Tumor de cuerpo carotídeo en adolescente. Reporte de un caso / Carotid body tumor in an adolescent. A case report

Introducción. Los paragangliomas del cuerpo carotídeo son neoplasias infrecuentes y representan el 0,6 % de los tumores de cabeza y cuello. La edad de presentación promedio es la quinta década de vida. El objetivo de este artículo fue describir un caso de paraganglioma del cuerpo carotídeo en una adolescente. Caso clínico. Se presenta el caso de una paciente adolescente con paraganglioma de cuerpo carotídeo derecho, de 5x3x3 cm, Shamblin III. Se analizaron la historia clínica, los exámenes diagnósticos, la técnica quirúrgica utilizada y su evolución correspondiente. La información fue obtenida a partir de la ficha clínica, previa autorización por consentimiento de los padres de la paciente. Resultados. Se hizo resección quirúrgica completa y reparo vascular con injerto de vena safena invertida, con evolución favorable y permeabilidad completa del puente vascular, sin secuelas. Conclusión. Este caso podía corresponder a un tumor de etiología familiar, dada su edad temprana de presentación. Se hizo necesario complementar su estudio con imágenes y objetivar el compromiso vascular asociado para la planificación quirúrgica. En estos pacientes, la complejidad de su localización y el compromiso vascular del tumor requiere de un equipo multidisciplinario, con cirujanos de cabeza y cuello y cirujanos vasculares para un resultado exitoso.

Introduction. Carotid body paragangliomas are rare, representing 0.6% of head and neck tumors, with average age of presentation in the fifth decade of life. The objective of this article is to describe a clinical case of carotid body paraganglioma in an adolescent. Clinical case. Review and analysis of the clinical case, reviewing its clinical history, study tests, surgical technique used and its corresponding evolution. Information obtained from the clinical record prior authorization by consent of the patient's parents. Results. Adolescent patient with paraganglioma of the right carotid body, 5x3x3 cm, Shamblin III. With complete surgical resection and inverted saphenous vein graft, favorable evolution, with complete permeability of the vascular bridge, without sequelae. Conclusion. This case could correspond to a tumor of familiar etiology, given its early age of presentation. It is necessary to complement the study with images and to objectively determine the associated vascular involvement for surgical planning. In these patients, the complexity of their location and vascular involvement of the tumor requires a multidisciplinary team with head and neck and vascular surgeons for a successful outcome.

Paraganglioma gigante del espacio parafaríngeo en una fémina / Giant paraganglioma of the parapharingeal space in a woman: en

Se describe el caso clínico de una paciente de 57 años de edad, con antecedente de hipertensión arterial, quien fue asistida en el Servicio de Cirugía de Cabeza y Cuello del Instituto Nacional de Oncología y Radiobiología de La Habana, remitida de la consulta de Otorrinolaringología de su hospital de cabecera por presentar manifestaciones clínicas de disfagia y diagnóstico clínico de sospecha de un paraganglioma carotídeo. Luego de realizados el examen físico y los estudios complementarios pertinentes, el caso fue discutido por los integrantes de un equipo multidisciplinario y se confirmó la existencia de un paraganglioma del espacio parafaríngeo, por lo que se decidió realizar tratamiento quirúrgico. La evolución fue satisfactoria y a los 2 años del procedimiento quirúrgico no presentaba secuelas.

The case report of a 57 years patient with history of hypertension is described who was assisted in the Head and Neck Surgery Service of the National Institute of Oncology and Radiobiology in Havana, referred from the Otolaryngology Service of her head hospital due to clinical manifestations of dysphagia and suspected clinical diagnosis of a carotid paraganglioma. After the physical exam and the pertinent complementary studies, the case was discussed by the members of a multidisciplinary team and the existence of a paraganglioma in the parapharingeal space was confirmed, therefore it was decided to carry out surgical treatment. The clinical course was satisfactory, and 2 years after the surgical procedure there were no sequels.

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.

