Study on the Influencing Factors of Short-Term Recovery of Neurological Symptoms after Carotid Body Tumor Resection.

OBJECTIVE: To compare the differences in the short-term recovery from neurological symptoms (SRN) (≤ 6 months) and clinical characteristics of patients with different Shamblin classifications carotid body tumor (CBT) resection and to analyze the risk factors affecting SRN after surgery. METHODS: Patients who underwent CBT resection between June 2018 and September 2022 were recruited. Perioperative factors and indicators of the nature of the tumor were recorded. The risk factors affecting SRN after CBT resection were analyzed using logistic regression analysis. RESULTS: Eighty-five patients (43.86 ± 12.7 years, 46 females) were included, 40 (47.06%) of whom exhibited SRN. Univariate logistic regression showed that preoperative symptoms, surgical side, bilateral posterior communicating artery (PcoA) opening, some indicators of tumor size, operative/anesthesia time, and Shamblin III classification were correlated with postoperative neurological prognosis (all p < 0.05). After adjusting for confounders, preoperative symptoms (OR, 5.072; 95% CI 1.027-25.052; p = 0.046), surgical side (OR, 0.025; 95% CI 0.003-0234; p = 0.001), bilateral PcoA opening (OR, 22.671; 95% CI 2.549-201.666; p = 0.005), distance from the tip of the C2 dens to the superior aspect (dens-CBT) (OR, 0.918; 95% CI 0.858-0.982; p = 0.013) and Shamblin III classification (OR, 28.488; 95% CI 1.986-408.580; p = 0.014) were correlated with postoperative neurological symptom recovery. CONCLUSION: Preoperative symptoms, surgical side (right), bilateral PcoA opening, a short dens-CBT and Shamblin III classification are risk factors affecting SRN after CBT resection. Early resection is recommended for small-volume CBTs without neurovascular compression or invasion to obtain SRN.

Long-Term Outcomes of Patients With Carotid and Aortic Body Tumors.

Chemodectomas are tumors derived from parasympathetic nonchromaffin cells and are often found in the aortic and carotid bodies. They are generally benign but can cause mass-effect symptoms and have local or distant spread. Surgical excision has been the main curative treatment strategy. The National Cancer Database was reviewed to study all patients with carotid or aortic body tumors from 2004 to 2015. Demographic data, tumor characteristics, treatment strategies, and patient outcomes were examined, split by tumor location. Kaplan-Meier survival estimates were generated for both locations. In total, 248 patients were examined, with 151 having a tumor in the carotid body and 97 having a tumor in the aortic body. Many variables were similar between both tumor locations. However, aortic body tumors were larger than those in the carotid body (477.80 ± 477.58 mm vs 320.64 ± 436.53 mm, p = 0.008). More regional lymph nodes were positive in aortic body tumors (65.52 ± 45.73 vs 35.46 ± 46.44, p <0.001). There were more distant metastases at the time of diagnosis in carotid body tumors (p = 0.003). Chemotherapy was used more for aortic body tumors (p = 0.001); surgery was used more for carotid body tumors (p <0.001). There are slight differences in tumor characteristics and response to treatment. Surgical resection is the cornerstone of management, and radiation can often be considered. In conclusion, chemodectomas are generally benign but can present with metastasis and compressive symptoms that make understanding their physiology and treatment important.

Carotid body paraganglioma metastatic to spine causing cord compression: a case report.

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

Carotid body tumors: A retrospective case series of 11 patients.

