Current management of carotid body tumors.

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.

Resorbable Purified Porcine Skin Gelatin Cross-Linked with Glutaraldehyde Spheres for Preoperative Embolization of Carotid Body Paraganglioma.

Chemodectomas are rare neuroendocrine tumors that typically arise at the carotid bifurcation and progressively englobe the internal and external carotid artery. Surgical asportation of the capsulated mass is the elective treatment. Pre-procedural embolization of this high vascular tumors is highly recommended because it has shown to improve surgical outcome by reducing both, mean blood loss and total operation time. Many different embolization techniques have been described in literature. In the here presented case we opted for an endovascular approach using resorbable purified porcine skin gelatin cross-linked with glutaraldehyde microspheres (Optisphere - MEDTRONIC) as an embolic agent. These turned out to be very safe and effective in improving surgical outcome by reducing operative blood loss and thus reducing treatment-related morbidity.

Contemporary management of carotid body tumors in a Midwestern academic center.

BACKGROUND: Carotid body tumors are rare, neurogenic tumors arising from the periadventitial chemoreceptive tissue of the carotid body. The purpose of this study is to ascertain the presentation and preoperative risk factors associated with surgical resection. METHODS: A single-center retrospective review of 25 carotid body tumor resections from 2002 to 2019. Demographics, periprocedural details, and postoperative outcomes were analyzed using Stata (Stata Corporation, College Station, TX). RESULTS: Among 25 patients, 64% were women, 84% were asymptomatic, and the mean age was 49 years (range 21-79). Forty-four percent of tumors were Shamblin III. Nine patients underwent preoperative embolization but did not correlate with decreased blood loss (299 cm3 vs 205 cm3, P = .35). The 30-day death, stroke, and cranial nerve injury rates were 0%, 8%, and 32%, respectively. Cranial nerve injuries included 20% vagus, 4% hypoglossal, 4% facial, and 4% glossopharyngeal, with permanent deficits in 4% (n = 1). Mean length of stay was 3.0 days (range 1-9 days). At a mean follow-up of 12 months (range 1-63 months), there has been no recurrence. CONCLUSION: Although carotid body tumors are uncommon in the Midwest, complete surgical resection is curative of these typically hormonally inactive tumors. Preoperative embolization did not affect blood loss, and the incidence of death, stroke, and permanent cranial nerve injury rates remained very low.

Identifying Factors Influencing Decision Making in Patients Diagnosed with Carotid Body Tumors: An Exploratory Study.

BACKGROUND: Carotid body tumors (CBTs) are rare highly vascularized and slow enlarging tumors arising from the paraganglionic tissue at the carotid bifurcation. Main treatment options for CBTs are surgical resection or "wait and scan" strategy. The choice for either strategy may be equally good medically in many patients. A structured "shared decision making" (SDM) might be helpful for guiding patients. OBJECTIVES: To develop an SDM strategy for the surgical treatment, we aim to (1) identify considerations and factors involved in the decision making of patients with CBTs and (2) evaluate the current practice in our clinic and explore the opinions of patients on their treatment. METHODS: This exploratory study was conducted in patients of the Leiden University Medical Centre (LUMC), The Netherlands. Patients who met the inclusion criteria were invited for a semi-structured interview. All conversations were fully audiotaped and transcripted. RESULTS: Fifteen patients were included and interviewed. Ten of these patients underwent previously surgical resection of at least one tumor. Five patients underwent the wait and scan policy. The most important factors influencing decision making in CBT treatment are family, fears, co-consultants, and doctor-patient relationship. CONCLUSIONS: This study has identified the factors influencing decision making in CBT and should be considered during consultations. The decision for surgery or not was mainly influenced by physician preferences and family members' prior experiences.

Preoperative Squid embolization of carotid paragangliomas with direct puncture.

