Prevalence, characteristics, evaluation, and management of carotid body tumors: Systematic analysis based on available evidence.

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

Epidemiology and survival of patients with malignant carotid body tumors in the SEER database.

OBJECTIVE: The objective of this study was to investigate population-based epidemiology, survival outcomes, and prognostic factors of malignant carotid body tumors (CBTs). METHODS: Patients with malignant CBTs who were diagnosed between 1975 and 2018 were screened from nine registries of the Surveillance, Epidemiology, and End Results (SEER) database. Cases that were coded as "carotid body tumor, malignant" or malignant tumors with the primary site recorded as "carotid body" were screened for inclusion in the study. The incidence of malignant CBT was calculated with SEER∗Stat software. Survival outcomes were analyzed using the Kaplan-Meier method and log-rank tests. RESULTS: A total of 72 patients with malignant CBT were screened for inclusion in the study, including 41 females (56.9%) and 31 males (43.1%). Based on the SEER program data, the incidence of malignant CBT was found to fluctuate between 0 to 0.02 cases per 100,000 people per year, with a slow but noticeable uptick after 1990. The most commonly affected populations included women and patients between the ages of 35 and 44, which accounted for 59.9% and 27.8% of patients in the study, respectively. During a median follow-up of 82 months, four patients were lost to follow-up, and 28 deaths were identified. Of those, 20 were considered disease-specific deaths. Further analysis found that the 5-year and 10-year overall survival rates were 78.9% and 67.8%, respectively, whereas the 5-year and 10-year disease-specific survival rates were 84.5% and 75.2%, respectively. The Kaplan-Meier method and log-rank tests indicated that age <50 years, sex, race, tumor number, and surgical treatment were unrelated to both overall survival and disease-specific survival. CONCLUSIONS: A retrospective review of the SEER database found that the incidence of malignant CBT was extremely rare and prone to fluctuation, but that it slowly trended upward over time. Malignant CBT was found to more likely affect females, and it could be diagnosed at any age. The overall prognosis for malignant CBT appeared to be good, with acceptable 5-year and 10-year survival rates. Due to a number of factors complicating malignant CBT surgery, surgical treatment should be considered with caution.

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.

SDHD gene mutation in Mexican population with carotid body tumor.

INTRODUCTION: Among the U.S. population, the p81L SDHD (11q23) gene mutation is present in 6-36% of patients with sporadic carotid body tumor (CBT), but in familial cases is high as 80%. That is why the P81L mutation is used as a screening method for carotid body tumor in the U.S. METHODS: We included 25 patients who underwent resection of a CBT from January 2010 to June 2015. After informed consent, a blood sample was taken for genetic testing on real-time polymerase chain reaction, in order to identify p81L mutation in the SDHD gene. The information was analyzed with descriptive statistics, using central tendency and description measures. RESULTS: In our group, 92% were females, a mean age of 55.5 years, and 52% were Shamblin type II. The most common place of residence was Mexico City, 8% of the patients had family history, about 20% of the patients had a contralateral tumor and 16% had antecedent of another kind of tumor, 4 (16%) p81L SDHD gene mutations were detected, all of them were heterozygous. CONCLUSIONS: The p81L mutation in the SDHD gene was found in the Mexican population in higher grade that in the U.S. population, which explain the high incidence of this pathology in our country, but we need more studies about this subject.

INTRODUCCIÓN: La mutación p81L del gen SDHD (11q23) se encuentra presente en el 6-36% de los pacientes con tumores del cuerpo carotídeo (TCC) esporádicos y hasta en el 80% de los que presentan TCC familiares. En los EE.UU. se usa como método de cribado para TCC. MÉTODO: Se incluyeron 25 pacientes consecutivos operados de resección de TCC entre enero de 2010 y junio de 2015. Se les tomó muestra sanguínea venosa que se sometió a reacción en cadena de la polimerasa en tiempo real para identificar la mutación p81L del gen SDHD (11q23). La información se analizó con estadística descriptiva mediante medidas de tendencia central y dispersión. RESULTADOS: Del grupo en estudio, el 92% eran mujeres, la edad promedio era de 55.5 años y el 52% tenían tumor Shamblin tipo II. El lugar de residencia más frecuente fue la Ciudad de México. El 8% presentaban antecedentes familiares, el 20% tumor bilateral y el 16% presentaron un tumor en otra región. Se encontró la mutación p81L del gen SDHD (11q23) en el 16% de los pacientes de forma heterocigota. CONCLUSIONES: La mutación p81L del gen SDHD se encuentra presente en la población mexicana en un grado más elevado que lo reportado en los EE.UU., lo que podría explicar la alta incidencia en nuestro medio.