BACKGROUND: Carotid body tumors (CBTs) are uncommon neuroendocrine tumors located near the carotid bifurcation within the carotid body. CBTs are slow-growing; affected individuals may remain asymptomatic for years and are often diagnosed incidentally following imaging studies. When present, symptoms are nonspecific. Because incidence is low, retrospective review of CBT case series is an ideal study design for investigating common symptomology, identifying successful diagnostic tools, and evaluating treatment outcomes. METHODS: This article describes a case series of 11 patients treated for CBTs at an academic medical center in the Southeastern US. A retrospective chart review of 11 patients who had been discharged from the hospital following treatment for CBTs between 2017 and 2021 was conducted. Descriptive statistics were used to delineate the case series. RESULTS: There were four males (36%) and seven females (64%) included in the retrospective case series. The age range was 34 to 79 with a mean of 56.73 (standard deviation 16.038). Three patients were found to have a neck mass on physical exam. Additional symptoms included vocal cord paralysis, facial paralysis, ischemic stroke, Bell palsy, dysphonia, and dizziness. Two patients had familial CBTs. Ten patients underwent surgical excision. CONCLUSION: This case series showed that patients with CBTs should be assessed with a combination of history and physical exams along with imaging studies to confirm the diagnosis. Because CBTs may become malignant and metastasize, it is important for NPs to be knowledgeable about presenting symptoms and appropriate diagnostic studies to be able to refer patients to vascular surgeons early to prevent complications.

Paraganglioma cuerpo carotídeo: experiencia en 20 años y revisión de la literatura / Carotid body paraganglioma: 20-year experience and literature review

Resumen El paraganglioma carotideo es un tumor infrecuente, originado de las células de la cresta neural. Raramente son secretores y tienen un bajo potencial maligno. El diagnóstico es difícil y requiere una alta sospecha clínica, combinada con estudios imagenológicos. Su tratamiento está basado en la cirugía, con especial cuidado de las estructuras vasculonerviosas que se encuentran en intimo contacto. Se describe la casuística de paragangliomas de cuerpo carotídeo en Clínica Las Condes y compararla con una revisión de la literatura actualizada del tema.

Abstract Carotid paraganglioma is a rare tumor, originated from neural crest cells. Usually they lack hormone secretion function, and have a low malignant potential. Diagnosis is difficult, and requires high clinical suspicious, combined with image and pathologic findings. Its treatment is based on surgery, with special care of close anatomic relation with important vascular-nervous structures. Here, we present cases of carotid paragangliomas evaluated at Clinica Las Condes comparing them with an updated literature review.

[A case of cervical lymph node metastasis after carotid body tumor surgery].

Malignant carotid body tumor is rare, reported this case who had experienced the totally resection of carotid body tumor 16 years ago and presented with cervical tumor now. The patient was a 68 year old male who presented with a low growing left-sided painless neck mass for half a year. Carotid body tumor resection and left carotid artery artificial vascular reconstruction were performed 16 years ago. Physical examination showed that multiple hard and painless masses can be touched behind the left sternocleidomastoid muscle of the neck. Blood routine and biochemical tests were normal. MRI enhancement of the neck showed multiple circular abnormal signals behind the left sternocleidomastoid muscle, with obvious enhancement. The cervical masses of this case were experienced totally resected again, and the postoperative pathology confirmed that it was carotid body tumor and cervical lymph node metastasis. The clinical diagnosis was malignant carotid body tumor with cervical lymph node metastasis. Postoperative PET-CT examination showed multiple lymphadenopathy and abnormal metabolism in hilar, mediastinum and abdominal aorta. Multiple systemic metastasis of malignant carotid body tumor was considered. It was suggested that preoperative whole-body PET-CT examination can be used to evaluate whether the tumor has systemic metastasis.

Resección-reparación arterial como parte de la resección de un paraganglioma carotídeo / Arterial resection-repair as part of the resection of a carotid paraganglioma

Los autores de este artículo, con base en su experiencia personal, que supera las tres décadas (1991-2021) y el medio centenar de pacientes (53 con 58 paragangliomas carotídeos y 5 casos bilaterales), explican cómo realizan la resección quirúrgica del paraganglioma carotídeo. En esta segunda parte, menos frecuente, se exponen los aspectos técnicos y tácticos de la resección-reparación arterial (eje carotídeo) como parte de la resección de ciertos paragangliomas carotídeos. Aunque siguen patrones estándares, los autores muestran aspectos personales de la técnica quirúrgica que implementan actualmente.(AU)

The authors of this article, based on their personal experience that exceeds three decades (1991-2021) and fifty patients (53 with 58 carotid paragangliomas and 5 bilateral cases), indicate how they perform surgical resection. In this second part, which is less frequent, the technical (and tactical) aspects of arterial resection-repair (carotid axis) are exposed as part of the resection of certain carotid paragangliomas. Although they follow standard patterns, the authors show personal aspects of the surgical technique that they currently implement.(AU)

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.

