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1.
Front Endocrinol (Lausanne) ; 13: 903085, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36187102

RESUMEN

Composite pheochromocytoma (CP) is a very rare tumor originating from neural crest cells, predominantly composed of pheochromocytoma (PCC), a chromaffin cell tumor arising in adrenal medulla, and ganglioneuroma, a tumor derived from autonomic ganglion cells of the nervous system. Moreover, CP may be present in the hereditary syndromes of which pheochromocytoma is part. Literature offers scarce data on this subject, and particularly about its biological behavior, clinical evolution, and molecular profile. We report the phenotype and outcome of three cases of CP (PCC and ganglioneuroma components), followed up at the Endocrine Service of the Clementino Fraga Filho University Hospital, Federal University of Rio de Janeiro, UFRJ, Rio de Janeiro, Brazil. Two nonsyndromic patients (cases 1 and 2) were negative to germline mutations in genes VHL, SDHB, SDHC, SDHD, SDHAF2, TMEM127, and MAX, while the third case (case 3) had clinical diagnosis of neurofibromatosis syndrome. Cases 1, 2, and 3 were diagnosed at 29, 39, and 47 years old, respectively, and were followed up for 3, 17, and 9 years without no CP recurrence. All cases had apparent symptoms of catecholaminergic excess secreted by PCC. Ganglioneuroma, the neurogenic component present in all three cases, had a percentage representation ranging from 5% to 15%. Tumors were unilateral and large, measuring 7.0 cm × 6.0 cm × 6.0 cm, 6.0 cm × 4.0 cm × 3.2 cm, and 7.5 cm × 6.0 cm × 4.5 cm, respectively. All cases underwent adrenalectomy with no recurrence, metastasis, or development of contralateral tumor during follow-up. Genetic testing has been scarcely offered to CP cases. However, a similar frequency of genetic background is found when compared with classic PCC, mainly by the overrepresentation of NF1 cases in the CP subset. By literature review, we identified a notorious increase in cases reported with CP in the last decade, especially in the last 3 years, indicating a recent improvement in the diagnosis of this rare disorder in clinical practice.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Ganglioneuroma , Paraganglioma , Feocromocitoma , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Neoplasias de las Glándulas Suprarrenales/genética , Neoplasias de las Glándulas Suprarrenales/cirugía , Brasil , Ganglioneuroma/diagnóstico , Ganglioneuroma/genética , Ganglioneuroma/cirugía , Humanos , Paraganglioma/patología , Feocromocitoma/diagnóstico , Feocromocitoma/genética , Feocromocitoma/cirugía
2.
Rev. med. Chile ; 150(8): 1115-1118, ago. 2022. ilus, tab
Artículo en Español | LILACS | ID: biblio-1431865

RESUMEN

Up to 40% of Pheochromocytoma/paraganglioma syndromes are associated with germline mutations. Therefore, they are considered familial and heritable. We report a 65 year old woman with hypertension, bilateral adrenal nodules found in the CT scan and elevated urinary metanephrines. Her genetic testing showed a c.117_120delGTCT TMEM127 gene mutation. She was subjected to a laparoscopic bilateral adrenal excision. After five years of follow up, no recurrence of the disease has been recorded.


Asunto(s)
Humanos , Femenino , Anciano , Feocromocitoma/cirugía , Feocromocitoma/genética , Feocromocitoma/diagnóstico por imagen , Neoplasias de las Glándulas Suprarrenales/genética , Neoplasias de las Glándulas Suprarrenales/diagnóstico por imagen , Mutación de Línea Germinal , Predisposición Genética a la Enfermedad , Proteínas de la Membrana/genética , Mutación
3.
Rev Med Chil ; 150(8): 1115-1118, 2022 Aug.
Artículo en Español | MEDLINE | ID: mdl-37358160

RESUMEN

Up to 40% of Pheochromocytoma/paraganglioma syndromes are associated with germline mutations. Therefore, they are considered familial and heritable. We report a 65 year old woman with hypertension, bilateral adrenal nodules found in the CT scan and elevated urinary metanephrines. Her genetic testing showed a c.117_120delGTCT TMEM127 gene mutation. She was subjected to a laparoscopic bilateral adrenal excision. After five years of follow up, no recurrence of the disease has been recorded.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Feocromocitoma , Humanos , Femenino , Anciano , Feocromocitoma/diagnóstico por imagen , Feocromocitoma/genética , Feocromocitoma/cirugía , Predisposición Genética a la Enfermedad , Proteínas de la Membrana/genética , Mutación , Mutación de Línea Germinal , Neoplasias de las Glándulas Suprarrenales/diagnóstico por imagen , Neoplasias de las Glándulas Suprarrenales/genética
4.
Rev. chil. anest ; 51(3): 335-338, 2022. ilus
Artículo en Español | LILACS | ID: biblio-1571315

