Assuntos
Progressão da Doença , Tumor do Glomo Jugular , Humanos , Tumor do Glomo Jugular/radioterapia , Tumor do Glomo Jugular/cirurgia , Tumor do Glomo Jugular/patologia , Neoplasias da Orelha/radioterapia , Neoplasias da Orelha/patologia , Conduta Expectante , Paraganglioma/radioterapia , Paraganglioma/patologia , Paraganglioma/diagnóstico por imagemAssuntos
Progressão da Doença , Tumor do Glomo Jugular , Humanos , Tumor do Glomo Jugular/radioterapia , Tumor do Glomo Jugular/cirurgia , Tumor do Glomo Jugular/patologia , Tumor do Glomo Jugular/diagnóstico por imagem , Conduta Expectante , Neoplasias da Orelha/radioterapia , Neoplasias da Orelha/patologia , Paraganglioma/radioterapia , Paraganglioma/patologia , Paraganglioma/diagnóstico por imagemRESUMO
INTRODUCTION: Tympanojugular paragangliomas (TJ PGLs) are rare tumors characterized by bone infiltration and erosion and a close relationship with critical structures, such as cranial nerves and internal carotid artery. For these reasons, their management represents a tough challenge. Since the fifties, radio-therapy (RT) has been proposed as an alternative treatment aimed at avoiding tumor progression. However, the indolent nature of the tumor, characterized by slow growth, is a crucial factor that needs to be considered before offering radiation. METHODS: This study aims to examine tumor progression in RT patients through a systematic review of the literature and in TJ PGL patients who underwent solely wait and scan at our department. RESULTS: The rate of tumor progression in the RT group was 8.9%, while in the wait and scan cohort was 12.9%. This data suggests the innate slow growth of PGLs. However, it is not possible to draw certain conclusions because of the wide heterogeneity of the studies. CONCLUSION: When complete surgical excision of TJ PGLs is not feasible, appropriate counseling and patient selection, including comprehensive tumor classification, should be performed before proposing RT to control tumor progression, since wait and scan may represent a reasonable option in selected cases.
Assuntos
Progressão da Doença , Tumor do Glomo Jugular , Humanos , Tumor do Glomo Jugular/radioterapia , Tumor do Glomo Jugular/cirurgia , Tumor do Glomo Jugular/patologia , Tumor do Glomo Jugular/diagnóstico por imagem , Conduta Expectante , Masculino , Feminino , Neoplasias da Orelha/radioterapia , Neoplasias da Orelha/patologia , Neoplasias da Orelha/diagnóstico por imagem , Pessoa de Meia-Idade , Paraganglioma/radioterapia , Paraganglioma/patologia , Paraganglioma/diagnóstico por imagem , AdultoRESUMO
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.
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Paraganglioma/patologia , Tumor do Glomo Jugular/patologia , Pescoço/patologia , Paraganglioma/diagnóstico por imagem , Tumor do Glomo Jugular/genética , Tumor do Glomo Jugular/radioterapia , Tumor do Glomo Jugular/terapia , Tumor do Glomo Jugular/diagnóstico por imagem , Neoplasias de Cabeça e Pescoço/patologia , Metástase NeoplásicaRESUMO
Os autores apresentam os achados clínicos, radiológicos e anátomo-patológico de 6 casos de tumor glômico de osso temporal. Cinco pacientes eram do sexo feminino; a idade variava de 22 a 76 anos (média 48 anos). As manifestaçöes clínicas predominantes foram hipoacusia, zumbido e achados otoscópicos sugestivos do diagnóstico. Säo revistos e discutidos vários aspectos relacionados `a origem e fisiopatogênese dos tumores glômicos, assim como seu diagnóstico e tratamento