BACKGROUND AND PURPOSE: Preoperative embolization of carotid paragangliomas is a common procedure in interventional neuroradiology. Direct puncture embolization has shown less morbidity and mortality than endovascular embolization and a higher percentage of devascularization. We describe our experience using Squid® as the only embolic agent in direct puncture glomus embolization. METHODS: We retrospectively reviewed pre-embolization imaging tests, emphasizing the volume of the lesion, clinical history data, technical aspects of the procedure, as well as the approximate amount of blood lost during the surgical procedure in all patients with preoperative embolization of carotid paragangliomas performed at our tertiary care hospital. RESULTS: Six patients met our criteria from May 2017 to August 2018. The volume of the mass ranged from 1.4-18.5 mL and the quantity of Squid® injected varied from 1.1-15 mL. Total devascularization was achieved in almost all cases (>90%), with one puncture needed in all but one patient, who was punctured two times. No hemorrhagic complications were described in surgery, no blood transfusions were needed, and the mean decrease of hemoglobin after surgery was 1.04 g/dL. CONCLUSIONS: Direct puncture embolization of carotid paragangliomas only using Squid® is a safe and relatively simple procedure that facilitates the subsequent surgery with minimal blood loss.

Descending musculospinal branch of the ascending pharyngeal artery as a feeder of carotid body tumors: Angio-architecture and embryological consideration.

INTRODUCTION: Although the ascending pharyngeal artery (APhA) is known as a main feeder of carotid body tumors (CBTs), its detailed architecture and embryological consideration have not been described. The objective of this study was to describe the architecture of a descending feeder of the APhA and to consider its origin embryologically through a review of our CBT embolizations. METHODS: We retrospectively analyzed data from patients with CBTs who underwent transarterial embolization or angiographic examination-only between July 2010 and February 2017. The arterial supply of the tumors, the number of feeder pedicles, the mean tumor size, embolization materials, complication of embolization, and extent of tumor removal were assessed. The embryological origin of feeding artery was considered based on the literature. RESULTS: Eighteen patients with 20 CBTs underwent preoperative embolization or angiographic examination. The number of feeder pedicles was significantly related to the size of the CBT (P = 0.0002). The main feeding artery was the descending branch of APhA, which was hypertrophied and tortuous (18/20, 90%). Embryologically, this artery originated from the musculospinal branch and is termed the "descending musculospinal branch". CONCLUSION: The main feeder of the CBTs was the "descending musculospinal branch" of the APhA and needs special consideration such as dangerous anastomosis for embolization.

Carotid body tumor contemporary management in a high-volume center.

BACKGROUND: The aim of this study is to report our results with carotid body tumor (CBT) surgical management. METHODS: Between 2010 and 2018, 100 CBTs (mean age: 48.0 years, range 21-80 years old) were treated in our center. The patients were classified in 3 groups according to the size: group I (<3 cm), group II (3 to 5 cm) and group III (>5 cm). RESULTS: Surgical resection was performed in 88 patients and conservative treatment in 2 cases. Nine patients were treated for bilateral involvement and one of them was also treated again for a relapse. Postoperatively, cranial nerve injury (CNI) was significantly higher in group II [3 (8.3%) vs. 16 (31.4%) vs. 2 (15.4%); P=0.030] with no statistical differences between the CN involved. At univariate analysis for CNI, CBT group (P=0.030), maximum diameter (P=0.046), patients presenting with dysphonia (P=0.035) and dysphagia (P=0.007) and patients suffering from any intraoperative complication (P=0.047) were statistically significant. At multivariate analysis the only significant variable was CBT group II (P=0.016). For blood loss, CBT group III (P<0.001), Shamblin class III (P<0.001), Pulmonary disease (P=0.034) and surgery time (P<0.001) were statistically significant. The follow-up of 79 patients (87.8%) showed a 100% overall survival at median follow-up of 37.7 months (range 2-84.7 months) with freedom from local recurrence of 97.8% (77/79). CONCLUSIONS: Surgical resection remains the gold standard to obtain complete recovery, although tumor size is to be considered a risk factor for CNI because large CBTs remain at high risk for CNIs.

Role of Preoperative Embolization in Surgical Treatment of the Carotid Body Paragangliomas.