Paragangliomas of the head and neck.

Paragangliomas are rare neuroendocrine tumors that can be found from the skull base to the pelvis. Head and neck paragangliomas have been historically treated with surgery. However, surgical resection adds risk of injury to vascular structures and cranial nerves that can lead to morbidity such as hoarseness, dysarthria, dysphagia, or aspiration. Recently, improved understanding of the behavior of these tumors and increasing experience in non-surgical treatments, such as observation and radiation therapy, have changed the paradigms of management of this entity. Multiple series now show a trend toward a more conservative management, with a higher percentage of patients being observed or treated with radiotherapy. Several factors should be taken into consideration when deciding the most appropriate treatment for head and neck paragangliomas, starting by differentiating carotid body tumors from non-carotid body tumors. In general, surgical resection is normally recommended for carotid body tumors as the complications from treatment are usually minimal. In contrast, for non-carotid body tumors, surgery is often associated with significant functional impairment due to cranial nerve paralysis. As such, non-surgical treatment is now usually recommended for this subset of head and neck paragangliomas. In young patients with no comorbidities and a small to medium carotid body tumors, surgery should be considered. Moreover, surgery should be offered for secreting tumors, malignant tumors, tumors with rapid growth or increase in symptomatology, and when radiotherapy cannot be performed. Conversely, conservative management with active surveillance or radiotherapy can be offered in the remaining cases in order to avoid unnecessary morbidity while still providing acceptable tumor control.

Carotid glomus in childhood: presentation of a clinical case. / Glomus carotídeo en la infancia. Presentación de un caso clínico.

INTRODUCTION: Carotid glomus is an exceptional extra-adrenal paraganglioma in childhood originating at the carotid body. Only 3% of paragangliomas occur in the head and the neck. Familial forms, associated with Succinate Dehydrogenase (SDH) gene mutations, account for 10% of cases, the proportion being higher in childhood. They are benign in 95% of patients, but they can extend to both carotids. Treatment is surgical with or without previous embolization. Metastasis is rare and associated with malignant cases, which are limited. CLINICAL CASE: 8-year-old patient with a cervical mass originating 4 months ago and normal serum levels. Regarding family history, she had an aunt who underwent cervical surgery. Ultrasound examination demonstrated a greatly vascularized hypoechoic mass most likely related to carotid glomus. Full surgical resection without embolization was decided upon, which proved uneventful. The genetic study was positive for SDH gene mutation. CONCLUSION: Carotid glomus in childhood should be considered as a differential diagnosis in cervical masses. Surgical treatment without previous embolization represents a safe therapeutic option in selected cases.

INTRODUCCION: El glomus carotídeo es un paraganglioma extraadrenal, excepcional en la infancia, cuyo origen es el cuerpo carotídeo. Solo el 3% de los paragangliomas se presentan en cabeza y cuello. Existe un 10% de formas familiares asociadas a mutaciones en el gen de la succinato deshidrogenasa (SDH), porcentaje que es mayor en la infancia. Son tumores benignos en un 95% de los pacientes, pero pueden afectar por extensión a ambas carótidas. Su tratamiento es quirúrgico con o sin embolización previa. Las metástasis son raras y están asociadas a los escasos casos de malignidad. CASO CLINICO: Paciente de 8 años de edad con masa cervical de 4 meses de evolución, serologías normales. Como antecedente destaca una tía sometida a cirugía cervical. Ecográficamente se objetiva masa hipoecoica muy vascularizada en probable relación con glomus carotídeo. Se decide resección quirúrgica sin embolización que resulta completa y sin incidencias. Estudio genético positivo para la mutación en el gen de la SDH. CONCLUSION: El glomus carotídeo en la infancia ha de ser tenido en cuenta como diagnóstico diferencial en masas cervicales. El tratamiento quirúrgico sin embolización previa es una opción terapéutica segura en casos seleccionados.

