Two stage, hybrid endovascular and open surgical approach to treat difficult carotid body tumors.

OBJECTIVE: Present the feasibility, applicability, clinical effectiveness, and results of complicated Shamblin II or III carotid body tumors treated with a two-stage hybrid surgical approach. MATERIALS AND METHODS: Retrospective, observational, cross-sectional, descriptive study of the successful treatment of 16 cases of difficult Shamblin II or III carotid body tumors, consisting of a two-stage surgical approach. We conducted a retrospective, observational, cross-sectional, descriptive study of a series of patients with complicated Shamblin II or III carotid body tumors, which we treated with a two-stage hybrid surgical procedure, in which we first placed a carotid endoprosthesis and 45 days later performed surgical resection of the tumor, following our originally published technique. This study was conducted from February, 2007 to November, 2019, in a third level care centre. RESULTS: We treated 16 patients with a mean age of 50.5 years. All resided at more than 2000 meters above sea level. In all 16 a complete resection was performed. The average duration of surgery was 103.9 min, the average intraoperative bleeding was 69 ml. There were three cases of neuropraxia. The ansa cervicalis nerve had to be sectioned in three cases and there was permanent upper laryngeal nerve injury in two cases. There were no permanent cerebrovascular injuries from placement of the endoprostheses. One patient developed transient cerebral ischaemia (TIA) with no long-term sequelae. There were two cases of asymptomatic late occlusion of the endoprostheses. The average initial volume of the tumors was 54.4 cc. The average tumor volume 35 days after implant of the endoprosthesis was 30.9 cc. SYMPTOMS: Presence of tumors in the neck in all cases and two cases of dysphagia. CONCLUSION: This two-stage hybrid technique allowed for the complete resection of difficult Shamblin II or III carotid body tumors, with one case of TIA and two with permanent upper laryngeal nerve injuries and without mortality.

Clinical Characteristics and Surgical Outcomes of Carotid Body Tumors: Data from the Carotid Paraganglioma Cooperative International Registry (CAPACITY) Group.

BACKGROUND: This study's objective was to conduct a multinational registry of patients with carotid body tumors (CBTs) and to analyze patients' clinical characteristics, treatments, and outcomes. METHODS: Retrospective study from the Carotid Paraganglioma Cooperative International Registry involving eleven medical centers in Bolivia, Ecuador, Mexico, and Spain, of all patients with a CBT who underwent resection between 2009 and 2019. RESULTS: A total of 1432 patients with a CBT surgically treated were included. Median patient age was 54 years (range: 45-63 years), and 82.9% (1184) of the study cohort were female. While at low altitude, the proportion of female-to-male cases was 2:1, at high altitude, this proportion increased to 8:1, with statistically significant differences (p = .022). Median operative time was 139 min (range: 110-180 min), while median operative blood loss was 250 ml (range: 100-500 ml), with statistically significant difference in increased blood loss (p = .001) and operative time (p = .001) with a higher Shamblin classification. Eight (0.6%) patients suffered stroke. Univariate analysis analyzing for possible factors associated with increased odds of stroke revealed intraoperative vascular lesion to present an OR of 2.37 [CI 95%; 1.19-4.75] (p = 0.001). In 245 (17.1%), a cranial nerve injury was reported. Seven (0.5%) deaths were recorded. CONCLUSION: The most common CBT type on this cohort was hyperplasic, which might be partially explained by the high altitudes where these patients lived. Increased blood loss and operative time were associated with a higher Shamblin classification, and the risk of stroke was associated with patients presenting transoperative vascular lesions.

Bilateral Giant Familial Carotid Body Tumors With Concomitant Skull-Base Paraganglioma and Facial Nerve Palsy.

Carotid body tumors, rare neck paragangliomas arising from the common carotid artery bifurcation, can be classified as sporadic, hyperplastic, or familial. The familial type is often bilateral and associated with germline mutation of the mitochondrial enzyme succinate dehydrogenase. We report the rare case of a 42-year-old man who presented with bilateral giant familial carotid body tumors associated with a concomitant skull-base paraganglioma, left-sided facial nerve palsy, and an incomplete circle of Willis. We describe the excision of the tumors in 2 stages (the left mass and associated paraganglioma first and the right mass second), 6 months apart, with use of general anesthesia, and we discuss other operative considerations.

