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.

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.

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.

Current surgical management of carotid body tumors.

BACKGROUND: Carotid body tumors (CBTs) are rare. Management guidelines may include genetic testing for succinate dehydrogenase (SDH) mutations. We performed an institutional review of the surgical management of CBT. METHODS: A retrospective analysis (1994-2015) of CBT excisions at our institution was performed. Data obtained included demographics, genetic testing (if performed), intraoperative details, postoperative morbidity, and long-term outcomes. Data from the first CBT excision were included in patients with bilateral tumors. Genetic testing was routinely offered in patients with a family history of CBT or multiple paragangliomas. RESULTS: A total of 183 CBTs (124 female [67.7%]) were excised. A neck mass was present in 106 patients (57.9%), 24 patients (12.1%) presented with tenderness or neck pain, and 3 (1.6%) presented with cranial nerve dysfunction. Computed tomography (57.9%) or magnetic resonance imaging (51.3%) were the most commonly used imaging modalities. Preoperative angiography was performed in 73 patients (39.8%), and 62 of them (84.5%) underwent embolization or internal carotid balloon occlusion testing, or both. Mean tumor diameter was 3.2 cm (range, 0.6-7.2 cm). There were 71 (38.8%), 75 (41%), and 37 (20.2%) Shamblin type 1, 2, and 3 tumors, respectively. Average operating time was 224 minutes (range, 52-696 minutes). Average blood loss was 143.9 mL (range, 10-2000 mL). Arterial reconstruction with an interposition graft was required in 10, and patch angioplasty was performed in four. Cranial nerve injury was permanent in 10 (5.5%), and the rate of stroke was 1% (n = 2). A total of 382 lymph nodes were excised, and all were benign. There were no deaths ≤30 days. Only one patient presented with malignant disease 2 years after CBT excision, and this patient did not undergo genetic testing. Thirty-four (18.6%) had a family history of CBT. SDH testing was performed in 18 patients, and 17 tested positive. Positive genetic testing had a correlation with earlier age at operation (P < .0001). Mean age at diagnosis of patients with SDH mutations was 38.0 years, and patients without known SDH mutations presented at a mean age of 50.3 years. In patients with SDH mutations, tumor diameter, operating time, blood loss, and distribution of Shamblin type 1, 2, and 3 lesions were not significantly different compared with the control group. CONCLUSIONS: CBT can be treated with minimal morbidity and mortality; however, the subgroup of patients with positive SDH mutations may represent a variant group of younger patients. Vascular surgeons should be aware of genetic testing to identify patients and family members who should undergo additional preoperative testing and monitoring for other paragangliomas. Concomitant lymph node dissection does not appear to add value in absence of clinic suspicion for malignancy.

Magnetic Resonance Imaging and Magnetic Resonance Angiography Findings of External Carotid Artery Aneurysm and Coexisting Carotid Body Tumor.

Carotid body tumors originate from paraganglionic tissue in the bifurcation of the common carotid artery. Magnetic resonance imaging is a frequently used diagnostic method in the preoperative diagnosis of these tumors and provides appropriate information for surgical planning. In this study, the authors emphasize magnetic resonance imaging and magnetic resonance angiography findings of the external carotid artery aneurysm associated with carotid body tumor. This highlights that the diagnosis of carotid artery aneurysms is essential to avoid accidental injury to the vessel during tumor surgery.

Neurological complications in carotid body tumors: a 6-year single-center experience.

OBJECTIVE: Carotid body tumors are considered rare. However, there has been an increase in the number of these tumors managed at our center in recent years. Delayed presentation with large tumors is common. We studied the clinical profile, interventions, and outcomes of these tumors and assessed the factors influencing operative neurological morbidity and recurrence. METHODS: This retrospective study was conducted at the Christian Medical College in Vellore, a tertiary care center in south India. We analyzed the inpatient and outpatient records of patients diagnosed to have carotid body tumors undergoing excision from January 1, 2005 to December 31, 2011. Patients diagnosed to have vagal paragangliomas were excluded. RESULTS: Thirty-four of 48 tumors were excised from 32 patients (11 female, 21 male). Average age at presentation was 38.2 years, and three patients had familial bilateral tumors. All patients presented with a painless neck mass. There were 27 Shamblin group III, six Shamblin group II, and one Shamblin group I tumor. Eleven Shamblin group II/III tumors were associated with transient cranial nerve palsy or paresis (32.3%). Two Shamblin group III tumors were associated with perioperative stroke (5.8%). Preoperative embolization was done in 17 tumors, 12 of which were associated with neurological complications (two stroke, nine nerve palsy, one hemianopia). One patient underwent thrombolysis for a middle cerebral artery thrombus and recovered completely on follow-up, and another with a capsuloganglionic infarct managed conservatively had minimal persistent disability. Three patients had persistent nerve palsy (8.8%). Although complications were more common in patients with higher Shamblin group tumors, the difference was not statistically significant. CONCLUSIONS: The overall rate of neurological complications is higher with tumors of higher Shamblin groups. Preoperative embolization was not effective in reducing neurological complications. The rates of postoperative stroke and permanent cranial nerve palsy after resection of large tumors are acceptable.

A comparison of particulate and Onyx embolization in preoperative devascularization of carotid body tumors.