Paragangliomas cervicais: experiência de 114 casos em 14 anos / Cervical paragangliomas: experience of 114 cases in 14 years

Resumo Introdução e objetivo: Relatar a experiência de um único centro com casos de paraganglioma do corpo carotídeo tratados pelo mesmo cirurgião em uma cidade com alta prevalência de paragangliomas devido à alta altitude. Método: Foram investigados retrospectivamente os dados demográficos, clinico-patológicos e radiológicos de 104 pacientes com diagnóstico de paragangliomas cervicais entre 2003 e 2017. Os pacientes foram classificados de acordo com a classificação de Shamblin. Resultados: Neste estudo, foram incluídos 104 pacientes (33 homens e 71 mulheres, com média de 54,6 ± 13 anos, entre 2003 e 2017) com diagnóstico de paraganglioma cervical na bifurcação carotídea. Entre esses pacientes, 10 tinham tumores bilaterais e, no total, 114 paragangliomas foram tratados nesse período. O diâmetro médio dos tumores foi de 5,12 ± 1,45 cm. Um tumor maligno foi determinado em apenas um (0,9%) paciente. Todos os pacientes foram operados. Em 12 pacientes com diâmetro do tumor maior do que 5 cm, foi possível fazer embolização pré-operatória com molas; em 14 pacientes, foi feita embolização angiográfica e em 4 pacientes foram aplicadas injeções de agentes esclerosantes. Após o tratamento cirúrgico, paralisia facial foi observada em 2 pacientes, disfagia em um, síndrome de Horner em um e rouquidão em 7. Todas essas complicações melhoraram durante o acompanhamento. Não foi relatada mortalidade. Conclusão: A cirurgia é o tratamento definitivo em pacientes com paragangliomas cervicais. Embora possa ser difícil em pacientes com os tipos avançados de Shamblin, em mãos experientes as taxas de complicações são muito baixas.

Risk Factors for Patients Using Intraoperative Vasopressor Infusions During Carotid Body Tumor Excision / 中国医学科学院学报

Objective To investigate the risk factors for patients using intraoperative vasopressor infusions during carotid body tumor(CBT)excision.Patients' mean arterial pressure(MAP)and heart rate(HR)fluctuations as well as their requirements for vasoactive agents during surgery were assessed. Methods The patients receiving CBT excision in Peking Union Medical College Hospital from May 1,2013 to July 31,2017 were included for a retrospective cohort study.The potential factors of intraoperative requirement for vasopressor infusions were investigated using univariate analysis and Logistic multivariate analysis.Furthermore,the relationships of Shamblin types of CBT with intraoperative MAP/HR fluctuations and requirements for vasoactive agents were analyzed. Results A total of 108 patients with 116 CBTs were included.Univariate analysis revealed that maximum tumor diameter >4 cm,intraoperative internal carotid artery injury,internal carotid artery reconstruction,malignant pathology,advanced Shamblin types(type Ⅱ and Ⅲ),estimated blood loss ≥400 ml,and operation duration >4 hours were associated with intraoperative requirements for vasopressor infusions.Logistic analysis showed that Shamblin type Ⅲ(OR=2.286,95% CI=1.324-14.926,P=0.016)and operation duration >4 hours(OR=3.874,95% CI=1.020-14.623,P=0.046)were risk factors for intraoperative requirements for vasopressor infusions during CBT surgery.In addition,Shamblin type Ⅲ was associated with intraoperative abnormal HR elevation and requirements for vasopressors.Conclusions Shamblin type Ⅲ and operation duration>4 hours are risk factors for intraoperative requirements of patients for using vasopressor infusions during CBT surgery.Shamblin type Ⅲ is associated with intraoperative abnormal HR elevation and requirements for vasopressors.

Propuesta de clasificación tomográfica preoperatoria para el tumor del cuerpo carotideo / Proposal for a preoperative tomographic classification for carotid body tumor