Surgical Management of Bilateral Carotid Body Tumors.

BACKGROUND: Carotid body tumors (CBTs) are rare neoplasms located in the carotid bifurcation. The majority of these tumors are unilateral; bilateral CBTs represent approximately 5% of all affected patients, and the recommended treatment is to surgically remove them in staged-planned surgeries. We describe the experience, outcomes, and the surgical management of bilateral CBTs in our institution. METHODS: A retrospective review of CBTs patients was completed; patient demographics, comorbidities, lesion location, anatomic characteristics, surgical techniques, complications, reinterventions, and other factors that may influence outcomes were evaluated. RESULTS: A total of 109 patients with CBTs were treated surgically; of these, 8 had bilateral CBTs (7%); the mean age was 56 years, and 7 (87%) were females. Thirteen surgical resections were performed, and in 2 of the cases, the pathology report was malignant (15%). Five were classified as Shamblin I (31%), 5 as Shamblin II (31%), and remaining 6 as Shamblin III (38%). The mean time between the first and second procedure was of 10.7 months. Complications included one case of neck hematoma requiring evacuation and postoperative neurologic complications occurred in three patients (one patient with facial and two with vocal cord palsies). None of the studied individuals had a family history of CBT, and all of them lived in altitude areas higher than 2000 meters above mean sea level (mamsl). The mean tumor size was 3.55 cm and 2.75 cm for right and left CBTs, respectively. CONCLUSIONS: A better understanding of the clinical characteristics of patients with bilateral CBTs may lead to a more standardized and optimal management with fewer complications and a better quality of life afterward.

SDHD gene mutation in Mexican population whit carotid body tumor. / Mutación del gen SDHD en población mexicana con tumor del cuerpo carotídeo

Introduction: Among the U.S. population, the p81L SDHD (11q23) gene mutation is present in 6-36% of patients with sporadic carotid body tumor (CBT), but in familial cases is high as 80%. That is why the P81L mutation is used as a screening method for carotid body tumor in the U.S. Methods: We included 25 patients who underwent resection of a CBT from January 2010 to June 2015. After informed consent, a blood sample was taken for genetic testing on real-time polymerase chain reaction, in order to identify p81L mutation in the SDHD gene. The information was analyzed with descriptive statistics, using central tendency and description measures. Results: In our group, 92% were females, a mean age of 55.5 years, and 52% were Shamblin type II. The most common place of residence was Mexico City, 8% of the patients had family history, about 20% of the patients had a contralateral tumor and 16% had antecedent of another kind of tumor, 4 (16%) p81L SDHD gene mutations were detected, all of them were heterozygous. Conclusions: The p81L mutation in the SDHD gene was found in the Mexican population in higher grade that in the U.S. population, which explain the high incidence of this pathology in our country, but we need more studies about this subject.

Introducción: La mutación p81L del gen SDHD (11q23) se encuentra presente en el 6-36% de los pacientes con tumores del cuerpo carotídeo (TCC) esporádicos y hasta en el 80% de los que presentan TCC familiares. En los EE.UU. se usa como método de cribado para TCC. Método: Se incluyeron 25 pacientes consecutivos operados de resección de TCC entre enero de 2010 y junio de 2015. Se les tomó muestra sanguínea venosa que se sometió a reacción en cadena de la polimerasa en tiempo real para identificar la mutación p81L del gen SDHD (11q23). La información se analizó con estadística descriptiva mediante medidas de tendencia central y dispersión. Resultados: Del grupo en estudio, el 92% eran mujeres, la edad promedio era de 55.5 años y el 52% tenían tumor Shamblin tipo II. El lugar de residencia más frecuente fue la Ciudad de México. El 8% presentaban antecedentes familiares, el 20% tumor bilateral y el 16% presentaron un tumor en otra región. Se encontró la mutación p81L del gen SDHD (11q23) en el 16% de los pacientes de forma heterocigota. Conclusiones: La mutación p81L del gen SDHD se encuentra presente en la población mexicana en un grado más elevado que lo reportado en los EE.UU., lo que podría explicar la alta incidencia en nuestro medio.

