity of BRF and Shamblin classification or laterality. It is paramount that research in this area continues as many features are yet unknown regarding CBT pathogenesis, hence, BRF may be present yet not affect significantly quality of life.

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Carotid space lesions present diagnostic challenges due to their diverse etiology and varied clinical manifestations. This article critically reviews the anatomy of the carotid space and highlights the spectrum of pathologies within this complex region, illustrated by three case studies. The cases were examined with ultrasonography (USG), computed tomography (CT), and magnetic resonance imaging (MRI). Schwannomas appeared heterogeneously hypodense on plain CT and partially hyperdense on contrast-enhanced CT (CECT), with displacement of adjacent vessels. Vagal-origin schwannomas caused the anteromedial displacement of the internal carotid artery. Paragangliomas were typically homogeneously hyperdense on CECT, with lateral displacement of the internal carotid artery when of carotid body origin. The management of carotid space lesions depends on the resectability of the tumors; unresectable tumors are managed with chemotherapy. This overview enhances clinical understanding and diagnostic accuracy, facilitating improved patient outcomes in managing carotid space lesions.

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Carotid body tumors are rarely encountered pathologies in the pediatric age group but still exist in the differential diagnosis of cervical painless masses. Genetic and familial background should be studied in addition to contrast imaging studies. Complete subadventitial resection, with or without prior embolization, which is still controversial, is the mainstay of therapy. Follow-up gains importance due to its nature. Herein, we report a nine-yearold girl presented with a unilateral asymptomatic mass on the neck with a diagnosis of carotid body tumor treated surgically without embolization. We emphasize the specific aspects of the pediatric age group.

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BACKGROUND: Lateral neck mass management frequently challenges surgeons. Nerve tissue neoplasms are an uncommon cause of such nodules. Neurogenic tumors form a tiny percentage of the head and neck neoplastic lesions. Considering the number of nerves in this area, it is surprising that such neoplasms are not more frequently seen. METHODS: A retrospective study was conducted on all patients who presented to the National Cancer Institute of Cairo, Egypt, with head and neck neurogenic neoplasms. RESULTS: During the last 10 years at the National Cancer Institute of Egypt (2006-2015), 40 cases of neurogenic tumors of the head and neck were treated at the head and neck unit. Patients' ages ranged from two to 78 years with a mean age of 34.7 years. Childhood neurogenic tumors accounted for nine cases (22.5%) only in this study. Male patients diagnosed with these tumors comprised 16 cases, while female patients comprised 24 cases, with a female-to-male ratio of 1.5:1. Patient presentation depends on the biological behavior of the tumor; for instance, some of them present by slowly growing painless well-circumscribed mobile swelling, and others present by rapidly growing swelling with neurological deficit. Clinical picture and imaging studies such as CT and MRI raise suspicion and may help delineate such tumors, but a definitive diagnosis is obtained by tissue biopsy. Surgery is the mainstay of treatment in most head and neck neurogenic tumors, whereas adjuvant therapy is of limited benefit in some types of neurogenic tumors. The five-year survival rate was 60% for the malignant group, while death was reported in six out of 15 cases (40%).  Conclusion: Most neurogenic head and neck tumors are benign. Accurate preoperative assessment and a high degree of suspicion are the initial steps in the management. Proper treatment involves complete surgical excision; however, debulking procedures have an important role. Malignant neurogenic tumors are aggressive and are treated with combined radical surgical resection and radiation. Chemotherapy is tried for locally advanced unresectable or metastatic disease.

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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.

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OBJECTIVE: Although carotid body tumors (CBTs) are rare, they attract particular attention because of their propensity for malignant transformation and the high surgical risk. Because data are scarce and as it is difficult to achieve a large sample size, no study has yet comprehensively analyzed the characteristics, management, or operative complications of CBTs. Therefore, we collected and analyzed all currently available information on CBTs and used the pooled data to derive quantitative information on disease characteristics and management. METHODS: We systematically searched PubMed, Embase, the Cochrane Library, and the Web of Science up to December 1, 2022, for studies that investigated the characteristics and management of CBTs. The primary objective was to identify the prevalence of the various characteristics and the incidence of complications. The secondary objective was to compare patients who underwent preoperative embolization (PE) and those who did not (non-PE), as well as to compare patients with different Shamblin grades and those with and without succinate dehydrogenase (SDH) mutations in terms of CBT characteristics and complications. Two reviewers selected studies for inclusion and independently extracted data. All statistical analyses were performed using the standard statistical procedures of Review Manager 5.2 and Stata 12.0. RESULTS: A total of 155 studies with 9291 patients and 9862 tumors were identified. The pooled results indicated that the median age of patients with CBT was 45.72 years, and 65% were female. The proportion of patients with bilateral lesions was 13%. In addition, 16% of patients had relevant family histories, and the proportion of those with SDH gene mutations was 36%. Sixteen percent of patients experienced multiple paragangliomas, and 12% of CBTs had catecholamine function. The incidence of cranial nerve injury (CNI) was 27%, and 14% of patients suffered from permanent CNI. The incidence rates of operative mortality and stroke were both 1%, and 4% of patients developed transient ischemic attacks. Of all CBTs, 6% were malignant or associated with metastases or recurrences. The most common metastatic locations were the lymph nodes (3%) and bone (3%), followed by the lungs (2%). Compared with non-PE, PE reduced the estimated blood loss (standardized mean difference, -0.95; 95% confidence interval [CI], -1.70 to -0.20) and the operation time (standardized mean difference, -0.56; 95% CI, -1.03 to -0.09), but it increased the incidence of stroke (odds ratio, 2.44; 95% CI, 1.04-5.73). Higher Shamblin grade tumors were associated with more operative complications. Patients who were SDH gene mutation-positive were more likely to have a relevant family history and had more symptoms. CONCLUSIONS: CBT was most common in middle-aged females, and early surgical resection was feasible; there was a low incidence of serious operative complications. Routine PE is not recommended because this may increase the incidence of stroke, although PE somewhat reduced the estimated blood loss and operation time. Higher Shamblin grade tumors increased the incidence of operative complications. Patients who were SDH gene mutation-positive had the most relevant family histories and symptoms.