OBJETIVO: proponer una clasificación preoperatoria a los pacientes con tumor de cuerpo Carotídeo y relacionarlos con complicaciones postoperatorias. MATERIAL Y MÉTODOS: todos los pacientes operados con diagnóstico de tumor del cuerpo Carotídeo entre el 2005 al 2014 en el Hospital Obrero N° 1 de la Caja Nacional de Salud en La Paz - Bolivia RESULTADOS: se analizaron y operaron 115 pacientes con un promedio de edad de 52 años (DE±11,725 y una moda de 57 años) de los cuales el 109 (94,80%) correspondieron al género femenino con una razón de 18:1. Todos los pacientes eran originarios y residentes de ciudades ubicadas a más de 2500 m.s.n.m. El promedio de evolución fue de 3 años (DE±2,189), y solo 7 pacientes (6,1%) presentan hábito tabáquico. 83 pacientes (72,2%) de los TCC se localizan en el lado izquierdo En las manifestaciones clínicas, todos los pacientes presentan el tumor localizado en el ángulo mandibular, por delante del musculo esternocleidomastoideo, describiéndose un crecimiento paulatino y permanente en 72 sujetos (62,6%), cefalea en 45 (39,1%), presencia de latido en 30 sujetos (26,1%), disfagia en 9 (7,9%), mareos en 16 (13,9%) y disfonía en 6 (5,2%). Entre los signos más evidentes de TCC, se describe el signo de Fontaine en 114 sujetos (99,2%), adenomegalia en 20 (17,4%) y otros menos frecuentes como soplo, abombamiento parafaringeo y compromiso de pares craneales. Todos los pacientes fueron clasificados en ambos sistemas (Shamblin y la nuestra llamada de los Andes). Se describen 39 pacientes (33,2%) con complicaciones postoperatorias, Grado I: 1 paciente sin complicaciones; Grado II: de 58 sujetos, 4 (3,5%) presentaban parálisis temporal del Hipogloso; en el Grado III: de los 41 sujetos, 24 (20,8%) presentaron ligadura de la arteria carótida externa, parálisis del hipogloso y glosofaringeo, lesión de recurrente y del laríngeo superior. En el grupo IV, de los 15 sujetos operados, 11 presentaron complicaciones (9,6% del total y 73% del grupo) entre las cuales están ligadura de la arteria carótida externa, lesión del hipogloso y un paciente con AVC y hemiparesia. Se describe una reoperación (0,86%) y ninguna mortalidad. CONCLUSIÓN: proponer una clasificación preoperatoria que tenga la posibilidad de asociarse a complicaciones y pronóstico.

OBJECTIVE: to propose a preoperative classification of patients with Carotid Body Tumor and relate them to postoperative complications. MATERIAL AND METHODS: all patients operated with a diagnosis of Carotid Body Tumor between 2005 and 2014 at the Obrero Hospital No. 1 of the National Health Fund in La Paz - Bolivia RESULTS: 115 patients with an average age of 52 years (SD±11.725 and a mode of 57 years) were analyzed and operated on, of which 109 (94.80%) corresponded to the female gender with a ratio of 18: 1. All the patients were from and residents of cities located more than 2,500 meters above sea level. The mean evolution was 3 years (SD±2.189), and only 7 patients (6.1%) had a smoking habit. 83 patients (72.2%) of CBTs are located on the left side In the clinical manifestations, all patients present the tumor located in the mandibular angle, in front of the sternocleidomastoid muscle, describing a gradual and permanent growth in 72 subjects (62.6%), headache in 45 (39.1%), presence of heartbeat in 30 subjects (26.1%), dysphagia in 9 (7.9%), dizziness in 16 (13.9%) and dysphonia in 6 (5.2%). Among the most obvious signs of CBT, the Fontaine sign is described in 114 subjects (99.2%), adenomegaly in 20 (17.4%) and other less frequent signs such as murmur, parapharyngeal bulging and cranial nerve involvement. All patients were classified in both systems (Shamblin and ours called from the Andes). 39 patients (33.2%) with postoperative complications were described, grade I: 1 patient without complications; Grade II: of 58 subjects, 4 (3.5%) had temporary hypoglossal paralysis; in Grade III: of the 41 subjects, 24 (20.8%) presented external carotid artery ligation, hypoglossal and glossopharyngeal paralysis, recurrent lesion and superior larynx. In group IV, of the 15 operated subjects, 11 presented complications (9.6% of the total and 73% of the group), among which are external carotid artery ligation, hypoglossal injury and one patient with stroke and hemiparesis. A reoperation (0.86%) and no mortality are described. CONCLUSION: propose a preoperative classification that has the possibility of being associated with complications and prognosis.

Value of Head and Neck CT Angiography in the Clinical Evaluation of Intraoperative Bleeding Volume of Carotid Body Tumours / 中国医学科学院学报

To investigate the value of head and neck CT angiography(CTA)in the evaluation of intraoperative hemorrhage of carotid body tumours. Head and neck CTA images of 36 patients with carotid body tumours confirmed by pathology were retrospectively analyzed.Patients were divided into two groups based on the intraoperative bleeding volume:<500 ml and≥500 ml groups.The patient's age,sex,Shamblin classification,size of the lesion,number of blood supply arteries,course of the disease,plain scan,and enhanced CT value between two groups were compared and analyzed.Logistics regression equation was established based on the CTA parameters with significant differences between the two intraoperative bleeding volume groups,and combined parameter was acquired.The receiver operating characteristic curve was established based on CTA single and combined parameters. The bleeding volume during the operation of carotid body tumors was significantly correlated with the age of patients(=0.019),the maximum diameter of tumours on axial images(=0.003),the maximum upper and lower diameters(=0.004),Shamblin classification(=0.012),and number of blood supply arteries(<0.001).The area under the receiver operating characteristic curve of the number of feeding arteries,the maximum diameter of axial images,maximum upper and lower diameters,Shamblin classification,and combined parameters were 0.865,0.781,0.806,0.766,and 0.927,respectively.When the optimal critical value was 0.408,the Youden index was 0.794,and the corresponding accuracy,sensitivity,and specificity were 0.919,0.909,and 0.923,respectively. Preoperative head and neck CTA can be used to evaluate the intraoperative blood loss.Combined parameters has the best diagnostic performance compared with single parameters.