Mean Platelet Volume, Red Cell Distribution Width, and Neutrophil-to-Lymphocyte Ratio Before and After Surgery in Patients With Carotid Body Tumors.

OBJECTIVE: Carotid body tumors (CBTs) are rare hypervascular benign tumors that originate from the paraganglia at the carotid bifurcation. The red cell distribution width (RDW), neutrophil-to-lymphocyte ratio (NLR), and mean platelet volume (MPV) may serve as markers in inflammatory, cardiovascular, and neoplastic diseases. However, their prognostic value in CBTs is unknown. The purpose of this study was to assess the leukocyte count, MPV, RDW, and NLR before and after surgery in patients with CBTs. METHODS: This retrospective trial included data from 80 patients with CBTs and 65 age-matched healthy controls. The leukocyte, neutrophil, lymphocyte and platelet counts, RDW, and MPV were extracted and NLR was calculated from the complete blood count results of participants. RESULTS: The leukocyte and neutrophil counts as well as RDW levels were significantly increased in patients with CBTs compared with healthy controls (P = 0.005, P = 0.003, and P = 0.026; respectively). Patients with CBTs had lower lymphocytes counts (P = 0.241) and higher NLRs (P = 0.054); however, the difference was statistically insignificant. Moreover, no statistically significant difference was detected between groups in terms of platelet counts and MPV levels. Furthermore, the leukocyte count, platelet numbers, MPV, RDW, and NLR levels in patients with CBTs were not statistically significant after surgery (all; P > 0.05). CONCLUSION: This is the first study that documents the increased RDW levels and leukocyte count in patients with CBTs. The combined use of RDW and the leukocyte count along with other clinical assessments can be used as a biomarker for CBTs. Further clinical trials with larger cases series are required to determine the actual predictive roles of these systemic biomarkers.

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.

Tumor del cuerpo carotídeo. A propósito de un caso / Tumor of the carotid body. Concerning a case

Los tumores del cuerpo carotídeo son relativamente infrecuentes y raramente se diagnostican antes de la exposición quirúrgica, su diagnóstico es benigno, pero suelen ser muy vasculares por lo que su extirpación quirúrgica muchas veces resulta difícil. Estos tumores son de crecimiento lento y pueden evolucionar durante años. El objetivo de este estudio es presentar un caso con un tumor del cuerpo carotídeo en región lateral derecha del cuello. Clínicamente se observó un aumento de volumen de aproximadamente 4 cm, no doloroso a la palpación, de tipo gomoso y adherido a planos profundos, asintomático, tratado en nuestra institución durante el año 2014. Se le realizó exéresis simple de la lesión, y el departamento de Anatomía Patológica reportó el diagnóstico de referencia. La evolución de la paciente después de 17 meses ha sido satisfactoria (AU).

The tumors of the carotid body are relatively uncommon and rarely diagnosed before the surgical exeresis, their diagnosis is benign, but they are usually very vascular and its extirpation is very difficult. These tumors have a slow growth and it can evolve during years. The aim of this study is to present a case with a tumor of the carotid body in the right lateral region of the neck. An increase of volume was observed of approximately 4 cm, not painful, of gummy type and stuck to deep, asymptomatic plans, treaty in our institution during the year 2014. It was carried out exeresis of the lesion, and the department of Pathological Anatomy reported the reference diagnosis. The patient's evolution after 17 months has been satisfactory (AU).

