RESUMEN
Paragangliomas are extra-adrenal tumors that are derived from neuroendocrine chromaffin cells. The rare disease has a variable presentation depending upon its anatomic location and functionality. We describe the case of a 56-year-old female patient who had an incidental mass found on imaging. The patient underwent biochemical testing and a mediastinal biopsy due to the nonspecific imaging findings. The mediastinal mass was resected through hemisternotomy and found to be a functional paraganglioma. This case highlights the importance of radiologists to consider the prospect of paragangliomas in the differential diagnosis of an intra-thoracic mass.
Asunto(s)
Cuerpos Aórticos/diagnóstico por imagen , Cuerpos Aórticos/cirugía , Neoplasias del Mediastino/diagnóstico por imagen , Neoplasias del Mediastino/cirugía , Paraganglioma/diagnóstico por imagen , Paraganglioma/cirugía , Toracoscopía , Tomografía Computarizada por Rayos X , Cuerpos Aórticos/patología , Biopsia , Medios de Contraste , Diagnóstico Diferencial , Femenino , Humanos , Neoplasias del Mediastino/patología , Persona de Mediana Edad , Paraganglioma/patologíaRESUMEN
Introducing the diagnostic protocol and therapy of a rare disease reviewing the worldwide literature named as vagal paraganglioma is our main target. This type is specific for women of middle age, and it is appearing as a unilateral neck mass, with or without Fontaine's sign. Giving a heavy aspect on the ear, nose and throat examination, the most important diagnostic tool is CT-angiography. Carefully looking at the size and relationship with the narrowing arteries, veins, nerves, muscles and bony structures (skull base, cervical spine), in most of the cases we choose the surgical procedure. Orv Hetil. 2019; 160(34): 1358-1362.
Asunto(s)
Cuerpos Aórticos/diagnóstico por imagen , Arterias Carótidas/diagnóstico por imagen , Tumor Glómico/diagnóstico por imagen , Paraganglioma Extraadrenal/diagnóstico por imagen , Angiografía , Femenino , Tumor Glómico/patología , Tumor Glómico/cirugía , Humanos , Persona de Mediana Edad , Paraganglioma Extraadrenal/patología , Paraganglioma Extraadrenal/cirugía , Tomografía Computarizada por Rayos XRESUMEN
Paragangliomas of the head and neck are rare vascular skull-base tumors derived from the paraganglionic system with an estimated incidence of 1:30,000 accounting for 3% of all paragangliomas. The most common paraganglioma locations of the head and neck in descending order are the carotid body, jugular, tympanic, and vagal paragangliomas. This article discusses the clinical characterics, normal anatamy, imaging findings and protocols, pathology, staging, and differential diagnosis for paragangliomas of the head and neck.
Asunto(s)
Neoplasias de Cabeza y Cuello/diagnóstico por imagen , Paraganglioma/diagnóstico por imagen , Cuerpos Aórticos/diagnóstico por imagen , Arterias Carótidas/diagnóstico por imagen , Oído Medio/diagnóstico por imagen , Humanos , Venas Yugulares/diagnóstico por imagen , Imagen por Resonancia Magnética , Tomografía Computarizada por Rayos XRESUMEN
There is emerging evidence linking cyanotic heart disease and pheochromocytoma-paraganglioma through a proposed hypoxia pathway. We present a 59-year-old woman with known Eisenmenger syndrome secondary to delayed diagnosis of ventricular septal defect, in whom F-FDG PET/CT provided comprehensive evaluation of both an F-FDG-avid left glomus vagale paraganglioma and also increased right ventricular myocardial glucose metabolism in response to severe pulmonary hypertension in the chronic shunting state.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales/diagnóstico por imagen , Cuerpos Aórticos/diagnóstico por imagen , Complejo de Eisenmenger/diagnóstico por imagen , Paraganglioma Extraadrenal/diagnóstico por imagen , Feocromocitoma/diagnóstico por imagen , Tomografía de Emisión de Positrones , Tomografía Computarizada por Rayos X , Complejo de Eisenmenger/complicaciones , Femenino , Fluorodesoxiglucosa F18 , Humanos , Persona de Mediana Edad , Imagen Multimodal , Paraganglioma Extraadrenal/complicaciones , Feocromocitoma/complicaciones , RadiofármacosRESUMEN