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

Clinical features and surgical treatment of neurilemmoma versus carotid body tumors at bifurcation of carotid artery / 浙江大学学报·医学版

OBJECTIVE@#To analyze clinical features, surgical treatment and outcomes of neurilemmoma and carotid body tumors in bifurcation of carotid artery.@*METHODS@#The clinical data of 17 patients with neurilemmomas and 76 patients with carotid body tumors at the bifurcation of carotid artery, who were surgically treated in Zhongshan Hospital of Fudan University from March 2012 to November 2016, were retrospectively analyzed. The clinicopathological characteristics, surgical procedures and outcomes were compared between two groups.@*RESULTS@#No difference of preoperative clinical demographic data was found between two groups. Operation time of the neurilemmoma group was significantly shorter than that of the carotid body tumor group[(93.9±30.8) min vs. (159.3±52.9) min, 0.05).@*CONCLUSIONS@#The clinical manifestations of neurilemmoma and carotid body tumors at carotid artery bifurcation are similar. The carotid body tumor group has a longer operating time, larger intra-operative blood loss, higher external carotid resection rate and relative higher incidence of malignancy. More cautions should be given when carotid body tumors at carotid artery bifurcation are treated.

Paraganglioma de cuerpo carotideo / Carotid body paraganglioma

Contexto: Los paragangliomas del cuerpo carotideo son tumores adrenales de mayor frecuencia entre los paragangliomas; en términos generales, son infrecuentes y deben ser diagnosticados por técnicas de imagen para caracterizar la anatomía y grado de invasión del tumor, a fin de su planificación quirúrgica. Objetivo: evaluar la experiencia institucional del Hospital Carlos Andrade Marín en el manejo de los tumores del cuerpo carotideo. Sujetos y métodos: el estudio retrospectivo analizó los registros médicos de 26 pacientes con paragangliomas del cuerpo carotideo tratados en el Hospital Carlos Andrade Marín de Quito, en un periodo de 21 meses; se complementa con una revisión de la bibliografía. Resultados: del total del universo estudiado el paragaglioma se presentó en 20 mujeres que corresponde al 76%, y en 6 hombres que corresponde al 23. El tamaño promedio del tumor en los pacientes fue de 3,7 ± 1,4 cm. Según la clasificación de Shamblin el paraganglioma en el 74% de los pacientes se encontraba en clasificación ll, el 22% se encontraban en clasificación l, mientras que el 37% se encontraban en clasificación lll. El promedio de sangrado en los pacientes fue 331 ± 284 ml .El tiempo quirúrgico fue de 112 ± 37 minutos. La resección completa del tumor se realizó en el 100 % de los pacientes, en donde en el 7,4% hubieron complicaciones de lesion del nervio craneal y otras complicaciones ocurrieron en el 7,4 % de los pacientes. Conclusión: la experiencia del equipo quirúrgico del Hospital Carlos Andrade Marín ofreció una alternativa segura, con resultados satisfactorios y baja incidencia de secuelas a largo plazo. (AU)

Context: The paragangliomas of the carotid body are adrenal tumors of greater frequency among the paragangliomas; In general terms, they are infrequent and must be diagnosed by imaging techniques to characterize the anatomy and degree of tumor invasion, in order to plan their surgery. Objective: To evaluate the institutional experience of the Hospital Carlos Andrade Marin in the managemen of tumors of the carotid body. Subjects and methods: The retrospective study analyzed the medical records of 26 patients with paragangliomaof the carotid body treated in the Carlos Andrade Marín Hospital in Quito, in a period of 21 months; It icomplemented by a review of the bibliography. Results: of the total universe studied, paragaglioma was present in 20 women, corresponding to 76%, and in 6 men, corresponding to 23%. The average tumor size in the patients was 3.7 ± 1.4 cm. According to the classification of Shamblin paraganglioma in 74% of patients were in classification II, 22% were in classification I, while 37% were in classification III. The average bleeding in the patients was 331 ± 284 ml. The surgical time was 112 ± 37 minutes. Complete resection of the tumor was performed in 100% of the patients, where in 7.4% there were cranial nerve lesions and other complications occurred in 7.4% of the patients. Conclusions: The experience of the surgical team of the Hospital Carlos Andrade Marín offers a safe alternative, with satisfactory results and low incidence of long-term sequelae. (AU)