Tumor del cuerpo carotídeo. A propósito de un caso / Tumor of the carotid body.Concerning a case

Los tumores del cuerpo carotídeo son relativamente infrecuentes y raramente se diagnostican antes de la exposición quirúrgica, su diagnóstico es benigno, pero suelen ser muy vasculares por lo que su extirpación quirúrgica muchas veces resulta difícil. Estos tumores son de crecimiento lento y pueden evolucionar durante años. El objetivo de este estudio es presentar un caso con un tumor del cuerpo carotídeo en región lateral derecha del cuello. Clínicamente se observó un aumento de volumen de aproximadamente 4 cm, no doloroso a la palpación, de tipo gomoso y adherido a planos profundos, asintomático, tratado en nuestra institución durante el año 2014. Se le realizó exéresis simple de la lesión, y el departamento de Anatomía Patológica reportó el diagnóstico de referencia. La evolución de la paciente después de 17 meses ha sido satisfactoria (AU).

The tumors of the carotid body are relatively uncommon and rarely diagnosed before the surgical exeresis, their diagnosis is benign, but they are usually very vascular and its extirpation is very difficult. These tumors have a slow growth and it can evolve during years. The aim of this study is to present a case with a tumor of the carotid body in the right lateral region of the neck. An increase of volume was observed of approximately 4 cm, not painful, of gummy type and stuck to deep, asymptomatic plans, treaty in our institution during the year 2014. It was carried out exeresis of the lesion, and the department of Pathological Anatomy reported the reference diagnosis. The patient's evolution after 17 months has been satisfactory (AU).

Carotid Body Tumor: Clinical Features.

Although carotid body tumor (CBT) is a rare neoplasm, it should always be considered in differential diagnosis of lateral neck masses. We shared the 10 years of CBT experience in our clinic and started a discussion on CBT with literature support. A total of 21 patients with CBT diagnosis in Firat University Hospital, Otorhinolaryngology Clinic, participated in the study. Patients were evaluated based on demographical data and particularities of the tumor. Participant patients were 19 women and 2 men, and their ages were between 21 and 79 (mean age 54.06 ± 12.48). The most frequent reason for the patients to apply to the hospital was painless swelling in the neck (76.2%). Tumor was located in the right side of the neck in 10 patients (47.6%), and in the left side of the neck in 11 (52.4%). Twenty patients (95.2%) had undergone computerized tomography angiography. Surgical treatment was applied to 19 patients (90.5%) and the tumor was totally excised. According to Shamblin classification, 15 of the tumors of these patients were class II (78.9%) and 4 were class III (21.1%). In 1 patient (5.3%), postoperative contusion infection that recovered after medical treatment was observed; in 2 patients (10.5%), n. vagus injury was observed because of tumor's pervasion of n. vagus; and in 1 of these patients vocal cord paralysis was developed and this patient was later taken into thyroplasty surgery. Two patients (10.5%) suffered n. hypoglossus injury, 1 of these recovered within 3 months postoperative and the other developed n. hypoglossus palsy. The size and extension of the tumor should be determined by preoperative imaging for the correct planning of surgical procedure. It should be taken into consideration that despite advanced surgical techniques, the rate of postoperative cranial nerve damage is still high.

[Carotid body paraganglioma in a teenager. Case report]. / Paraganglioma del cuerpo carotídeo en adolescente. Reporte de caso.

BACKGROUND: Paragangliomas of the head and neck are neuroendocrine tumors. They have a low incidence (0.6%), are generally benign, have a poorly defined etiology, and multiple factors have been associated with their origin. Humans and other species living at high altitudes (>2000 m above sea level) are subjected to a relatively chronic hypoxia and there is a high prevalence of the development of carotid body hyperplasia and eventually paragangliomas. This disease is usually seen in patients in their 50s and in their 30s if there is a family history. CLINICAL CASE: We present the case of a 16 year-old female with acute pharyngitis and growing tumor located on the left side of the neck, without symptoms. A duplex Doppler ultrasound showed a solid nodular lesion on the left carotid bifurcation. A left lateral cervicotomy was performed, finding a highly vascularized tumor of 4 × 3 × 3 cm involving the common carotid from its middle third, the internal carotid up to the cranial base, and the external carotid to its upper third, and intimately related to the trachea, esophagus and cervical spine. The tumor was completely resected and the histopathological analysis corroborated the presence of paragangliomas. CONCLUSIONS: The publication of this case is relevant and of clinical interest due to the uncommon age of presentation and the fact that it should be considered as a diagnostic possibility.