Surgical Excision of a Functional Carotid Body Tumor Presenting Intraoperative Hypertensive Crisis: A Case Report and Literature Review.

Carotid body tumor (CBT) is a rare neoplasm arising from the carotid bifurcation. Functional CBTs are extremely rare and are usually associated with elevated serum catecholamine and catecholamine-induced symptoms such as paroxysmal hypertension, palpitations, dizziness, flushing and tachycardia. We reported a 47-year-old female with a functional CBT that was biochemically silent and had no catecholamine-induced symptoms preoperatively, but experienced hypertensive crisis during surgical excision of the lesion. Postoperative pathology confirmed the diagnosis of CBT, and a functional tumor was considered due to the hypertensive crisis during surgical manipulation of the lesion. Under careful management of intraoperative blood pressure and close monitoring of vital sign along with adequate crystalloid infusion after surgery, the tumor was successfully excised and the patient recovered uneventfully during a follow-up period of 12 months. To the best of our knowledge, this is an unusual report of functional CBT presenting merely hypertensive crisis during surgery, with no elevated serum catecholamine or catecholamine-induced symptoms preoperatively. Clinicians should be aware of such lesions so that appropriate medication and gentle manipulation are given when encountering hypertensive crisis during surgical excision of CBTs, to prevent life-threatening cardiovascular complications. The appropriate management and recognition of functional CBTs were also discussed by means of a review of the literature.

[Rare Cause for Difficulty in Swallowing with Neck Swelling - Paraganglioma].

Rare Cause for Difficulty in Swallowing with Neck Swelling - Paraganglioma Abstract. Pain-free lateral swelling of the neck should be taken seriously and promptly clarified by using sonography. If a cervical paraganglioma is suspected, an interdisciplinary clarification should be initiated. In addition to an MRI of the neck, a vascular-surgical, an ENT specialist and an endocrinological examination should be carried out. If there is a high degree of suspicion of a carotid body tumor, surgical removal or a prospective procedure should be used in accordance with the Shamblin classification, the genetic clarification and the patient's individual life situation. If there is suspicion of vagal paraganglioma due to the location of the tumor and the symptoms, an expectative approach with regular check-ups should be considered in a symptom-poor and elderly patient.

Head and Neck Paraganglioma Atypically Carrying a Succinate Dehydrogenase Subunit B Mutation (L157X).

A 53-year-old woman was admitted to a hospital for gradual left-ear hearing loss over 2 years. Head computed tomography revealed a 2-cm mass along the left jugular bulb and another at the right carotid bulb. The right tumor was resected; the pathological diagnosis was carotid body paraganglioma. Mutations of succinate dehydrogenase (SDH) were suspected, but SDHB staining remained in the tumor. Genetic testing identified a known SDHB mutation (L157X). The patient had head and neck paraganglioma with an SDHB mutation (L157X) more typical of an SDHD mutation. SDHB immunohistochemistry is useful for detecting SDHx mutations, but careful interpretation is needed.

Risk assessment of hypertension in carotid body surgeries: A NSQIP analysis.

OBJECTIVES: Carotid body tumors (CBT) are rare paragangliomas of the carotid body at the carotid bifurcation. The purpose of this study was to determine the effect of hypertension on outcomes in carotid body tumor surgery. STUDY DESIGN: A retrospective database review. METHODS: Data on carotid body resections performed from 2005 to 2014 were drawn from the American College of Surgeons' National Surgical Quality Improvement database. Two groups were created based on the presence of preoperative hypertension. These groups were analyzed for demographics, comorbidities, and postoperative complications using bivariate and multivariate methods. RESULTS: Of the 452 patients included in the analysis, 49.3% had hypertension. Those with hypertension were significantly more likely to have additional comorbidities, which were controlled for by multivariate analysis to focus on hypertension. These hypertensive patients also had significantly longer hospital stays. Multivariate analysis showed that patients with hypertension undergoing carotid body resections had increased risk for overall medical complications but did not have increased risk for postoperative surgical complications or specific medical complications CONCLUSION: This statistically robust study revealed that hypertension does not independently increase a patient's risk for specific postoperative surgical complications following a carotid body tumor resection. However, hypertension increases the risk for postoperative medical complications and longer hospital stays. It is notable that almost half of all CBT patients have hypertension, and these hypertensives patients are significantly more likely to carry additional comorbid conditions that may have an adverse effect on outcomes including overall medical complications. LEVEL OF EVIDENCE: NA Laryngoscope, 130: 2008-2012, 2020.

