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1.
Acta Otorrinolaringol Esp ; 66(6): 332-41, 2015.
Article in English, Spanish | MEDLINE | ID: mdl-25638014

ABSTRACT

INTRODUCTION: Head and neck paragangliomas are rare tumours of a neuroendocrine nature. The aim of this study was to show our experience in the management of these kind of tumours. METHODS: This was a retrospective study of head and neck paragangliomas diagnosed between 1978 and 2014. A total of 126 patients with 162 tumours were analysed. The paragangliomas included 88 jugulotympanic tumours, 53 carotid tumours and 21 vagal paragangliomas. RESULTS: Mean age at diagnosis was 53.1 years; 87 patients were female (69.1%) and 39, male (30.9%). Multifocality was present in 24 patients (19.1%). Germline mutations were found in 20 patients analysed; SDHD and SDHB were the most frequent. Surgery was performed on 72 isolated paragangliomas: these were 9 carotid, 21 tympanic, 37 jugular and 5 vagal paragangliomas. There were 25 isolated tumours that were observed periodically: 7 carotid, 3 tympanic, 9 jugular and 6 vagal paragangliomas; 5 jugular tumours were irradiated. Multifocal paragangliomas were individually treated, with a total of 26 surgical procedures and 36 tumours resected, 9 irradiated and 12 tumours periodically observed. Postoperative cranial nerve deficits in isolated carotid paragangliomas were lower (15%) compared with jugular tumours (45.5%, P=.04). Nerve deficit was found more frequently in tumours with intradural extension (100%) than in extradural tumours (37.5%, P=.007). CONCLUSIONS: Management of head and neck paragangliomas include surgery, radiotherapy and wait and scan policies. A combination of all of them is usually needed in patients with multifocal paragangliomas.


Subject(s)
Head and Neck Neoplasms/epidemiology , Paraganglioma/epidemiology , Adult , Cranial Nerve Injuries/epidemiology , Dura Mater/pathology , Female , Genetic Predisposition to Disease , Germ-Line Mutation , Head and Neck Neoplasms/genetics , Head and Neck Neoplasms/radiotherapy , Head and Neck Neoplasms/surgery , Humans , Male , Middle Aged , Neoplasm Invasiveness , Neoplastic Syndromes, Hereditary/epidemiology , Paraganglioma/genetics , Paraganglioma/radiotherapy , Paraganglioma/surgery , Postoperative Complications/epidemiology , Retrospective Studies , Watchful Waiting
2.
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
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