Antecedentes: los paragangliomas de cabeza y cuello son tumores neuroendocrinos de baja incidencia (0.6%), en su mayor parte benignos, en cuyo origen se han involucrado múltiples factores. Los seres humanos y otras especies que viven a grandes alturas (por arriba de 2000 metros sobre el nivel del mar) son propensos a sufrir hipoxia crónica relativa, hiperplasia del cuerpo carotídeo y, eventualmente, paragangliomas. Este padecimiento aparece en la quinta década de la vida y en la tercera, en caso de presentación familiar. Caso clínico: se comunica el caso de una adolescente de 16 años de edad que un año antes tuvo faringitis aguda con tumor localizado en la cara lateral izquierda del cuello, de crecimiento gradual, sin ningún síntoma. El ultrasonido Doppler dúplex de cuello mostró una lesión nodular sólida sobre el trayecto de la bifurcación carotídea izquierda. Fue intervenida quirúrgicamente mediante cervicotomía lateral izquierda, en la cual se encontró una tumoración de aproximadamente 4 × 3 × 3 cm, sumamente vascularizada, que afectaba la carótida común desde su tercio medio, la carótida interna hasta la base del cráneo y la carótida externa hasta el tercio superior, con íntima relación con la tráquea, el esófago y la columna cervical. La tumoración se resecó en su totalidad, el estudio histopatológico corroboró los paragangliomas. Conclusiones: la edad de presentación es poco común, por lo que se considera relevante y de interés clínico comunicar este caso, toda vez que debe considerarse como posibilidad diagnóstica.

Carotid body tumors are not associated with an increased risk for sleep-disordered breathing.

PURPOSE: Tumors in the carotid bodies may interfere with their function as peripheral chemoreceptors. An altered control of ventilation may predispose to sleep-disordered breathing. This study aimed to assess whether patients with unilateral or bilateral carotid body tumors (uCBT or bCBT, respectively) or bilateral CBT resection (bCBR) display sleep-disordered breathing and to evaluate the global contribution of the peripheral chemoreceptor to the hypercapnic ventilatory response. METHODS: Eight uCBT, eight bCBT, and nine bCBR patients and matched controls underwent polysomnography. The peripheral chemoreflex drive was assessed using euoxic and hyperoxic CO2 rebreathing tests. Daytime sleepiness and fatigue were assessed with the Epworth Sleepiness Scale and the Multidimensional Fatigue Index. RESULTS: All patient groups reported significant fatigue-related complaints, but no differences in excessive daytime sleepiness (EDS) were found. The apnea/hypopnea index (AHI) did not differ significantly between patient groups and controls. Only in bCBT patients, a trend towards a higher AHI was observed, but this did not reach significance (p=0.06). No differences in the peripheral chemoreflex drive were found between patients and controls. CONCLUSIONS: Patients with (resection of) CBTs have more complaints of fatigue but are not at risk for EDS. The presence or resection of CBTs is neither associated with an altered peripheral chemoreflex drive nor with sleep-disordered breathing.

Tumores del cuerpo carotídeo. Experiencia en 22 años y protocolo de seguimiento y despistaje familiar / Carotid body tumours. Experience over 22 years and a family screening and follow-up protocol