In this study, we aimed to show the benefits of preoperative embolization on surgery of carotid body tumors.This is a retrospective study of the medical records of 20 patients who were operated for carotid body paragangliomas during 2011-2017. Computed tomography angiography (BTA) and/or magnetic resonance angiography (MRA) were performed for the entire patients. Patients were assessed according to the Shamblin classification. All patients were subjected to direct cerebral angiography by the femoral route 24 to 48 hours before the surgery and tumor embolization was performed in the appropriate cases (19 patients). The amount of hemorrhage and transfusion need during surgery has been reported. The ages of the patients ranged from 21 to 66, with an average of 45.65. In 20 patients ascendan pharyngeal artery was found as the main artery of tumor. No complications were seen after the embolization procedure. Tumor sizes ranged from 12 mm to 95 mm diameter (mean: 45, 5 mm). 10 patients (50%) were Shamblin type 1, 6 patients (30%) were Shamblin type 2, 4 patients (20%) were Shamblin type 3. In 5 patients (25%), the nerve (N. vagus or hypoglossus) was sacrificed due to cranial nerve involvement. Vagal and hypoglossal nerve sacrificed together in 4 patients.These patients' tumor sizes were bigger than 70 mm. Only in 1 patient, internal carotid artery injured and reconstructed with saphenous vein graft. Neurological deficit did not develop postoperatively. Bleeding was mean 275 mL (125 mL-700 mL). In conclusion, we believe that preoperative embolization of carotid paraganglioma is an effective procedure that helps surgeon during dissection with minimal blood loss. The embolization is relatively easy procedure without no additional complications.

Effective, same-day preoperative embolization and surgical resection of carotid body tumors.

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

A Systematic Review and Meta-Analysis of the Presentation and Surgical Management of Patients With Carotid Body Tumours.

OBJECTIVES: The aim was to determine the mode of presentation and 30 day procedural risks in 4418 patients with 4743 carotid body tumours (CBTs) undergoing surgical excision. METHODS: This is a systematic review and meta-analysis of 104 observational studies. RESULTS: Overall, 4418 patients with 4743 CBTs were identified. The mean age was 47 years, with the majority being female (65%). The commonest presentation was a neck mass (75%), of which 85% were painless. Dysphagia, cranial nerve injury (CNI), and headache were present in 3%, while virtually no one presented with a transient ischaemic attack (0.26%) or stroke (0.09%). The majority (97%) underwent excision, but only 21% underwent pre-operative embolisation. Overall, 27% were Shamblin I CBTs; 44% were Shamblin II; and 29% were Shamblin III. The mean 30 day mortality was 2.29% (95% CI 1.79-2.93). The mean 30 day stroke rate was 3.53% (95% CI 2.91-4.29), while the mean 30 day CNI rate was 25.4% (95% CI 24.5-31.22). The prevalence of persisting CNI at 30 days was 11.15% (95% CI 8.42-14.64). Twelve series (544 patients) correlated 30 day stroke with Shamblin status. Shamblin I CBTs were associated with a 1.89% stroke rate (95% CI 0.92-3.82), increasing to 2.71% (95% CI 1.43-5.07) for Shamblin II CBTs and 3.99% (95% CI 2.34-6.74) for Shamblin III tumours. Twenty-six series (1075 patients) correlated CNI rates with Shamblin status: 3.76% (95% CI 2.62-5.35) for Shamblin I CBTs, 14.14% (95% CI 11.94-16.68) for Shamblin II, and 17.10% (95% CI 14.82-19.65) for Shamblin III tumours. The prevalence of neck haematoma requiring re-exploration was 5.24% (95% CI 3.45-7.91). The proportion of patients with a neck haematoma requiring re-exploration was not reduced by pre-operative embolisation (5.92%; 95% CI 2.56-13.08) vs. no embolisation (5.82%; 95% CI 2.76-11.88). Pre-operative embolisation did not reduce drainage losses (639 mL vs. 653 mL). CONCLUSIONS: This is the largest meta-analysis of outcomes after CBT excision. Procedural risks associated with tumour excision were considerable, especially with Shamblin III tumours where 4% suffered a peri-operative stroke and 17% suffered a CNI.

Surgical resection of carotid body tumors with versus without preoperative embolization: Retrospective case-control study.

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

[Carotid body tumor and its treatment. A case report]. / A glomus caroticum paragangliomája és ellátása.

