Hipertensão arterial secundária a tumor raro da glândula adrenal / Secondary hypertension to rare adrenal gland tumor / Hipertensión arterial secundaria a un tumor raro de la glándula

O ganglioneuroma é um tumor do sistema nervoso simpático, podendo estar associado à hipersecreção de substâncias vasoativas responsáveis por sintomas e sinais variados, como a hipertensão arterial. Os autores apresentam um caso de ganglioneuroma e uma revisão da literatura, focando os aspectos mais importantes do diagnóstico e da terapêutica. A paciente apresentava crises hipertensivas sintomáticas recorrentes, tendo realizado estudo imaginológico que detectou uma imagem nodular na adrenal direita. Sendo os tumores neuroblásticos indistinguíveis radiologicamente, procedeu-se à excisão, confirmando-se o diagnóstico pela análise anatomopatológica. Este caso demonstra a variabilidade clínica dos ganglioneuromas, a utilidade da imaginologia e a importância do estudo histológico.

The ganglioneuroma is a tumor of the sympathetic nervous system and may be associated with hypersecretion of vasoactive substances responsible for various symptoms and signs such as hypertension. The authors report a case of ganglioneuroma and a literature review, focusing on the most important aspects of diagnosis and therapy. The patient had recurrent symptomatic hypertensive crises, having performed an imaging study that found a nodule in the right adrenal gland. As neuroblastic tumors are radiologically undistinguishable, the patient underwent excision, confirming the diagnosis by pathology analysis. This case demonstrates the clinical variability of ganglioneuroma, the usefulness of imaging and the importance of histological study.

El ganglioneuroma es un tumor del sistema nervioso simpático, y puede estar asociado con la hipersecreción de sustancias vasoactivas responsables de diversos síntomas y signos, como la hipertensión arterial. Los autores presentan un caso de ganglioneuroma y una revisión de la literatura, centrándose en los aspectos más importantes del diagnóstico y la terapéutica. El paciente tenía crisis hipertensivas sintomáticas recurrentes, e hizo un estudio imaginológico que detectó una imagen nodular en la glándula adrenal derecha. Al ser los tumores neuroblásticos radiológicamente indistinguibles, se procedió a la extirpación, lo que confirma el diagnóstico mediante el análisis anatomopatológico. Este caso demuestra la variabilidad clínica de ganglioneuroma, la utilidad de la imagiologia y la importancia del estudio histológico.

Hereditary hemorrhagic telangiectasia: rare cause of pulmonary hypertension?

A 73-year-old woman was admitted to the emergency room with predominantly right-sided heart failure and anemia. Following clinical and imagiological evaluation, a diagnosis of pulmonary hypertension (PH) associated with Hereditary Hemorrhagic Telangiectasia (HHT) was confirmed. The initial response to bosentan plus sildenafil was good, including improvement in functional class and reduction of edema, allowing her to be discharged. Unfortunately, the patient died, due to her underlying condition, before the effects of the combination treatment could be fully assessed. PH should be considered in patients with HTT and screening for pulmonary hypertension should be performed in these patients and their relatives.

Telangiectasia hereditária hemorrágica: causa rara de hipertensão pulmonar? / Hereditary hemorrhagic telangiectasia: rare cause of pulmonary hypertension?

Uma mulher de 73 anos foi admitida ao Pronto-Socorro com insuficiência cardíaca predominantemente direita e anemia. Após avaliação clínica e imagenológica, um diagnóstico de hipertensão pulmonar (HP) associado com telangiectasia hemorrágica hereditária (THH) foi confirmado. A resposta inicial à terapia com bosentan mais sildenafil foi boa, incluindo melhora na Classe Funcional e redução do edema, permitindo que ela recebesse alta hospitalar. Infelizmente, a paciente faleceu devido à sua condição básica, antes que o efeito do tratamento combinado pudesse ser completamente avaliado. A HP deve ser considerada em pacientes com THH e o screening para HP deve ser conduzido nesses pacientes e em seus familiares.

A 73-year-old woman was admitted to the emergency room with predominantly right-sided heart failure and anemia. Following clinical and imagiological evaluation, a diagnosis of pulmonary hypertension (PH) associated with Hereditary Hemorrhagic Telangiectasia (HHT) was confirmed. The initial response to bosentan plus sildenafil was good, including improvement in functional class and reduction of edema, allowing her to be discharged. Unfortunately, the patient died, due to her underlying condition, before the effects of the combination treatment could be fully assessed. PH should be considered in patients with HTT and screening for pulmonary hypertension should be performed in these patients and their relatives.

Secondary hypertension to rare adrenal gland tumor.

The ganglioneuroma is a tumor of the sympathetic nervous system and may be associated with hypersecretion of vasoactive substances responsible for various symptoms and signs such as hypertension. The authors report a case of ganglioneuroma and a literature review, focusing on the most important aspects of diagnosis and therapy. The patient had recurrent symptomatic hypertensive crises, having performed an imaging study that found a nodule in the right adrenal gland. As neuroblastic tumors are radiologically undistinguishable, the patient underwent excision, confirming the diagnosis by pathology analysis. This case demonstrates the clinical variability of ganglioneuroma, the usefulness of imaging and the importance of histological study.