Malignant paraganglioma of the glomus vagale is a rare tumor entity originating from paraganglia or glomus cells. It typically affects middle age. It differs from benign paraganglioma because of its rapid growth and more aggressive clinical behavior. We report the case of a 40-year-old man presenting with a 5 cm lesion in the upper right cervical region detected by computed tomography (CT) and magnetic resonance imaging (MRI), which also showed enlargement of ipsilateral spinal and jugulodigastric lymph nodes with contrast enhancement. Clinical manifestations at diagnosis included a partial neurological deficit involving the right cranial nerves X, XI and XII. Tumor vascularization was assessed by digital angiography. The tumor mass was entirely removed by a right cervical approach with en-bloc resection with the regional lymph nodes. Histopathological examination showed a paraganglioma with cellular pleomorphism, necrotic microfoci, perineural infiltration and angiogenesis. Massive metastases in two of three jugulodigastric and one spinal lymph nodes on the right side were also detected. Postoperative workup included MRI, positron emission tomography (PET)/CT, meta-iodine-benzyl-guanidine (MIBG) scan, liver ultrasound and chest radiography. Subsequently, the patient underwent conformal radiotherapy with concomitant cisplatin administration. At the last clinical and radiological follow-up examination 5 years after completion of treatment, the patient was free of tumor recurrence. The integrated treatment by surgery and chemoradiation was feasible and effective in the management of this rare case of malignant paraganglioma of the glomus vagale. Multicenter studies should be done to increase the knowledge of tumor presentation and natural history and to analyze the possible treatment options.
Asunto(s)
Antineoplásicos/uso terapéutico , Cuerpos Aórticos , Cisplatino/uso terapéutico , Comunicación Interdisciplinaria , Neoplasias del Sistema Nervioso/terapia , Paraganglioma Extraadrenal/terapia , Radioterapia Conformacional , Adulto , Angiografía de Substracción Digital , Cuerpos Aórticos/diagnóstico por imagen , Quimioterapia Adyuvante , Humanos , Escisión del Ganglio Linfático , Metástasis Linfática , Masculino , Neoplasias del Sistema Nervioso/tratamiento farmacológico , Neoplasias del Sistema Nervioso/patología , Neoplasias del Sistema Nervioso/radioterapia , Neoplasias del Sistema Nervioso/cirugía , Paraganglioma Extraadrenal/tratamiento farmacológico , Paraganglioma Extraadrenal/patología , Paraganglioma Extraadrenal/radioterapia , Paraganglioma Extraadrenal/cirugía , Radioterapia Adyuvante , Resultado del TratamientoRESUMEN
This case report demonstrates the resection of an aortic root paraganglioma incidentally discovered in a 32-year-old male with replacement of the root for an oncologically complete resection.
Asunto(s)
Aorta/trasplante , Cuerpos Aórticos/cirugía , Implantación de Prótesis Vascular , Prótesis Vascular , Hallazgos Incidentales , Paraganglioma Extraadrenal/cirugía , Neoplasias Vasculares/cirugía , Adulto , Cuerpos Aórticos/diagnóstico por imagen , Aortografía/métodos , Implantación de Prótesis Vascular/instrumentación , Procedimientos Quirúrgicos Cardíacos , Humanos , Masculino , Paraganglioma Extraadrenal/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Trasplante Homólogo , Resultado del Tratamiento , Neoplasias Vasculares/diagnóstico por imagenAsunto(s)
Cabeza/diagnóstico por imagen , Cuello/diagnóstico por imagen , Absceso/diagnóstico por imagen , Adenoma Pleomórfico/diagnóstico por imagen , Cuerpos Aórticos/diagnóstico por imagen , Branquioma/diagnóstico por imagen , Tumor Glómico/diagnóstico por imagen , Neoplasias de Cabeza y Cuello/diagnóstico por imagen , Hemangioma/diagnóstico por imagen , Humanos , Ganglios Linfáticos/diagnóstico por imagen , Metástasis Linfática/diagnóstico por imagen , Linfoma/diagnóstico por imagen , Mucocele/diagnóstico por imagen , Glándula Parótida/diagnóstico por imagen , Neoplasias de la Parótida/diagnóstico por imagen , Cálculos de las Glándulas Salivales/diagnóstico por imagen , Enfermedades de las Glándulas Salivales/diagnóstico por imagen , Neoplasias de las Glándulas Salivales/diagnóstico por imagen , Sialadenitis/diagnóstico por imagen , Síndrome de Sjögren/diagnóstico por imagen , Quiste Tirogloso/diagnóstico por imagen , Enfermedades de la Lengua/diagnóstico por imagen , UltrasonografíaRESUMEN
Malignant vagal paraganglioma is very uncommon and the diagnosis of malignancy is made on the basis of presence of distant metastasis rather than the histological findings. We report angiography findings of metastatic cervical lymph nodes in a case of malignant vagal paraganglioma.