Our goal was to report a 44-year-old woman with carotid body tumor, and to give a brief and comprehensive presentation about the disease and summarize its complex management. Carotid body tumor is a rare, mostly sporadic, benign head and neck mass originating from the glomus caroticum. It occurs mostly in middle-aged women as a slowly growing, semifix, painless neck mass. The diagnosis is based upon the physical examination (pulsatile mass, Fontaine-sign) and - as the gold standard - CT-angiography. It should be established that preoperative embolisation by digital substraction angiography plays a crucial role in the treatment of carotid body tumors that should be followed by surgical removal. In case of inoperable/irresecable and residual tumors, radiotherapy is the treatment option. The patient was treated in our department in April 2017. Orv Hetil. 2018; 159(36): 1487-1492.

Impact of preoperative transarterial embolization of carotid body tumor: A single center retrospective cohort experience.

OBJECTIVE: To evaluate the feasibility and efficacy of preoperative hyperselective transarterial embolization in carotid body tumor resection. METHODS: Retrospectively analyze the clinical feature, imaging examination, treatment strategy and prognosis of 29 carotid body tumor patients from January 2006 to January 2016. According to whether to carry out the pre-operative transarterial embolization, the patients were classified into embolization group and non-embolization group. The blood loss, operative time and perioperative complications were compared between the 2 groups. RESULTS: 29 carotid body tumors were resected. The median of blood loss was 80 mL in embolization group and 200 mL in non-embolization group, the difference was statistically significant (P = 0.001). The median of operative time was 120 min in embolization group and 160 min in non-embolization group, the difference was statistically significant (P = 0.006). No death, paralysis or ectopic embolism occurred in the study population. 4 patients in embolization group and 4 in non-embolization group suffered from cranial nerve injury. CONCLUSION: Surgical resection of carotid body tumor is safe and reliable, which is the first choice in the treatment of carotid body tumor. Preoperative transaterial hyperselective embolization can significantly reduce blood loss and shorten operative time, but it dose not decrease the incidence of cranial never injury.

Acquired Vascular Tumors of the Head and Neck.

Vascular neoplasms of the head and neck present with a wide spectrum of signs and symptoms. Diagnosis requires a high index of suspicion and is usually made after tumors are large enough to be visually apparent or cause symptoms. This article discusses the most common acquired benign and malignant vascular tumors, with an emphasis on their evaluation and treatment.

Carotid Body Tumors: A Case Series and Review of the Literature.

BACKGROUND: Paragangliomas of the head and neck are rare vascular tumors derived from the paraganglia tissues originating from the neural crest. They are usually benign and hypervascularized. Diagnosis is relatively easy in condition to consider it in evaluating every lateral neck mass. METHODS: We made a retrospective study of the records of 10 patients who presented with carotid body tumors at the Department of Vascular surgery of the Military Hospital Avicenne in Marrakech during the period between 2008 and 2013. Epidemiologic, etiologic, diagnostic, and therapeutic features were analyzed. RESULTS: The average age of our patients was 35.4 years (26-55 years), with a male predominance (sex ratio = 2.33). We noted 7 cases of isolated carotid locations and 3 cases of multiple locations. A slow-growing neck mass was the main clinical presentation. Other signs were pain, dysphonia, dizziness, headache, and tinnitus. Physical examination showed, in most cases, a neck nontender mass with side to side mobility. Imaging techniques included Doppler ultrasound, computed tomography (CT) scan, magnetic resonance imaging, and catheter arteriography. Urinary analysis for metanephrine was carried out in 1 case. The clinical presentation and imaging results strongly suggested the diagnosis of carotid paraganglioma in all cases. Treatment was surgical excision in all cases associated with a preoperative embolization in 1 case and a postoperative radiotherapy in 2 cases. Pathology confirmed the diagnosis, and a lymph node metastasis was suspected of malignity in 1 case. The evolution was favorable in all our patients. CONCLUSIONS: Carotid body tumor requires early diagnosis and an adequate multidisciplinary team. The diagnosis must be considered in the case of any pulsatile cervical mass. Surgery is the treatment of choice despite its risks especially in large tumors. The therapeutic indication should, ideally, be set in a multidisciplinary consultation.