Improving Outcomes in Carotid Body Tumors Treatment: The Impact of a Multidisciplinary Team Approach.

BACKGROUND: The purpose of this study was to evaluate how a multidisciplinary approach, including patients and familiar genetic counseling, preoperative succinate-dehydrogenase (SDH) gene mutation analysis, preoperative adjunctive endovascular procedures (PAEPs) and postoperative rehabilitative team may affect the outcomes in patients who underwent surgery for carotid body tumors (CBTs). METHODS: Fifty-seven consecutive CBT resections were performed from January 1995 to December 2019 in a single center institution. Two groups of patients were compared: group A (1995-2003; n = 10) and group B (2004-2019; n = 47), treated before and after the establishment of a multidisciplinary approach to CBTs. Group A and group B were evaluated retrospectively and prospectively for SDH mutations, respectively. PAEPs (external carotid artery stenting, percutaneous transfemoral embolization or direct percutaneous puncture of the tumor with simultaneous embolization) were performed only in patients of group B, when the size of the tumor exceeded the 45 mm. Primary endpoints were blood loss (BL) and cranial nerve injuries. Secondary endpoint was the number of new silent masses (NSMs) discovered after genetic evaluation. RESULTS: SDH mutations were found in 2 patients of group A and in 11 patients of group B. There were no significant differences in mass diameter between the groups. A significant difference regarding the surgical procedure time was observed in the 2 groups, with a higher time in the group A (Group A: 180 ± 77.3; Group B: 138 ± 54.5, P= 0.04). BL was significantly lower in group B (203 ± 69.5 mL vs. 356 ± 102 mL; P = 0.0001), as well as for patients underwent PAEPs vs. those underwent direct surgery (n = 15, 149 ± 53 mL vs. n = 42, 273 ± 88 mL; P = 0.0001). No differences between transient and persistent cranial nerve injuries were observed between the 2 groups. Carotid reconstruction was necessary for 2 patients of group A (n = 2 vs. n = 0; P = 0.02). Unilateral tumor recurrence was detected in 7 patients, with a significantly higher rate (P ≤ 0.002) in patients carrying SDH mutations compared to those without SDH mutation (wild-type). SDH mutations detected in the groups lead to discover 7 NSMs (group A n = 1 vs. group B n = 6; P = 1.00). CONCLUSION: The impact of the multidisciplinary team suggests that surgical resection still remains the gold standard for the treatment of CBTs, but the use of PAEPs in selected cases may reduce surgical procedure time, BL and the need for reconstructive carotid surgery. Genetic counseling and SDH gene analysis allow to diagnose NSMs in asymptomatic patients. Larger studies should be considered to evaluate the effectiveness of postoperative rehabilitative program.

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.

[Surgical treatment of a huge paraganglioma of the right common carotid artery bifurcation]. / Khirurgicheskoe lechenie krupnoi paragangliomy bifurkatsii pravoi obshchei sonnoi arterii.

Described herein is a clinical case report regarding successful surgical treatment of a female patient presenting with a large paraganglioma of the right common carotid artery. On admission, the woman had complained of a mass in her neck, having significantly enlarged within the previous 6 months, with the appearance of dysphagia and moderate pain syndrome. The findings of multislice computed angiography and ultrasonographic duplex angioscanning of the brachiocephalic arteries helped to verify the location, size, and topography of the tumour. Taking into account the diagnosed secondary foci in the lungs, it was decided to first perform embolization of the artery supplying the tumour, which was followed by biopsy of tissue of the neoplasm. After histological verification and ruling out malignancy, successful radical resection of the paraganglioma was performed.

Simple and complex carotid paragangliomas. Three decades of experience and literature review.

