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1.
Rev. otorrinolaringol. cir. cabeza cuello ; 80(3): 334-343, set. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1144898

ABSTRACT

Resumen El paraganglioma (PG) es una neoplasia infrecuente originada de las células paraganglionares, embriológicamente derivadas de la cresta neural. Se localizan en la cabeza, base de cráneo, cuello, mediastino, abdomen y pelvis. La mayor parte de los PG muestran un curso clínico benigno, sin embargo, algunos casos pueden mostrar un comportamiento biológico agresivo con invasión local y metástasis a distancia. Un avance significativo en patología molecular ha sido el reconocimiento que el 30%-40% de estas neoplasias presentan alteraciones genéticas. Se han descrito más de 45 genes involucrados, incluyendo mutaciones de la línea germinal succinato deshidrogenasa. Actualmente se recomienda hacer test genético a todos los portadores de PG incluyendo los de presentación esporádica. El PG más frecuente se ubica en la glándula suprarrenal llamado feocromocitoma. El diagnóstico definitivo se realiza con histología, sin embargo, el estudio imagenológico puede entregar una aproximación diagnóstica certera. Debido a la aceptación actual que todos los PG tienen potencial metastásico, el concepto de PG benigno y maligno ha cambiado a uno de estimación de riesgo de metástasis, aunque no existe un esquema único aceptado para tal efecto. El tratamiento considera la cirugía, la radioterapia, la observación y terapias combinadas. Dado el lento crecimiento de este tipo de neoplasia y las potenciales complicaciones de la terapia quirúrgica, la observación es una opción especialmente para pacientes añosos dejando las otras opciones para pacientes más jóvenes. En este trabajo se presenta un caso de paraganglioma yugular bilateral gigante tratado con radioterapia de intensidad modulada incluyendo una revisión bibliográfica pertinente.


Abstract Paraganglioma (PG) is a rare neoplasm derived from paraganglionic cells of the neural crest. They are located in the head, skull base, neck, mediastinum, abdomen and pelvis. Most PGs show a benign clinical course, however, some cases may show aggressive biological behavior with local invasion and distant metastasis. A significant advance in molecular pathology has been the recognition that 30%-40% of these neoplasms present genetic alterations; more than 45 genes have been described, including mutations of the germline succinate dehydrogenase. Currently it is recommended to make genetic test to all patients with PG, including sporadic presentation. The most frequent PG is located in the adrenal gland called pheochromocytoma. The definitive diagnosis is made with histology; however, the imaging study can provide an accurate diagnostic approach. It is now accepted that all PG have a metastatic potential, therefore the concept of benign or malignant has been changed to a metastasis risk stratification approach however no single scheme is been widely used. The treatment considers surgery, radiotherapy, observation and combination therapies. Given the slow growth of this type of neoplasia and the potential complications of surgical therapy, observation is an option especially for elderly patients leaving the other options for younger patients. In this work we present a case of giant bilateral jugular paraganglioma treated with intensity modulated radiation therapy, including a pertinent literature review.


Subject(s)
Humans , Female , Middle Aged , Paraganglioma/pathology , Glomus Jugulare Tumor/pathology , Neck/pathology , Paraganglioma/diagnostic imaging , Glomus Jugulare Tumor/genetics , Glomus Jugulare Tumor/radiotherapy , Glomus Jugulare Tumor/therapy , Glomus Jugulare Tumor/diagnostic imaging , Head and Neck Neoplasms/pathology , Neoplasm Metastasis
2.
Rev. chil. cardiol ; 39(2): 147-153, ago. 2020. graf
Article in Spanish | LILACS | ID: biblio-1138527

ABSTRACT

Abstract: A 49-year-old woman presented with dyspnea and palpitations, leading to Functional Class III.An echocardiogram showed a heterogeneous mass adhered to the right heart cavities. This was confirmed by NMR. A large right coronary artery was occluded in relation to the tumor, which was hyper vascularized. Resection of the tumor was performed; the right ventricular wall was sutured, and an atrial defect was closed using pericardial tissue. Post operative course was uneventful and she was asymptomatic 4 years after surgery.