Caracterización Epidemiológica de los Pacientes con Diagnóstco de Glomus Carotdeo Sometdos a Resección en la Unidad de Cirugía Cardiovascular de Guatemala / Epidemiological Characterization of patients with carotid body tumors who underwent resection in the vascular unit in Guatemala (UNICAR)

Introducción: Los tumores del cuerpo carotideo son tumores infrecuentes, generalmente benignos y muy vascularizados, por lo que su resección es un reto para el cirujano. El objetivo de este estudio es realizar una caracterización epidemiológica de los pacientes con glomus carotideo operados en la Unidad de Cirugía Cardiovascular de Guatemala (UNICAR) Metodología: El estudio fue descriptvo retrospectivo analizando los registros clínicos de todos los pacientes sometidos a resección de glomus carotideo en UNICAR de enero de 2,002 a diciembre de 2,015. Resultados: Se documentaron 17 pacientes a quienes se les realizo resección de glomus en la unidad, de los cuales 1 expediente no fue posible analizar porque no se encontró en el archivo. El 93% de los pacientes fueron de sexo femenino lo cual está descrito que es el género más afectado. La altitud es característica de esta patología ya que el 93%, 15 de los 16 venían de una altura mayor de los 1,500 mts sobre el nivel del mar. No se pudo determinar la etnia como factor de riesgo ya que el 50% de los pacientes fueron de etnia indígena y el 50% de etnia ladina. Se obtuvo un caso de herencia familiar ya que tanto la abuela como la nieta presentaron glomus carotideo. Las biopsias que se documentaron fueron solamente en 5 pero pensamos que hay un subregistro ya que consultando con los especialistas estás pudieron haber sido más. Conclusiones: El Glomus Carotideo es una patología poco frecuente, sin embargo, debe ser tratado en unidades especializadas vasculares debido a su compleja resección y el importante riesgo de sangrado. En este estudio se logró caracterizar epidemiológicamente a los pacientes, coincidiendo con lo reportado en la literatura mundial.

Background: Carotid body tumors are uncommon, generally benign, hypervascular turmors; resection is a challenge for surgeons. The aim of this study is to characterize patents with carotid body tumors who underwent resection at the Cardiovascular Surgical Unit in Guatemala (UNICAR) Methods and Results: There were a total of 17 patents in this descriptve and retrospectve trial who underwent resection of carotid body tumor during 2002 to 2015, but we only had access to 16 of the patents clinical records. 93% were female patents, this is the same as reported in other trials being women more affected than men. Altitude of more than 1,500 m from sea level was present in 93% of the patents. This is remarkable due to the country's geography where there can be in some department's altitudes from 0 m at sea level to as high as 2,800 m. Their background in race speaking isn't related to the presence of this pathology, 50% were indigenous and 50% were ladinos. There was only one case of family related heritage where grandmother and granddaughter had a carotid body tumor resection. Previous biopsy was only recorded in 5 clinical records but we think this can be a sub registry due to the experience of some of the surgeons who said most of them had previous scars and it made the resection more difficult. Conclusions: Carotid body tumors are rare in presentation and they must be treated in a specialized vascular unit due to its complex resection and hemorrhage risk.

Serie de casos: Tumores del Golfo de la yugular / Case series: Glomus jugulare tumors

Introducción: Los tumores del foramen yugular son lesiones de la base del cráneo poco frecuentes, que presentan dificultades tanto para el diagnóstico como para el manejo. El objetivo del presente reporte es presentar una serie de casos y el manejo que se dio a estos pacientes. Métodos: El presente estudio descriptivo incluye pacientes con tumores del foramen yugular manejados quirúrgicamente desde el 2002 hasta 2006 en el Hospital Oncológico "Solón Espinosa Ayala", donde se revisó la técnica quirúrgica y el pronóstico. Los tumores fueron abordados por un mismo equipo multidisciplinario, neurocirujano, cirujano otorrinolaringólogo y radio-oncólogo. Resultados: Se presentan 10 casos. El nervio facial fue reconstruido con injertos del nervio auricular mayor o con anastomosis en cinco casos. Los paragangliomas fueron las lesiones más frecuentes seguidas por Schwannomas y meningiomas. La escisión completa fue posible en 8 casos de tumores benignos y en 4 paragangliomas. Los nervios craneales bajos fueron los más frecuentemente comprometidos con lesiones transitorias en cuatro pacientes. Y la función del nervio facial se recobró espontáneamente en 3 casos. Dos casos desarrollaron fístula de líquido céfalo raquídeo. Un paciente falleció después de la cirugía. Conclusión: Los paragangliomas con extensión intracraneal deben ser radicalmente resecados a través de abordajes multidisciplinarios con preservación de los nervios. La embolización preoperatoria debería considerarse. La extubación precoz, traqueotomía en casos seleccionados y temprana rehabilitación son muy importantes en el manejo postoperatorio.

