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.

Head and neck paragangliomas: clinical and molecular genetic classification

Head and neck paragangliomas are tumors arising from specialized neural crest cells. Prominent locations are the carotid body along with the vagal, jugular, and tympanic glomus. Head and neck paragangliomas are slowly growing tumors, with some carotid body tumors being reported to exist for many years as a painless lateral mass on the neck. Symptoms depend on the specific locations. In contrast to paraganglial tumors of the adrenals, abdomen and thorax, head and neck paragangliomas seldom release catecholamines and are hence rarely vasoactive. Petrous bone, jugular, and tympanic head and neck paragangliomas may cause hearing loss. The internationally accepted clinical classifications for carotid body tumors are based on the Shamblin Class I-III stages, which correspond to postoperative permanent side effects. For petrous-bone paragangliomas in the head and neck, the Fisch classification is used. Regarding the molecular genetics, head and neck paragangliomas have been associated with nine susceptibility genes: NF1, RET, VHL, SDHA, SDHB, SDHC, SDHD, SDHAF2 (SDH5), and TMEM127. Hereditary HNPs are mostly caused by mutations of the SDHD gene, but SDHB and SDHC mutations are not uncommon in such patients. Head and neck paragangliomas are rarely associated with mutations of VHL, RET, or NF1. The research on SDHA, SDHAF2 and TMEM127 is ongoing. Multiple head and neck paragangliomas are common in patients with SDHD mutations, while malignant head and neck paraganglioma is mostly seen in patients with SDHB mutations. The treatment of choice is surgical resection. Good postoperative results can be expected in carotid body tumors of Shamblin Class I and II, whereas operations on other carotid body tumors and other head and neck paragangliomas frequently result in deficits of the cranial nerves adjacent to the tumors. Slow growth and the tendency of hereditary head and neck paragangliomas to be multifocal may justify less aggressive treatment strategies.

Tumor del corpúsculo carotídeo / Carotid corpuscle tumor

Los tumores del cuerpo carotídeo, paragangliomas o quemodectomas son neoplasias poco frecuentes que se originan a partir de los tejidos del paraganglio, son generalmente benignos, de crecimiento lento y curso indoloro, presentan un gran componente vascular en su estructura. Pueden manifestar un comportamiento locorregional agresivo a pesar que histológicamente no muestren signos de malignidad. Se observan frecuentemente en pacientes que habitan en regiones de elevada altitud. Pueden ser esporádicos o familiares y se asocian en ocasiones a paragangliomas en otros sitios. Presentamos un caso clínico tratado en nuestro servicio en el que se evidencia la agresividad local en su comportamiento

Carotid body tumors, paragangliomas or chemodectomas are uncommon neoplasms originating from paraganglion tissues, generally are benign, of slow growth and painless course, have a great vascular component in its structure. They may to show an aggressive locoregional behavior despite histologically haven't malignancy signs. Frequently are present in patients inhabit in high altitude regions. May to be sporadic or familial and occasionally are associated with paragangliomas in other sites. Present case was treated in our service, which demonstrates its local aggressive behavior

Tumor del cuerpo carotídeo: A propósito de 10 casos tratados / Carotid body tumors: Report of ten cases

Background: Carotid body tumors arise from a cellular conglomerate located at the carotid bifurcation. Progressive enlargement can involve the arterial wall and neighbor cranial nerves. Aim: To report a series of 10 patients treated of carotid body tumors and review national experience. Patients and methods: Between 1984 and 2006, we operated 8 women and 2 men, aged 19 to 75 years, with this type of tumor. Results: The most common cause for consultation was a cervical mass in 90 percent, with a mean evolution lapse of 13.2 months (range 3 to 126). In all cases, diagnosis was confirmed with angiographic imaging and histopathology. Ten tumors were surgically removed with no complications. Eighty percent of tumors were in stage II according to Shamblin classification. During long term follow up all patients have remained asymptomatic. Only 31 carotid body tumors have been reported in Chilean medical literature during a 43 year period. Conclusions: Paragangliomas of the carotid body can be diagnosed in clinical grounds, requiring vascular imaging. These infrequent lesions are generally benign, early surgical removal by surgeons with vascular expertise avoids neurological and or vascular complications.