Introducción: Los tumores del cuerpo carotídeo son tumores raros, muy vascularizados, originados en los quimiorreceptores de la bifurcación carotídea. Se ha descrito su malignización y asociación con otros tumores endocrinos. Pueden ser familiares en el 10-50% de los casos. Objetivo: Analizar nuestros resultados en el manejo de estos tumores y presentar un protocolo de actuación y seguimiento. Material y métodos: Entre 1986 y 2008 se trataron 25 casos en 23 pacientes (2 bilaterales), con una edad media de 51 años (r 31-78), el 82,6% mujeres, un caso familiar. Como pruebas diagnósticas se realizaron ecodoppler y arteriografía en todos los pacientes, y tomografía computarizada o resonancia magnética en 12. El seguimiento postoperatorio se llevo a cabo con ecodoppler. Resultados: En todos los casos se realizó la resección completa del tumor, los 14 últimos con embolización preoperatoria. Fue necesaria la reconstrucción vascular en 6 casos. Seis pacientes tuvieron lesión neurológica, uno rotura arterial y uno neumonía postoperatoria. Según los criterios de clasificación de Shamblin, 13 casos fueron tipo I, 5 tipo II y 7 tipo III. Las complicaciones y reconstrucciones vasculares estuvieron relacionadas con tumores tipo II y III. Durante el seguimiento se detectaron 4 recidivas, 2 tumores contralaterales (bilaterales), un feocromocitoma, una trombosis del injerto y 6 éxitus (uno por malignización y metástasis). Conclusiones: La cirugía en fase precoz disminuye la morbimortalidad. El seguimiento con ecodoppler permite detectar recidivas, bilateralidad y complicaciones de la reconstrucción vascular. La posible presentación familiar, recidivas, asociación a otros tumores o metástasis ,hacen necesario el seguimiento sistematizado del paciente(AU)

Background: Carotid body tumours are rare, highly vascular, arising from the chemoreceptors of the carotid bifurcation. Their relationship with malignancy and other endocrine tumours have already been described. There can be between 10% and 50% of cases in a family. Objective: To analyse the results in the management of these tumours, and present a protocol for action and monitoring. Material and methods: Between 1986 and 2008 a total 25 cases were treated in 23 patients (2 bilateral) with a mean age of 51 years (range 31-78), with 82.6% women, 1 family case. Doppler ultrasound and angiography were performed as diagnostic tests in all patients, with computed tomography or magnetic resonance imaging in 12 cases. They were monitored annually using Doppler ultrasound. Results: According to the Shamblin classification criteria, 13 cases were type I, 5 were type II and 7 type III. A complete resection of the tumour was performed in all cases, the last 14with preoperative embolisation. Vascular reconstruction was necessary in 6 cases. Six patients had neurological injury, an arterial rupture and a postoperative pneumonia. Complications and vascular reconstruction were associated with type II and III tumours. During follow-up four recurrences were detected, two contralateral tumours, a phaeochromocytoma, a graft thrombosis and six deaths, (1 from malignancy and metastasis). Conclusions: Surgery in the early stage reduces the morbidity and mortality. Follow-up should be with Doppler ultrasound to detect recurrence, bilaterality and complications of vascular reconstruction. The possible familial presentation, recurrence, association with other tumours or metastases, requires systematic monitoring of the patient(AU)

Carotid body tumours.

Carotid body tumours are a rare class of paraganglionoma arising from the upper neck, but should be considered in the differential diagnosis of neck lumps. A wrong diagnosis of cervical lymphadenopathy followed by excision biopsy may have serious consequences. The only way to minimize such risk is to be aware of their existence.

A 22-year Northern Irish experience of carotid body tumours.