Left Atrial and Carotid Body Paraganglioma.

We report a rare case of left atrial paraganglioma with a synchronous carotid body paraganglioma in a 30-year-old man with succinate dehydrogenase B gene mutation. The patient initially presented with a neck mass and palpitations. Laboratory test results showed elevated catecholamine levels. A cardiac paraganglioma was identified by computed tomography, meta-iodobenzylguanidine scintigraphy, and magnetic resonance imaging. Surgical resection of both paragangliomas were performed on two separate occasions. Serum and urine catecholamine levels returned to normal range. On follow-up, there was no recurrence of the cardiac paraganglioma. Radiotherapy was subsequently initiated for recurrence in the carotid body paraganglioma.

Management of carotid body tumor in pediatric patients: A case report and review of the literature.

Carotid body tumors (CBTs) are rare types of extra-adrenal paragangliomas, which originate from the neuroendocrine cells of the adventitial layer of carotid bifurcation. An 8-year-old girl was admitted to our department with left-sided and pulsating neck swelling, which progressively grew over several months. The patient had no family history of CBTs. Computed tomography (CT) and CT angiography (CTA) scans revealed a contrast material enhancing, hypervascularized mass involving the left carotid bifurcation, internal carotid artery (ICA), and external carotid artery (ECA), respectively. Preoperative embolization and surgical resection were performed. Histopathological examination later confirmed a benign CBT. Current follow-up examination revealed no evidence of residual or recurrent tumor. A systemic review of the literature indicates that early diagnosis, and experienced multi-disciplinary management is required in case of unilateral, resectable forms of CBTs with no distant metastasis, in order to provide a long-time survival of patients. Surgical intervention of unilateral, solitary CBTs with preoperative embolization is a relatively safe procedure, allowing complete tumor removal with minimal morbidity and low recurrence rate.

Tumor de corpo carotídeo em jovem adolescente com pré-síncope / Carotid body tumor in teenager with pre-syncope

O tumor do corpo carotídeo (TCC) é uma neoplasia rara, mas entre os paragangliomas é o mais freqüente, com incidência de 1: 1.170.000. Desenvolve-se na adventícia do vaso, próximo à bifurcação carotídea, afetando principalmente indivíduos entre a quarta e a quinta décadas de vida. É um tumor de crescimento lento, geralmente benigno e unilateral, e 10% deles são familiares. Em cerca de 5% dos casos, a lesão é bilateral, atingindo 32% se autossômica dominante. Podem ser assintomáticos até se manifestarem como uma massa cervical pulsante, de crescimento lento, indolor, na região lateral do pescoço, próximo ao ângulo da mandíbula. A TCC pode causar rouquidão e dificuldade de deglutição por compressão extrínseca e também síndrome do corpo carotídeo hipersensível (SCCHS). Esta síndrome é conseqüência da hiperestimulação dos barorreceptores, causando hipotensão postural, acompanhada de diaforese, pré-síncope ou síncope. Neste relato descrevemos uma paciente de 17 anos com TCC familiar e SCCHS, diagnosticada e tratada com sucesso, evoluindo sem seqüelas e assintomática. (AU)

Carotid body tumor (CBT) is a rare neoplasm, but among paragangliomas it is the most frequent, with an incidence of 1: 1,170,000. It develops in the adventitia of the vessel, near the carotid bifurcation, affecting mainly individuals between the fourth and fifth decades of life. It is a slow-growing tumor, usually benign and unilateral, and 10% of them are familial. In about 5% of the cases, the lesion is bilateral, reaching 32% if it is autosomal dominant. They may be asymptomatic until they manifest as a slowly growing, painless, pulsatile cervical mass in the lateral region of the neck, near the angle of the mandible. CBT can cause hoarseness and difficulty of swallowing by extrinsic compression and also hypersensitive carotid body syndrome (SCCHS). This syndrome is a consequence of hyperstimulation of the baroreceptors, causing postural hypotension, accompanied by diaphoresis, pre-syncope or syncope. In this case report we describe a 17-year-old patient with familial CBT and SCCHS, diagnosed and treated successfully, evolving without sequelae and asymptomatic. (AU)