Jarcho-Levin Syndrome and Concomitant Carotid Glomus Tumor: First Reported Case.

BACKGROUND: Jarcho-Levin syndrome (JLS) is a rare congenital disorder characterized by different clinical and radiologic findings. The disease was first reported by Jarcho and Levin in 1938, and it was described as the presence of various malformations or abnormal fusion in the thoracic vertebrae and ribs, short trunk, and respiratory distress. CASE DESCRIPTION: In our case, fusion at the thoracic and cervical vertebrae, butterfly vertebrae, and a crablike-shaped thorax was present. The patient had a short trunk, short stature, and long extremities. Moreover, he had a syndromic face and restrictive-type respiratory distress. There was a glomus tumor in the carotid space. In our literature review, we found that neural tube defects are frequently present in this syndrome. However, we could not identify any cases with affected neural crest cells. CONCLUSIONS: JLS may affect cells derived from the neural crest located between the neural tube and surface ectoderm. Therefore patients with JLS should be screened for other tumors located in this area.

Carotid body tumor with hidden internal carotid artery aneurysm.

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.

Multimodal management of pediatric carotid body tumors: a systematic review and case illustrations.

OBJECTIVE Carotid body tumors (CBTs), extraadrenal paragangliomas, are extremely rare neoplasms in children that often require multimodal surgical treatment, including preoperative anesthesia workup, embolization, and resection. With only a few cases reported in the pediatric literature, treatment paradigms and surgical morbidity are loosely defined, especially when carotid artery infiltration is noted. Here, the authors report two cases of pediatric CBT and provide the results of a systematic review of the literature. METHODS The study was divided into two sections. First, the authors conducted a retrospective review of our series of pediatric CBT patients and screened for patients with evidence of a CBT over the last 10 years (2007­2017) at a single tertiary referral pediatric hospital. Second, they conducted a systematic review, according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, of all reported cases of pediatric CBTs to determine the characteristics (tumor size, vascularity, symptomatology), treatment paradigms, and complications. RESULTS In the systematic review (n = 21 patients [includes 19 cases found in the literature and 2 from the authors' series]), the mean age at diagnosis was 11.8 years. The most common presenting symptoms were palpable neck mass (62%), cranial nerve palsies (33%), cough or dysphagia (14%), and neck pain (19%). Metastasis occurred only in 5% of patients, and 19% of cases were recurrent lesions. Only 10% of patients presented with elevated catecholamines and associated sympathetic involvement. Preoperative embolization was utilized in 24% of patients (external carotid artery in 4 and external carotid artery and vertebral artery in 1). Cranial nerve palsies (cranial nerve VII [n = 1], IX [n = 1], X [n = 4], XI [n = 1], and XII [n = 3]) were the most common cause of surgical morbidity (33% of cases). The patients in the authors' illustrative cases underwent preoperative embolization and balloon test occlusion followed by resection, and both patients suffered from transient Horner's syndrome after embolization. CONCLUSIONS Surgical management of CBTs requires an extensive preoperative workup, anesthesia, and multimodal surgical management. Due to a potentially high rate of surgical morbidity and vascularity, balloon test occlusion with embolization may be necessary in select patients prior to resection. Careful thorough preoperative counseling is vital to preparing families for the intensive management of these children. ABBREVIATIONS BTO = balloon test occlusion; CBT = carotid body tumor; CN = cranial nerve; ECA = external carotid artery; ICA = internal carotid artery; MIBG = iodine-123-meta-iodobenzylguanidine; PRISMA = Preferred Reporting Items for Systematic Reviews and Meta-Analyses.

Symptomatic carotid stenosis and ipsilateral carotid body tumor.