Paraganglioma with radiographic dye: an interesting histopatholological finding

Extraadrenal paraganglioma makes up a dispersed neuroendocrine system which extends from the base of the skull down to the pelvic floor. Paraganglioma have been found in particularly every site in which normal paraganglia are known to occur. Herein, we present a typical case of carotid body paraganglioma according to both histological and immunohistochemical findings. The presence of a large ribbon-like amphophilic to basophilic amorphous material which, according to the history of angiography, must result from the precipitation of angiographic dye makes this entity unique and the first to be reported

Carotid body paraganglioma

The aim of this paper is to review our experience in the diagnosis and treatment of carotid body paraganglioma [CBP] emphasizing the role of different imaging modalities and preoperative embolization. Between 1995 and 2003 four patients with CBP were analyzed. All patients were females. Their ages ranged from 25 to 60 years. All patients presented with unilateral asymptomatic cervical mass with no family history of paragangliomas. None of the tumours were functional. One patient had preoperative palsy of the vagus, glassopharyngeal, and marginal mandibular nerves. None had evidence of distant metastasis. Three patients were living in a mountainous region. Ultrasonography [US], Doppler sonography, Computed Tomography [CT], Computer Tomography Angiography [CTA], Magnetic Resonance Imaging [MRI] Magnetic Resonance Angiography [MRA], angiography, blood pooling scan and fine needle aspiration [FNA]. Preoperative embolization was carried out for 1 patient 24 hours before surgery with 85% reduction of blood flow. Three patients had resection of the tumour and one patient required resection of the carotid arteries with a vein graft interposition. The fourth patient refused surgery. The decision to perform tumour excision or additional arterial resection was based on preoperative and intraoperative evaluation of the individual tumours. Histopathology of the resected tumours confirmed the diagnosis of CBP. Electron microscopy [EM] showed characteristic catecholamine granules. According to Shamblin classification, one patient was type 1, the second patient type II and the third patient type III. No patient developed postoperative stroke or cranial nerve palsy. The patient with Shamblin type III tumour developed postoperative neurogenic dysphagia and silent aspiration. There was no mortality. During the follow-up of eighteen months to eight years there were no recurrences, while the tumour remained the same for the patient who refused surgery

Paragangliomas de cabeza y cuello / Parangangliomas of the head and neck

Los parangangliomas son tumores altamente vascularizados que se originan de células provenientes de la cresta neural. El tratamiento de elección es la resección quirúrgica completa, lo cual se ve dificultado por su abundante irrigación y por su estrecha relación anatómica con importantes estructuras vásculo-nerviosas. En el presente estudio se revisa retrospectivamenete la experiencia del Departamento de Otorrinolaringología de Clínica Las Condes en el manejo quirúrgico de estas neoplasias. Entre los años 1998 y 2003 se trataron 5 pacientes portadores de paragangliomas de cabeza y cuello: un glomus yugular (GY), 2 glomus carotídeo (GC) y 2 glomus timpánicos (GT). El estudio imagenológico consistió en tomografía computarizada (TC) para todos los casos, complementada con resonancia nuclear magnética (RNM) y/o angiografía en algunos pacientes. El abordaje quirúrgico fue el indicado para cada lesión: timpanotomía retroauricular (GT), cervicotomía (GC) y abordaje infratemporal tipo A de Fisch (GY). En todos se logró una resección tumoral completa, sin presentarse complicaciones perioperatorias de consideración ni recurrencias hasta la fecha. Contando con un adecuado estudio imagenológico y una cuidadosa planificación quirúrgica, la resección de los paragangliomas de cabeza y cuello puede realizarse con un bajo índice de complicaciones, reservando la radioterapia como alternativa terapéutica únicamente en aquellos casos inoperables.

Tumores do corpo carotídeo: revisão de oito casos e abordagem cirúrgica / Carotid body tumors: review of eight cases and surgical approach

Os tumores do corpo carotídeo são pouco frequentes. Apesar disto, contam com vasta literatura, que reflete as controvérsias em relação ao seu comportamento biológico e tratamento, especialmente no que se refere a abordagem cirúrgica. Estudamos 8 pacientes com tumores do corpo carotídeo (9 tumores, um bilateral), operados no serviço de Neurocirurgia da Santa Casa de Belo Horizonte, entre 1989 e 1999. A idade variou de 11 a 66 anos (35,6 +/- 17,7 anos), sendo 4 mulheres e 4 homens. A única alteração ao exame físico de todos os pacientes foi massa cervical palpável. Obteve-se resultado pós-operatório bastante satisfatório, com baixa morbidade. Não houve óbito nesta série. Baseados nessa experiência e na literatura, discutimos os principais aspectos desses tumores, com ênfase na abordagem cirúrgica. Concluimos que os tumores do corpo carotídeo são lesões incomuns, que devem ser tratadas com técnica cirúrgica cuidadosa, possibilitando baixo índice de morbi-mortalidade.

Paraganglioma: tumor de corpo carotídeo / Paraganglioma: carotid body tumor

Resumo: Os autores relatam dois casos com diagnóstico de tumor de corpo carotídeo, descrevendo os aspectos ultra-sonográficos e tomográficos das lesöes. Säo feitos comentários sobre a abrangência clínica dessa doença e discutimos os aspectos diagnósticos, histopatológicos e terapêuticos provenientes de dados clínicos relatados nos casos e da revisäo de literatura realizada.