OBJECTIVES: Carotid body tumours (CBTs) are rare vascular neoplasms originating in paraganglionic cells of the carotid bifurcation. The aim of this study was to review all patients diagnosed with CBTs in Northern Ireland. METHODS: A retrospective review was performed of all patients who had CBTs treated at our institutions between 1987 and 2009. Patient demographics, clinical symptomatology, investigative modality, therapeutic intervention, pathological analysis and long-term outcomes were assessed. RESULTS: Twenty-nine patients were identified with 33 CBTs and three glomus intravagale tumours (GITs). Six patients had bilateral CBTs (21%), one of whom had a synchronous GIT. Twenty-six patients underwent a total of 30 operative procedures for the resection of 28 CBTs and 3 GITs. Conventional operative treatment included subadventitial tumour excision. A vascular shunt facilitated arterial reconstruction following the removal of seven (23%) tumours and on six of these occasions (19%) continuity was restored with an interposition vein graft. For access the external carotid artery was ligated during the removal of four tumours (13%). Two tumours were considered malignant. No peri-operative mortalities were recorded. Immediate complications included peri-operative stroke secondary to an occluded vein graft (n=1), requirement of tracheostomy (n=2), emergency haematoma drainage (n=2) and transient cranial nerve damage (n=8). Late complications included pseudoaneurysm of vein graft with subsequent stoke (n=1), permanent cranial nerve damage (n=9), Horner's syndrome (n=1) and an asymptomatic vein graft occlusion (n=1). One patient had tumour recurrence two years post-operatively and died due to pulmonary metastases. Two other patients died of unrelated causes. All other patients remain well with no evidence of tumour recurrence at mean followup of 1801 days (range 159-9208 days). CONCLUSION: Our long-term experience is comparable with other reported case series where surgical intervention conferred a long-term survival advantage despite associated cranial nerve co-morbidities.

A multicenter review of carotid body tumour management.

OBJECTIVE: Carotid body tumour (CBT) is a rare but the most common form of head and neck paraganglioma (PGL). We present the biggest ever series on CBT in UK/EU discussing diagnostic challenges, surgical treatment and complications of surgical intervention. METHOD: A detailed proforma was designed and sent to all members of Joint Vascular Research Group (JVRG). Data of 95 patients was collected. Generic terms including carotid body tumour/s, or paraganglioma/s were used to search a variety of electronic database in order to get latest informations available in literature. RESULTS: A total of 95 patients were recorded in our data from 1979 to 2005. Mean age of presentation was 55 years. Incidence was higher in females. CBT was more common on right side (58%). 18% tumours were bilateral. Neck lump (98%) and pressure symptoms including cranial nerve deficits and pain were main presenting complaints. About 18% of tumours were familial. Only 4.2% were malignant. Duplex scan is the best investigation for diagnosis, though MRI, DSA and CT scan are important for preoperative assessment. Surgery is the treatment of choice. Stroke and cranial nerve injury constitute postoperative morbidity (35%) and mortality (1%). Incidence of postoperative cranial nerve deficit was about 19%. Combined ipsilateral and contralateral recurrence rate was 4.2%. CONCLUSION: CBT is a rare condition which needs surgical excision by experienced vascular surgeon. Surgical resection is associated with significant morbidity of 35% and mortality of 1%. Mostly CBT is benign but malignant forms are not uncommon.

Management of carotid body tumors.

The carotid body tumor is a rare neoplasm that has generated much literature over the past century, and for which continued controversy exists regarding natural history, biologic behavior, proper technique of excision, and the risk of morbidity and mortality. This article discusses overall management of carotid body tumors.

Changing trends in management of carotid body tumors.

UNLABELLED: The purpose of this retrospective study was to evaluate the preoperative diagnostic modalities and aggressive operating management as well as the determination of benefit of preop embolization on the course of carotid body tumor (CBT). Between 1975 and 1993, 14 patients were treated for CBT in our department. The preoperative evaluation included angiography (14/14, 100%), Duplex scanning (6/14, 43%), and CT scanning (9/14, 64%). Five tumors (36%) were type I, four (28%) type II, and five (36%) type III, according to the Shamblin classification. In twelve cases the tumors were successfully excised without ligation of the external carotid artery. One of these patients, who had a very large tumor, underwent preop embolization. In two patients the tumors were found to extend intracranially, and the patients were referred for radiation therapy. There were no deaths or cerebrovascular complications. Temporary cranial nerve injury was noted in two cases. The pathology report revealed paragangliomas without any evidence of malignancy. Follow up of patients (6 months-18 years) showed no recurrence of the tumors in the operated cases and local recurrence in the radiation group. CONCLUSION: 1. Arteriography remains the golden standard for diagnosis of CBT. Tripplex scanning and CT are helpful for postop follow-up. 2. Surgical removal is the ideal treatment. Preoperative embolization is helpful in diminishing intraoperative blood requirements. 3. CBT, although benign, should be treated as soon as it is diagnosed, before difficulty in excision arises.