We report an unusual combination of a symptomatic carotid stenosis and ipsilateral carotid body tumor. This patient was successfully treated with simultaneous carotid endarterectomy and tumor resection. Following, the unique challenges and technique are discussed.

New predictors of complications in carotid body tumor resection.

OBJECTIVE: This study examined the relationship between two new variables, tumor distance to base of skull (DTBOS) and tumor volume, with complications of carotid body tumor (CBT) resection, including bleeding and cranial nerve injury. METHODS: Patients who underwent CBT resection between 2004 and 2014 were studied using a standardized, multi-institutional database. Demographic, perioperative, and outcomes data were collected. CBT measurements were determined from computed tomography, magnetic resonance imaging, and ultrasound examination. RESULTS: There were 356 CBTs resected in 332 patients (mean age, 51 years; 72% female); 32% were classified as Shamblin I, 43% as Shamblin II, and 23% as Shamblin III. The mean DTBOS was 3.3 cm (standard deviation [SD], 2.1; range, 0-10), and the mean tumor volume was 209.7 cm3 (SD, 266.7; range, 1.1-1642.0 cm3). The mean estimated blood loss (EBL) was 257 mL (SD, 426; range, 0-3500 mL). Twenty-four percent of patients had cranial nerve injuries. The most common cranial nerves injured were the hypoglossal (10%), vagus (11%), and superior laryngeal (5%) nerves. Both Shamblin grade and DTBOS were statistically significantly correlated with EBL of surgery and cranial nerve injuries, whereas tumor volume was statistically significantly correlated with EBL. The logistic model for predicting blood loss and cranial nerve injury with all three variables-Shamblin, DTBOS, and volume (R2 = 0.171, 0.221, respectively)-was superior to a model with Shamblin alone (R2 = 0.043, 0.091, respectively). After adjusting for Shamblin grade and volume, every 1-cm decrease in DTBOS was associated with 1.8 times increase in risk of >250 mL of blood loss (95% confidence interval, 1.25-2.55) and 1.5 times increased risk of cranial nerve injury (95% confidence interval, 1.19-1.92). CONCLUSIONS: This large study of CBTs demonstrates the value of preoperatively determining tumor dimensions and how far the tumor is located from the base of the skull. DTBOS and tumor volume, when used in combination with the Shamblin grade, better predict bleeding and cranial nerve injury risk. Furthermore, surgical resection before expansion toward the base of the skull reduces complications as every 1-cm decrease in the distance to the skull base results in 1.8 times increase in >250 mL of blood loss and 1.5 times increased risk of cranial nerve injury.

Concomitant Surgical Treatment of Symptomatic Carotid Artery Disease With a Coexisting Shamblin I Carotid Body Tumor.

Symptomatic carotid artery atherosclerotic disease is an indication for carotid artery endarterectomy. The coexistence of carotid body tumors (CBTs) with symptomatic carotid disease is rarely encountered and adds significant challenges to vascular surgeons, with a reported overall mortality for patients who undergo endarterectomy and tumor excision of 8.8%, as opposed to the 2% for those who had CBT excision only. We describe the case of a 79-year-old female who experienced an acute cerebrovascular accident in the left hemisphere; duplex ultrasound revealed high-grade carotid stenosis in the left side and the presence of a Shamblin I CBT. The risks and benefits of the planned operation were reviewed, and the decision was made to proceed with early carotid endarterectomy and concomitant surgical resection of the tumor using the retrocarotid dissection technique. The patient recovered well, and at 11 months from the combined procedure, her neurological deficits improved significantly.

Posterior reversible encephalopathy syndrome and reversible cerebral vasoconstriction syndrome after bilateral carotid paraganglioma resection: A case report.

Background Paraganglioma is a rare neuroendocrine tumour arising anywhere along the paravertebral sympathetic and parasympathetic chains. In the neck, paraganglioma may affect the carotid body (carotid body tumour). Case report We describe a 43-year-old woman who presented with a reversible vasoconstriction syndrome associated with a posterior reversible encephalopathy syndrome following a surgery for a left carotid paraganglioma (with a past medical history of surgery for a right carotid paraganglioma a few months before). Conclusion A consequence of a baroreflex modification is discussed in order to explain the rare occurrence of such symptoms.