Introduction: Jugular foramen tumors are rare lesions of the base of the skull, which present difficulties for both diagnosis and management. The objective of this report is to present a series of cases and the management that was given to these patients. Methods: The present descriptive study includes patients with jugular foramen tumors managed surgically from 2002 to 2006 in the "Solón Espinosa Ayala" Oncological Hospital, where the surgical technique and prognosis were reviewed. The tumors were treated by the same multidisciplinary team, neurosurgeon, otolaryngologist and radiation oncologist. Results: 10 cases are presented. The facial nerve was reconstructed with grafts of the greater auricular nerve or with anastomosis in five cases. Paragangliomas were the most frequent lesions followed by Schwannomas and meningiomas. Complete excision was possible in 8 cases of benign tumors and in 4 paragangliomas. Low cranial nerves were the most frequently compromised with transient lesions in four patients. And the function of the facial nerve recovered spontaneously in 3 cases. Two cases developed cerebrospinal fluid fistula. One patient died after surgery. Conclusion: Paragangliomas with intracranial extension must be radically resected through multidisciplinary approaches with preservation of the nerves. Preoperative embolization should be considered. Early extubation, tracheotomy in selected cases and early rehabilitation are very important in postoperative management.

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 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)

Prevalência, aspectos clínicos e anatomopatológicos de paraganglioma de corpo aórtico e carotídeo em cães / Prevalence, clinical signs and anatomic-pathological aspects of aortic and carotid body paragangliomas in dogs / Prevalencia, aspectos clínicos y anatomopatológicos de paraganglioma de cuerpo aórtico y carotideo en perros

Os paragangliomas ou quimiodectomas são neoplasias de quimiorreceptores, de crescimento lento e de comportamento frequentemente benigno, que ocorrem na base do coração, são incomuns, podendo ocorrer em cães e raramente em gatos e bovinos. A avaliação radiográfica e ultrassonográfica contribuem no diagnóstico do paraganglioma de corpo aórtico e carotídeo em cães, sendo necessárias maiores investigações semiológicas devido aos sinais clínicos inespecíficos. O Objetivo do presente artigo é relatar a ocorrência de paraganglioma de corpo aórtico e carotídeo em cães no período de 2004 a 2015 no hospital veterinário da Universidade de Uberaba, destacando-se os sinais clínicos, raça, idade, sexo e presença de metástases. Em um total de 225 exames histológicos de coração e vasos da base cardíaca realizados neste período, cinco cães (2,22%) foram diagnosticados como portadores de paraganglioma, sendo que duas (40%) eram fêmeas e três (60%) machos. A idade média de ocorrência foi de 10,4 ± 4,72 anos, não sendo observada uma predileção racial. Os paragangliomas de corpo aórtico representaram quatro (80%) dos tumores, enquanto os de corpo carotídeo representaram apenas um (20%). Os sinais clínicos foram variáveis por estarem relacionados com a compressão vascular local, com o comprometimento dos órgãos afetados e pela congestão venosa, com consequente aumento da pressão hidrostática e extravasamento de líquido para a cavidade torácica. Salienta-se que em um cão desenvolveu metástase hepática. Apesar da ocorrência incomum o paraganglioma deve ser incluído na lista de diagnósticos diferenciais de cardiopatia em cães.(AU)