INTRODUCTION: Preoperative embolization of a carotid body tumor (CBT) is a useful adjunct prior to surgical excision because it decreases operative blood loss and improves surgical outcomes. Traditionally, this is performed by transarterial particulate embolization (TAPE). More recently, direct percutaneous embolization (DPE) with Onyx is recognized as a promising technique for preoperative embolization. We compared these two techniques in patients treated for CBTs at our institution. METHODS: We retrospectively reviewed cases of preoperative devascularization of CBT from 1 January 1995 through 1 September 2012. Patient cases were placed into two groups: TAPE and DPE. Operative blood loss, operative length, angiographic devascularization, embolization procedure complications, operative transfusion requirements, postoperative hospital stay, intensive care unit (ICU) stay, and procedure-related mortalities were compared. RESULTS: A total of 17 patients underwent preoperative devascularization of their CBT with TAPE technique and ten patients using the DPE technique with Onyx. Average operative blood loss was significantly higher in the TAPE group (Mann-Whitney U test, p = 0.04). Operative time was also higher, although this difference was not significant. Two patients required intraoperative blood transfusions in the TAPE group while none required transfusions in the DPE group. There was no significant difference in ICU stay or length of hospitalization. One serious embolization procedure complication occurred in the TAPE group and none in the DPE group. CONCLUSION: Operative blood loss in the DPE group was significantly less than the TAPE group. Blood transfusion requirement, operative time, and complications were less in the DPE group, although they did not reach statistical significance.

Resection of carotid body tumors and the additional choice of intraoperative shunt in complicated tumors.

BACKGROUND: The purpose of this article is to describe an additional choice of intraoperative shunt in the surgical repair of complicated carotid body tumors (CBTs). METHODS: Between January 2005 and August 2010, 47 CBT resections were performed at our division. Thirty-seven patients underwent routine tumor resection (78.7%). However, 10 of the tumor resections were complicated because of severe adhesions and involvement of the carotid artery. It was difficult to excise the tumors using routine methods. Intraoperative shunts were used for resection of these 10 complicated tumors (21.3%). RESULTS: All patients underwent successful resection of the CBTs. No severe intraoperative or postoperative complication was observed in the shunted group. There were two cases with hypotension and one case with blood pressure fluctuation in the unshunted group. The mean follow-up duration was 35.3 (range, 12-60) months. CONCLUSION: Surgical resection is the treatment of choice for CBTs. Shunts are not routinely used in the repair and represent just an additional choice for the resection of complicated CBTs. In this study, shunts have been shown to maintain cerebral circulation, decrease the size of tumor by excluding the vascular supply of the external carotid artery, and guide the resection when the tumors were complicated and difficult to excise. Shunt insertion was found to be safe and not associated with severe cerebrovascular complications.

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.

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.

Carotid body tumor presenting with carotid sinus syndrome.

A healthy 46-year-old woman presented to our hospital with complaints of sudden and repeated episodes of syncope without accompanying signs or symptoms. Neurological investigation proved negative and a 24-hour Holter monitoring device revealed significant nocturnal cardiac pauses. As such, a pacemaker was implanted in the patient, which was effective at preventing the syncopes. A subsequent investigation led to the undertaking of a cervical computed tomography (CT) scan that showed the existence of a right carotid body tumor. Rotational flexing or stretching of the neck immediately triggered the start of the pacemaker. The tumor was surgically removed and the patient no longer felt the activation of the pacemaker, which was removed 6 months after the operation. One year later, the patient was found to be well and completely free of her previous symptoms.

Reversible cerebral vasoconstriction syndrome in two patients with a carotid glomus tumour.

We report two patients with reversible cerebral vasoconstriction syndrome (RCVS) and carotid glomus tumour. The first patient presented with multiple thunderclap headaches. Cervical and cerebral magnetic resonance imaging showed diffuse cerebral vasoconstriction on magnetic resonance angiogram (MRA) and a carotid glomus tumour. The second patient presented with a cervical mass and was diagnosed with a non-secreting paraganglioma of the carotid body. Surgery with pre-operative angiography was followed by thunderclap headaches and MRA showed segmental cerebral vasoconstriction. Both patients were treated with nimodipine and headaches stopped. Both had normal cerebral arteries on the control MRA at 3 months. These two cases suggest that a paraganglioma may increase the susceptibility to develop RCVS. As a consequence, patients with RCVS should be investigated for a carotid glomus tumour, and patients with paraganglioma reporting severe headaches should have a cerebral MRA in order to rule out cerebral vasoconstriction.

Fine needle aspiration diagnosis of carotid body tumor in a case of multiple paragangliomas presenting with facial palsy: a case report.

BACKGROUND: Carotid body tumors (CBTs) constitute the most common extraadrenal paragangliomas. Many lesions diagnosed as CBTs by fine needle aspiration (FNA) cytology are clinically unsuspected. The main differential diagnosis is a thyroid neoplasm. The location of the mass in the lateral neck with prolonged history, hemorrhagic FNA and cytologic features resembling the endocrine neoplasm help in arriving at a suggestive diagnosis of paraganglioma. CASE: A 32-year-old male presented with left-sided facial palsy and swelling in the left side of the neck of 8 months' duration. The FNA sample was hemorrhagic and showed loosely arranged groups and acini formed by round to oval cells. A diagnosis of CBT was suggested. It was supplemented by additional noninvasive methods, such as ultrasonography of the neck region with color Doppler, computed tomography and magnetic resonance imaging of the brain, which also revealed a neoplasm suggestive of carotid body tumor in the right side of the neck and neoplastic lesion in the left cerebellopontine angle, suggestive of paraganglioma. CONCLUSION: FNA, with the other noninvasive radiologic investigations, plays an important role in the diagnosis of CBT. We present this case of multiple paragangliomas for its unusual presentation and FNA diagnosis.

[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.

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.

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.