Asunto(s)
Cuerpos Aórticos/diagnóstico por imagen , Angiografía Cerebral , Tumor Glómico/diagnóstico por imagen , Adulto , Angiografía de Substracción Digital , Cuerpos Aórticos/patología , Cuerpos Aórticos/cirugía , Diagnóstico Diferencial , Femenino , Tumor Glómico/patología , Tumor Glómico/cirugía , Humanos , Metástasis Linfática , Imagen por Resonancia MagnéticaRESUMEN
PURPOSE: In this study, we describe the color Doppler imaging findings in carotid body tumors and vagal body tumors. METHODS: B-mode and color Doppler imaging were performed on 17 patients who had a total of 25 previously diagnosed paragangliomas (14 carotid body tumors and 11 vagal body tumors). RESULTS: Nineteen of 25 tumors were depicted. Five small vagal body tumors in the region of the nodose ganglion and 1 carotid body tumor could not be depicted. With B-mode imaging, paragangliomas appeared as well-defined, solid, hypoechoic masses. With color Doppler imaging, hypervascularity with a low-resistance flow pattern was demonstrated in all but 1 of the 19 tumors. CONCLUSIONS: The use of color Doppler imaging in the workup of an ambiguous neck mass is advocated.
Asunto(s)
Cuerpos Aórticos/diagnóstico por imagen , Tumor del Cuerpo Carotídeo/irrigación sanguínea , Neoplasias de Cabeza y Cuello/irrigación sanguínea , Paraganglioma Extraadrenal/irrigación sanguínea , Ultrasonografía Doppler en Color , Adulto , Anciano , Velocidad del Flujo Sanguíneo , Tumor del Cuerpo Carotídeo/diagnóstico por imagen , Femenino , Neoplasias de Cabeza y Cuello/diagnóstico por imagen , Humanos , Masculino , Persona de Mediana Edad , Paraganglioma Extraadrenal/diagnóstico por imagenRESUMEN
A patient with an aorticopulmonary paraganglioma was found to have normal plasma norepinephrine and epinephrine levels and elevated dopamine levels. Iodine-123-MIBG scintigraphy did not visualize this tumor. The same finding on a negative MIBG scan in two patients with exclusively dopamine-secreting chemodectomas has been previously reported. In our patient, [111In-DTPA-D-Phe1]-octreotide scintigraphy proved to be an effective imaging technique.
Asunto(s)
Cuerpos Aórticos/diagnóstico por imagen , Dopamina/metabolismo , Radioisótopos de Yodo , Yodobencenos , Paraganglioma Extraadrenal/diagnóstico por imagen , Paraganglioma Extraadrenal/metabolismo , 3-Yodobencilguanidina , Medios de Contraste , Femenino , Humanos , Radioisótopos de Indio , Persona de Mediana Edad , Cintigrafía , Somatostatina/análogos & derivadosRESUMEN
Two cases of vagal body tumours are reported for their surgical consequences of immediate post-operative airway obstruction in one case, and tachycardia hypotension and severe dysphagia requiring cricopharyngeal myotomy in another. An unusual case of carotid body tumour which had the same clinical picture was also reported for comparative surgical treatment. The surgical management of both tumours was reviewed and it was found that 28-43 per cent of carotid body tumour resection would have vascular operation while dissection of vagal body tumour usually did not. The angiographic diagnostic criteria for vagal body tumour were also reviewed and found to be inadequate. Additional angiographic criterion was proposed.