Paragangliomas or chemodectomas are uncommon slow-growing chemoreceptor neoplasms, often benign, which appears at the base of the heart. They may occur in dogs, but rarely in cats and cattle. Radiographic and ultrasound evaluation contribute to the diagnosis of paraganglioma in canine aortic and carotid body tumors, requiring further semiological investigation due to nonspecific clinical signs. The purpose of this article is to report the occurrence of canine aortic and carotid body paraganglioma tumors from 2004 to 2015 at the Uberaba University Veterinary Hospital, highlighting the clinical signs, breed, age, sex and presence of metastases. From a total of 225 histological examinations of heart and cardiac base vessels performed in this period, five dogs (2.22%) were diagnosed as having paraganglioma, with two (40%) being female and three (60%) male. The mean age of occurrence was 10.4 ± 4.72 years, and no breed preference could be observed. The paragangliomas of the aortic body represented four (80%) of the tumors, while the ones of the carotid body represented only one (20%). Clinical signs varied due to their relationship to local vascular compression, involvement of the affected organs and venous congestion, with consequent increase in hydrostatic pressure and liquid extravasation to the thoracic cavity. One dog developed liver metastasis. Despite the unusual occurrence, paragangliomas should be included in the list of differential diagnoses of canine heart disease.(AU)

Los paragangliomas o quemodectomas son neoplasias de quimiorreceptores, de crecimiento lento y de comportamiento frecuentemente benigno, que ocurren en la base del corazón, son inusuales, pudiendo ocurrir en perros y raramente en gatos y bovinos. La evaluación radiográfica y ultrasonografía contribuyen en el diagnóstico de paraganglioma de cuerpo aórtico y carotideo en perros, siendo necesarias mayores investigaciones semiológicas debido a los signos clínicos inespecíficos. El objetivo del presente artículo es relatar la ocurrencia de paraganglioma de cuerpo aórtico y carotideo en perros en el período de 2004 a 2015 en el hospital veterinario de la Universidad de Uberaba, destacándose los signos clínicos, raza, edad, sexo y presencia de metástasis. En un total de 225 exámenes histológicos de corazón y vasos de la base cardíaca realizados en este período, cinco perros (2,22%) fueron diagnosticados como portadores de paraganglioma, siendo que dos (40%) eran hembras y tres (60%) machos. La edad mediana de ocurrencia fue de 10,4 ± 4,72 años, no siendo observada una predilección racial. Los paragangliomas de cuerpo aórtico representaron cuatro (80%) de los tumores, mientras que los de cuerpo carotideo representaron sólo uno (20%). Los signos clínicos fueron variables por estar relacionados con la compresión vascular local, con el comprometimiento de los órganos afectados y por la congestión venosa, con consecuente aumento de la presión hidrostática y extravasación de líquido para la cavidad torácica. Se destaca que en un perro desarrolló metástasis hepática. A pesar de la ocurrencia inusual el paraganglioma debe ser incluido en la lista de diagnósticos diferenciales de cardiopatía en perros.(AU)

Paciente con tumor de cuerpo carotideo / Patient with carotid body tumor

Los tumores de cuerpo carotideo (paragangliomas) son neoplasias altamente vascularizadas, muy poco frecuentes y generalmente benignas, originadas en los quimiorreceptores del cuerpo carotideo. Se presenta el caso de un paciente de 54 años, con aumento de volumen cervical derecho, asintomático, con estudio preoperatorio y angiografía realizados por tomografía axial computarizada, que resultan compatibles con tumor de cuerpo carotideo. Se realiza disección subadventicial, informando la biopsia paraganglioma. El tumor fue completamente resecado, sin evidencia de recurrencia y sin complicaciones(AU)

Carotid body tumors (paragangliomas) are highly vascularized, infrequent and generally benign neoplasms that emerge in the carotid body chemoreceptors. This is a male patient aged 54 years with increased right cervical volume, preoperative study and angiography by computerized tomography; the result was carotid body tumor. Subadventitial dissection was made for biopsy which yielded the diagnosis of paraganglioma. The tumor was completely excised, with no evidence of recurrence or complication(AU)

Diagnóstico por imagen del tumor de cuerpo carotídeo / Image-based diagnosis of carotic body tumor

Introducción: los tumores de cuerpo carotídeo (TCC) son lesiones de relativa frecuencia en los Andes. Un diagnóstico correcto preoperatorio es muy importante para planificar y realizar una cirugía adecuada. Por este motivo hemos revisado el trabajo diagnóstico realizado en nuestros pacientes. Materiales y métodos: entre 1980 y junio del 2008 se diagnosticaron 160 TCC, de los cuales 138 fueron sometidos a cirugía, 18 pasaron a controles clínicos por edad, contraindicación médica o rechazo del paciente a la cirugía y 4 están esperando cirugía. Hemos estudiado retrospectivamente las modalidades de diagnóstico en estos pacientes. Resultados: de los 138 tumores operados, un diagnóstico correcto preoperatorio fue realizado en 127 casos (92%). En los 11 restantes, el diagnóstico fue de tumor benigno cervical no específico en 6 y de adenopatía en 5. Los estudios de imagen realizados por diferentes radiólogos fueron ultrasonido convencional (USC), ultrasonido doppler color (USD), angiografía carotídea convencional (AC), tomografía axial computarizada (TAC), resonancia nuclear magnética (RNM), angioresonancia y angiotomografía computarizada (ATC). La mayor parte de pacientes tuvieron más de un tipo de examen. Luego de revisar los reportes, la AC, RNM y ATC resultaron 100% correctos. Conclusiones: pensamos que con la sospecha clínica y los estudios de imagen disponibles actualmente, es factible obtener un diagnóstico preciso en la casi la totalidad de TCC.