Subject(s)
Humans , Female , Middle Aged , Paraganglioma/surgery , Heart Neoplasms/surgery , Paraganglioma/complications , Paraganglioma/diagnostic imaging , Angiography , Magnetic Resonance Spectroscopy , Dyspnea/etiology , Heart Neoplasms/complications , Heart Neoplasms/diagnostic imaging
3.
Rev. cuba. anestesiol. reanim ; 19(1): e579, ene.-abr. 2020.
Article in Spanish | LILACS, CUMED | ID: biblio-1093133

ABSTRACT

Introducción: El paraganglioma es un tumor neuroendocrino poco frecuente que surge de los paraganglios autonómicos extraadrenales en diversos lugares del organismo. También se les conoce como feocromocitomas extraadrenales. Alrededor de 90 por ciento son benignos y se curan mediante la extirpación quirúrgica, el restante 10 por ciento son malignos, y pueden provocar metástasis a distancia. El diagnóstico es importante por su riesgo de malignidad, por las implicaciones de otras neoplasias asociadas y dada la posibilidad de realizar estudios genéticos para la detección de otros casos dentro de una misma familia. Objetivo: Exponer aspectos clínico-epidemiológicos de esta enfermedad y las implicaciones perioperatorias concernientes a la conducta anestésica perioperatoria de estos pacientes. Caso clínico: Paciente femenina, de 56 años de edad con antecedentes de hipertensión arterial de más dos años de evolución y difícil control, diabetes mellitus e historia de cefalea, palpitaciones y sensación de calor. Diagnóstico imagenológico de una masa pararrenal sospechosa de paraganglioma que se confirmó por estudio histopatológico posterior a la exceresis. Conclusiones: La técnica anestésica para la resección de estos tumores es un desafío importante para el anestesiólogo que debe enfrentar dicha intervención quirúrgica, planteándose los riesgos de las etapas pre-, intra- y posoperatoria(AU)


Introduction: Paraganglioma is a rare neuroendocrine tumor that emerging from the extra-adrenal autonomic paraganglia and it may occur in several parts of the body. Paraganglioma is also named extra-adrenals pheochromocytomas. Roughly 90 percent of these tumors are benign and can be excised through surgery whereas 10 percent of them are malignant and cause distant metastasis. Paragangliomas are closely linked to pheochromocytomas because they cannot be differentiated at cell level and often share the same clinical manifestations such as hypertension, episodic headache, sweating and tachycardia. The diagnosis of these tumors is important because of risk of becoming malignant, the implications of other related neoplasias and the possibility of making genetic studies to detect other cases in the same family. Objective: To expose about epidemiology, clinical manifestations, diagnostic tests and perioperative management of these tumors by a presentation of a clinical case. Case Report: Female 56 years old, clinical history of 2 years of uncontrolled hypertension, diabetes mellitus, who presented with headache, palpitations and feeling of warm. She was diagnosed with a pararrenal mass suspected of paraganglioma associated with catecholamine hypersecretion, confirmed by histopathology. Conclusions: The treatment of these tumors requires a more complete surgical resection possible since the prognosis depends. Surgical management of pheochromocytoma needs special perioperative requirements. The anesthesiologist must observe this surgery, establish the risks of the phase pre-intra and postoperative(AU)


Subject(s)
Humans , Female , Middle Aged , Paraganglioma/surgery , Paraganglioma/diagnostic imaging , Surgical Procedures, Operative , Midazolam/therapeutic use , Early Detection of Cancer/methods , Lidocaine/therapeutic use , Anesthesiologists
5.
West Indian med. j ; 67(1): 25-30, Jan.-Mar. 2018. tab, graf
Article in English | LILACS | ID: biblio-1045811