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INTRODUCTION: Carotid body tumor (CBT), a neuroendocrine neoplasm, and benign multinodular goiter (BMNG) are distinct pathologies affecting the neck region. Although rare, they can occur concurrently. This case contributes to the limited evidence regarding the association between these distinct pathologies and their operative management. CASE PRESENTATION: The patient was a 45-year-old female with a palpable mass on the right side of her neck. She was diagnosed with Shamblin type III non-secretory CBT alongside BMNG. The surgical intervention included resection of the CBT, carotid artery bypass, and Dunhill thyroidectomy. DISCUSSION: This case is the third reported instance of coexisting CBT and BMNG. Their causative relationship is evident in the literature without a clear explanation of the underlying mechanism. Both conditions are treated surgically. Dunhill thyroidectomy for BMNG is a safer option, offering more flexibility and advantages over other thyroidectomies. CONCLUSION: This case highlights the complexity of managing such dual pathologies and may provide further evidence of their association.

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The carotid body, situated at the common carotid artery bifurcation, comprises specialized glomus cells responsible for sensing blood oxygen, carbon dioxide, pH, and temperature changes, crucial for regulating breathing and maintaining oxygen homeostasis. Carotid body tumors (CBTs), arising from these cells, are rare, representing only 0.5% of head and neck tumors, often presenting as benign, slow-growing, vascularized masses. In February 2023, this bibliometric analysis was conducted, which involved screening 1733 articles from the Web of Science database. The screening process was based on citation count, and articles were selected for inclusion based on specific criteria that focused on CBTs located within the carotid bifurcation. Rigorous selection involved independent screening and data extraction by four authors. The top 100 articles, published between 1948 and 2019, totaled 6623 citations and were authored by 98 unique first authors from 22 countries and 77 institutions, spanning 42 journals. Treatment articles were the predominant category, comprising 49% of the literature. This analysis offers insights into publication trends, identifies literature gaps, and outlines areas of research focus, providing a valuable resource to guide future studies on CBTs.

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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.

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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.

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Introducción. Los paragangliomas del cuerpo carotídeo son neoplasias infrecuentes y representan el 0,6 % de los tumores de cabeza y cuello. La edad de presentación promedio es la quinta década de vida. El objetivo de este artículo fue describir un caso de paraganglioma del cuerpo carotídeo en una adolescente. Caso clínico. Se presenta el caso de una paciente adolescente con paraganglioma de cuerpo carotídeo derecho, de 5x3x3 cm, Shamblin III. Se analizaron la historia clínica, los exámenes diagnósticos, la técnica quirúrgica utilizada y su evolución correspondiente. La información fue obtenida a partir de la ficha clínica, previa autorización por consentimiento de los padres de la paciente. Resultados. Se hizo resección quirúrgica completa y reparo vascular con injerto de vena safena invertida, con evolución favorable y permeabilidad completa del puente vascular, sin secuelas. Conclusión. Este caso podía corresponder a un tumor de etiología familiar, dada su edad temprana de presentación. Se hizo necesario complementar su estudio con imágenes y objetivar el compromiso vascular asociado para la planificación quirúrgica. En estos pacientes, la complejidad de su localización y el compromiso vascular del tumor requiere de un equipo multidisciplinario, con cirujanos de cabeza y cuello y cirujanos vasculares para un resultado exitoso.

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.

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The carotid body tumor (CBT) is a rare paraganglioma neoplasm that often occurs in the head and neck anatomical region. Carotid angiography continues to be widely regarded as the preferred diagnostic method for this particular malignancy. Surgical intervention has been widely acknowledged as the primary approach for managing CBTs. However, the resection of CBTs poses significant technical challenges due to its specific anatomical position. To mitigate the incidence of intraoperative and postoperative challenges, we have conducted a comprehensive review of both domestic and international literature to consolidate the surgical approach and strategies for preventing and managing complications associated with this particular tumor.