Introduction: carotid body tumors (CBT) are relatively frequent lesions encountered at high altitudes such as the Andean Region. A correct preoperative diagnosis is essential for surgical planning and performance. For this reason, we have reviewed our experience in the diagnosis of these tumors. Materials and methods: between 1980 and June 2008, 160 CBT were diagnosed. One hundred and thirty-eight underwent surgery, 4 are waiting surgery and 18 were not operated for age, medical conditions or patient refusal. We have reviewed retrospectively the modalities of diagnosis in our patients. Results: among 138 operated tumors, a correct diagnosis was done in 127 cases (92%). The preoperative diagnosis of the remaining 11 patients was unspecified benign tumor in 6 and neck lymph node in 5. The imaging methods performed by different radiologists were conventional ultrasound, color Doppler ultrasound, carotid angiography, axial tomography, magnetic resonance, angioresonance and angiotomography. Most patients had more than one image study performed. Review of radiologic reports revealed a correct diagnosis in all carotid angiographies, magnetic resonance and angiotomography studies. Conclusions: clinical suspicion and current imaging techniques allow a correct diagnosis in practically all cases of CBT.

Paraganglioma del cuerpo carotideo: Revisión bibliográfica / Carotid body paraganglioma: Review of the literature

Introducción: Los paragangliomas son tumores neuroectodérmicos que suelen encontrarse adyacentes a estructuras ganglionares autonómicas. El paraganglioma carotídeo es un tumor parasimpático, habitualmente no secretor de hormonas y de bajo potencial maligno. Objetivo: Realizar una revisión de la literatura con conceptos actuales respecto al abordaje clínico y quirúrgico de esta patología. Diseño: Revisión narrativa de la literatura. Materiales y métodos: Se realizó una búsqueda en bases virtuales como Pubmed y Scielo utilizando los términos "paraganglioma" y "tumor del cuerpo carotídeo" con el fin de encontrar documentos actualizados sobre este tema. Resultados: De las fuentes bibliográficas halladas, se depuraron según impacto, población objeto y tiempo de publicación hasta obtener 48 documentos incluyendo artículos de revisión, reportes de caso y libros, de los cuales se sintetizó información sobre el abordaje del paraganglioma del cuerpo carotídeo. Conclusiones: Ante la presencia de una masa cervical lateral debe considerarse la posibilidad de un paraganglioma; la tomografía computada y la resonancia magnética permiten la aproximación diagnóstica y su clasificación inicial, mientras que la angiografía permite el uso de técnicas de embolización selectiva, cuyo uso en estos tumores es controvertido. La cirugía es el único tratamiento curativo y se considera el manejo de elección en la mayoría de los casos, mientras que la radioterapia se indica en aquellos casos de resecciones incompletas o cuando la cirugía está contraindicada.

Introduction: Paragangliomas are neuroectodermal tumors often found adjacent to autonomic ganglion structures. The carotid paraganglioma is a parasympathetic tumor, usually without hormone secretion function and low malignant potential. Objective: To review current concepts regarding the clinical and surgical management of this condition. Design: Narrative review of the literature. Materials and methods: A search was conducted throughout virtual bases such as Pubmed and Scielo using the terms "paraganglioma" and "carotid body tumor" in order to find updated documents on this topic. Results: From the bibliographical sources found, they were depurated by their impact, target population and publication time until 48 papers including review articles, case reports and books; from these literature, information on carotid body paraganglioma was synthesized. Conclusions: In the presence of a lateral neck mass, paraganglioma should be considered; computed tomography and magnetic resonance allows to do a diagnostic approaching and its initial classification, while angiography allows the use of selective embolization techniques, whose use on these tumors is controversial. Surgery is the only curative treatment and is considered the treatment of choice in most cases, while radiation therapy is indicated in cases of incomplete resection or when surgery is contraindicated.