Tumor de corpo carotídeo: relato de dois casos / Carotid body tumor: report of two cases

Os autores apresentam dois casos de tumor do corpo carotídeo diagnosticados pelo Serviço de Angiologia e Cirurgia Vascular da Casa de Saúde Santa Marcelina. Aproveitam a oportunidade para comentar o diagnóstico clínico, angiológico e o tratamento desta doença.

Extirpación de tumor del cuerpo carotídeo y reemplazo de carótida interna con vena safena / Carotid body tumor resection and replace of internal carotid with saphenous vein

Se presenta un caso de tumor del cuerpo carotídeo. El tratamiento de estos tumores es de preferencia quirúrgico. El ecodoppler color, la tomografía computarizada y/o la resonancia nuclear magnética son importantes en el diagnóstico de esta enfermedad. Sin embargo, la angiografía de los vasos carotídeos es esencial en la evaluación preoperatoria. En manejo quirúrgico incluye la identificación y preservación de estructuras vasculares y nerviosas adyacentes al tumor. Algunos tumores estarán íntimamente adheridos a la circunferencia de la bifurcación carotídea, por lo que será necesario resecar el tumor con la porción de carotídea interna, que será reconstruida con vena safena. En los grandes tumores debiera considerarse la embolización preoperatoria para facilitar su resección.

Tumor maligno do corpo carotídeo - relato de caso / Malignant carotid body tumor - a case report

A ocorrência de malignidade nos tumores do corpo carotídeo tem sido objeto de muita discussao, devido à raridade dos casos em que tal malignidade pode ser histológica e clinicamente comprovada. No período entre 1938 e 1992 foram tratados no Hospital do Câncer (INCa-RJ), 25 casos de tumores do corpo carotídeo, sendo que em 01 (hum) caso a ocorrência de metástases e a evoluçao clínica apresentaram características que permitiram identificá-lo como maligno.

Paragangliomas--a diagnostic dilemma on fine needle aspirates.

Paragangliomas are uncommon tumours arising from the neural crest and are widely distributed throughout the body. Because of the morphologic diversity of these tumours fine needle aspirate (FNA) diagnosis is not very accurate in identifying these neoplasms. Review of FNA from seven paragangliomas (three extra-adrenal, three carotid body and one multicentric in location) was conducted to find any diagnostic criteria. On initial morphologic diagnosis five of the seven paragangliomas were considered malignant (four undifferentiated and one adenocarcinoma). Review of these cases in our series made us wiser. Pleomorphic nuclei, prominent nucleoli, clumped chromatin with finely granular cytoplasm and occasional intranuclear cytoplasmic inclusions were characteristically seen and these were probably the cause for misdiagnosis. This lesion must be entertained in an aspirate from the neck or retroperitoneum which contains cells showing marked nuclear pleomorphism with naked nuclei and have a tendency to spindle or from acini. To confirm these cases argyrophilic granules were demonstrated in two of the four cases studied. However, neuron specific enolase and calcitonin were found to be non-contributory. Ultrastructural examination was helpful and done in one case showed abundant neurosecretory granules.

Carotid body paraganglioma with coexistent pheochromocytoma in childhood.

A male child aged six years presented with a swelling in neck and hypertension. He was diagnosed to have and operated for Carotid body paraganglioma with Coexistent pheochromocytoma. Hypertension persisted even after excision of both the tumours which could be controlled with anti-hypertensive agents. The case is discussed with review of literature.

Paragangliomas del cuerpo carotídeo / Paraganglioma of the carotid body

Los tumores del cuerpo carotideo (paragangliomas, quimiodectomas), se originan en el tejido paraganglionico de la bifurcaci6n carotidea; son lesiones de lenta evolución que aumentan gradualmente en el curso de los años y pueden comprimir las estructuras neurovasculares del cuello; su transformación maligna es rara. La lesión, a menudo asintomatica, puede aparecer en la tercera o cuarta decadas de la vida y es mas frecuente en personas que viven a grandes alturas, y en pacientes con enfermedad pulmonar obstructiva crónica (EPOC). El propósito de este estudio es enfatizar los beneficios de nuestra tecnica quirurgica, ya que con ella hemos obtenido excelentes resultados. Vale destacar la utilidad de la angiografia carotidea bilateral en el estudio y tratamiento de esta patologia. En nuestros pacientes tratados quirurgicamente no se presentó ninguna complicacion definitiva, y la unica morbilidad fue una paresia de la rama mandibular del nervio facial y otra del hipogloso; tampoco hubo mortalidad.

Tumor del corpúsculo carotídeo / Carotid body tumor

La infrecuente patología tumoral del corpúsculo carotideo motiva la presentación de un caso detectado en un paciente de 29 años. Se comentan las características anatomoclínicas. Se destaca la utilidad de la arteriografía y del tratamiento quirúrgico temprano