BACKGROUND: Carotid paragangliomas are rare tumors. They are usually unique, non-secreting, resectable, and benign. However, additional rare cases of complex tumors (bilateral, secretory, nonresectable, or malignant) complicate the management and final outcomes. METHODS: Records of paragangliomas from our hospital are reviewed. Criteria defining complex paragangliomas have been previously defined. These are compared with those of the simple group. RESULTS: Fifty patients, two groups: simple (n = 39) and complex (n = 11). The patients in the complex group were significantly younger (47.7 vs 63.8 years). Postoperative nerve complications (45.4% vs 6.3%) and mortality during follow-up (27.3% vs 0%) were significantly more common in the complex group. Vascular complications (0% vs 3.1%) and early mortality (0%) were similarly in both groups. CONCLUSIONS: Patients with complex carotid paragangliomas are heterogeneous. The former are younger, exhibit a high degree of diagnostic and therapeutic complexity, and have poorer morbidity and mortality. Surgical experience and interdisciplinary collaboration are essential.

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.

Ancient schwannoma mimicking a carotid body tumor A case report and review of the literature.

Ancient Schwannoma is a type of peripheral neurogenic tumor formed by the Schwann cells presenting mainly as a benign and asymptomatic lesion. The neurilemmoma tumor appears in different sites and, in cases of cervical location, can mimic a carotid body tumor. Herein we describe a clinical case of a 51-year-old woman with latero-cervical swelling. A contrast-enhanced computed tomography scan revealed a classical wineglass image mimicking a carotid body tumor. During surgery the tumor appeared connected to the cervical sympathetic trunk without carotid involvement. The histological exam confirmed the nature of the mass which consisted of an ancient schwannoma. A subsequent systematic review of the literature on ancient schwannoma incidence and treatment confirms it being a benign and rare lesion primarily treatable with open surgery. KEY WORDS: Ancient Schwannoma, Carotid body tumor, Carotid artery, Vascular surgery.

Cirurgia híbrida na exérese de tumor glômico Shamblin II / Hybrid surgery in excision of a Shamblin II glomus tumor

O tumor glômico é uma neoplasia benigna rara originada de células paraganglionares da crista neural que se desenvolve na camada adventícia do vaso. São tumores não encapsulados e altamente vascularizados. Paciente feminina, 64 anos, foi diagnosticada com tumor glômico hipervascularizado com 5 cm posteriormente à bifurcação carotídea esquerda e oclusão de carótida contralateral. Optou-se por realizar embolização através de acesso endovascular seguida de punção percutânea direta, guiada por angiografia, para preenchimento da área remanescente. Após embolização, realizou-se a exérese cirúrgica do tumor com menor sangramento e maior facilidade para encontrar o plano de clivagem das estruturas adjacentes. Em acompanhamento tardio, a paciente apresenta-se sem recidiva tumoral. O tumor foi classificado como pertencente ao grupo Shamblin II, o qual inclui tumores apresentando de 4 a 6 cm com inserção arterial moderada. Através dessa dupla abordagem, foi possível notar uma redução relativa do sangramento intraoperatório e facilitação de identificação do plano de clivagem, colaborando para sua exérese e evitando o pinçamento cirúrgico

Glomus tumors are rare benign neoplasms originating from paraganglionic cells of the neural crest developing in the adventitious layer of the vessel. They are nonencapsulated and highly vascularized. A 64-year-old female patient was identified with a hypervascularized glomus tumor measuring 5 cm, posterior to the left carotid bifurcation and contralateral carotid occlusion. We performed preoperative embolization via endovascular access followed by direct percutaneous puncture, guided by angiography, to fill the remaining area. After embolization, surgical excision of the tumor was performed with reduced bleeding and it was easier to find the cleavage planes to adjacent structures. At late follow-up, the patient is free from tumor recurrence. The tumor was classified as Shamblin II, measuring 4 to 6 cm with moderate arterial insertion. Through this double approach we observed a relative reduction in intraoperative bleeding and improved identification of the cleavage plane, facilitating excision and avoiding surgical clamping