ABSTRACT

ABSTRACT Objective: Paragangliomas are slow-growing tumours that present with varied clinical spectra. Early recognition is paramount in achieving reduced morbidity and mortality. There is a paucity of data regarding head and neck paragangliomas (HNPGs) in the Caribbean literature. This study aimed to reflect the clinical experience in the management of HNPGs at two Jamaican tertiary referral centres: the Kingston Public Hospital (KPH) and the University Hospital of the West Indies (UHWI). Methods: A retrospective analysis was conducted on all patients presenting to the Ear, Nose and Throat (ENT) departments of the UHWI in 2004-14 and of the KPH in 2012-14 with the diagnosis of a HNPG. Results: There were 15 patients, 1 male and 14 females. The average age at presentation was 47.1 years. The HNPGs in this series included eight patients with glomus tympanicum (GT, 53%), four with glomus jugulare (GJ, 27%), two with carotid body tumours (CBTs, 13%) and one with glomus vagale (GV, 7%). Eight patients underwent surgical resection (two CBTs, four GT and two GJ). Treatment outcomes achieved included: complete resection (four patients), stable with residual disease (two patients), and recurrence (two patients). Seven patients were awaiting definitive treatment, one patient with GJ was referred overseas, and one patient with GV defaulted. Conclusion: Glomus tympanicum is the most common HNPG in this series which contrasts with that of most international series. Despite the limitations within this region, such as limited access to angio-embolization and stereotactic modalities, the management outcomes are similar in some respects to the reported international literature.


RESUMEN Objetivo: Los paragangliomas son tumores de crecimiento lento que se presentan con variados espectros clínicos. Su detección precoz es fundamental para lograr una reducción de la morbilidad y la mortalidad. Hay escasez de datos con respecto a los paragangliomas de cabeza y cuello (PgCC) en la literatura del Caribe. Este estudio tuvo como objetivo reflejar la experiencia clínica en el tratamiento de PgCC en dos centros de remisión terciarios de Jamaica: jamaiquinos: el Hospital Público de Kingston (KPH) y el Hospital Universitario de UWI (HUWI). Métodos: Se llevó a cabo un análisis retrospectivo de todos los pacientes diagnosticados con PgCC que acudieron a los Departamentos de Otorrinolaringología de HUWI en 2004-14 y de KPH en 2012-14. Resultados: Hubo 15 pacientes - 1 varón y 14 hembras. La edad promedio al momento de presentarse fue 47.1 años. El PgCC en esta serie incluyó a ocho pacientes con glomus timpánico (GT, 53%), cuatro con glomus yugular (GY, 27%), dos con tumores del cuerpo carotídeo (TCC, 13%), y uno con glomus vagal (GV, 7%). Ocho pacientes fueron sometidos a resección quirúrgica (dos TCC, cuatro GT, y dos GY). Los resultados logrados con el tratamiento incluyeron: resección total (cuatro pacientes), estables con enfermedad residual (dos pacientes), y recurrencia (dos pacientes). Siete pacientes esperaban un tratamiento definitivo, un paciente con GY fue remitido al extranjero, y un paciente con GV no se presentó. Conclusión: El glomus timpánico es el PgCC más común en esta serie que contrasta con el de la mayoría de las series internacionales. A pesar de las limitaciones dentro de esta región, tales como el acceso limitado a la angioembolización y las modalidades estereotácticas, los resultados del manejo de la enfermedad son similares en algunos aspectos a la literatura internacional reportada.


Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Aged , Young Adult , Paraganglioma/diagnostic imaging , Head and Neck Neoplasms/diagnostic imaging , Paraganglioma/surgery , Tomography, X-Ray Computed , Retrospective Studies , Magnetic Resonance Angiography , Head and Neck Neoplasms/surgery
6.
Rev. cuba. endocrinol ; 27(2): 0-0, mayo.-ago. 2016. ilus
Article in Spanish | LILACS | ID: lil-780733

ABSTRACT

Los paragangliomas son tumores neuroendocrinos que surgen de los paraganglios autonómicos extraadrenales, los cuales son pequeños órganos formados por células derivadas de la cresta neural embrionaria con capacidad de secretar catecolaminas. Los paragangliomas están estrechamente relacionados con los feocromocitomas porque son indistinguibles a nivel celular, y a menudo comparten las mismas manifestaciones clínicas, como hipertensión, cefalea episódica, sudoración y taquicardia. El diagnóstico de estos tumores es importante por su riesgo de malignización, por las implicaciones de otras neoplasias asociadas, y para la posibilidad de realizar estudios genéticos para detección de otros casos dentro de una misma familia. El objetivo de este artículo es desarrollar un resumen sobre la epidemiología, manifestaciones clínicas, pruebas diagnósticas y tratamiento de estos tumores. Se presenta el caso de un joven de raza negra diagnosticado de un paraganglioma(AU)


Paragangliomas are neuroendocrine tumors emerging from the extra-adrenal autonomic paraganglia, which are small organs formed by embryonic neural crest-derived cells with catecholamine-secreting capacity. Paragangliomas are closely linked to pheochromocytomas because they cannot be differentiated at cell level and often share the same clinical manifestations such as hypertension, episodic headache, sweating and tachycardia. The diagnosis of these tumors is important because of risk of becoming malignant, the implications of other related neoplasias and the possibility of making genetic studies to detect other cases in the same family. The objective of this article was to make an abstract about epidemiology, clinical manifestations, diagnostic tests and treatment of these tumors. This is the case of a young Black female who was diagnosed with paraganglioma(AU)


Subject(s)
Humans , Male , Adult , Biopsy/adverse effects , Laparoscopy/statistics & numerical data , Neuroendocrine Tumors/surgery , Paraganglioma/diagnostic imaging , Pheochromocytoma/diagnosis
7.
Article in English | WPRIM | ID: wpr-218636

ABSTRACT

Thoracic paragangliomas comprise only 1-2% of all paragangliomas, including the adrenal pheochromocytomas, and these tumors are mostly found in the mediastinal compartments (1). To the best of our knowledge, there is only one case report in the pathology literature of endobronchial involvement by a primary pulmonary paraganglioma (2). We report here on the CT and bronchoscopic findings of a case of pathologically proven endobronchial paraganglioma in a 37-year-old woman. In our case, bronchoscopy and CT demonstrated an endobronchial hypervascular mass, which indicated the presence of carcinoid or hypervascular metastasis based on the known incidence of such tumors.


Subject(s)
Adult , Bronchial Neoplasms/diagnostic imaging , Bronchoscopy , Contrast Media , Diagnosis, Differential , Female , Humans , Lung Neoplasms/diagnostic imaging , Paraganglioma/diagnostic imaging , Tomography, X-Ray Computed
8.
J. bras. pneumol ; 31(1): 76-79, jan.-fev. 2005. ilus
Article in Portuguese | LILACS | ID: lil-396575

ABSTRACT

Descrevemos uma paciente de 27 anos que se apresentou com paraganglioma de mediastino anterior e médio e nódulos pulmonares bilaterais. O tratamento consistiu na ressecção das lesões pulmonares através de toracotomia anterior bilateral transesternal e retirada do paraganglioma com auxílio de circulação extracorpórea. Como tratamento neoadjuvante foram usadas radioterapia e quimioterapia. A evolução pós-operatória foi satisfatória, e catorze meses depois a paciente encontrava-se assintomática.


Subject(s)
Humans , Female , Adult , Lung Neoplasms/secondary , Mediastinal Neoplasms/complications , Mediastinal Neoplasms/diagnostic imaging , Mediastinal Neoplasms/pathology , Paraganglioma/diagnostic imaging , Paraganglioma/pathology , Paraganglioma/surgery
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