RESUMEN

Pheochromocytoma is a catecholamine-producing tumor that can occur sporadically or in association with other entities and syndromes. The anesthetic management of these patients must be very careful, focused mainly on avoiding hypertensive crises and therefore stimuli that can trigger them. The role of the anesthesiologist is very important in these cases, not only intraopera- tively but from the moment a pre-anesthetic assessment is made, since patients usually require drugs such as alpha-blockers to reach surgery in optimal conditions. We present the anesthetic management in a 54-year-old woman previously diagnosed with Von Hippel-Lindau syndrome in whom a pheochromocytoma was observed in the clinical follow-up and required to be scheduled for surgery.


El feocromocitoma es un tumor productor de catecolaminas que se puede presentar de forma esporádica o asociada a otras entidades y síndromes. El manejo anestésico de estos pacientes debe ser muy cuidadoso, enfocado principalmente a evitar crisis hipertensivas y por tanto estímulos que puedan desencadenarlas. El papel del anestesiólogo es muy importante en estos casos, no solo de manera intraoperatoria sino desde el momento en el que se hace una valoración preanestésica, ya que los pacientes suelen precisar fármacos como los alfabloqueantes para llegar en unas condiciones óptimas a la cirugía. Presentamos el manejo anestésico en una mujer de 54 años, diagnosticada previamente de síndrome de Von Hippel-Lindau, en la que se objetiva un feocromocitoma en el seguimiento clínico y requiere ser programada para cirugía.


Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Feocromocitoma/cirugía , Enfermedad de von Hippel-Lindau/complicaciones , Anestesia , Feocromocitoma/diagnóstico por imagen , Cuidados Posoperatorios , Cuidados Preoperatorios , Tomografía Computarizada por Rayos X , Hipertensión Maligna/prevención & control
5.
Cambios rev. méd ; 20(2): 80-88, 30 Diciembre 2021. ilus, tabs.
Artículo en Español | LILACS | ID: biblio-1368378

RESUMEN

INTRODUCCIÓN. Los feocromocitomas son tumores que provienen de las células neuroendócrinas de la médula adrenal y producen alta secreción de catecolaminas. Generan complicaciones cardiovasculares graves que suelen asociarse con crisis hipertensivas. Es importante valorar el impacto cardiovascular de esta entidad. OBJETIVO. Realizar una revisión exhaustiva de las diversas manifestaciones de los feocromocitomas como causa de hipertensión arterial, su impacto cardiovascular, conducta diagnóstica y terapéutica. MATERIALES Y MÉTODOS. Revisión bibliográfica y análisis de 141 artículos científicos que incluyeron temas sobre el impacto cardiovascular, conducta diagnóstica y terapéutica del feocromocitoma como causa de hipertensión arterial. Se usó bases de datos: Medline, Embase, Scopus, Pubmed, Google Académico. Criterios de búsqueda en DECS, MeSH: "pheochromocytoma OR hypertension arterial AND cardiomyopathy", en inglés- español. Fueron seleccionados: 13 publicaciones de texto completo, 10 artículos retrospectivos, 2 guías de práctica clínica y 1 revisión. Se excluyeron 128 artículos científicos. RESULTADOS. Se realizó una revisión de las manifestaciones clínicas de los feocromocitomas como causa de hipertensión arterial y el impacto cardiovascular se relacionó con la producción de catecolaminas. Para el diagnóstico, la sensibilidad de la resonancia magnética es del 93-100%; la especificidad de resonancia magnética o tomografía computarizada en combinación con gammagrafía con metayodobencilguanidina con 123I es cercana al 100%. La resección del feocromocitoma tiene potencial curativo. CONCLUSIÓN. Los feocromocitomas presentan variabilidad clínica, se asocian a complicaciones cardiovasculares y cerebrovasculares graves por producción de catecolaminas. El diagnóstico oportuno y eficaz debe realizarse mediante resonancia magnética y gammagrafía en caso de alta sospecha clínica. El tratamiento quirúrgico es de elección.


INTRODUCTION. Pheochromocytomas are tumors arising from the neuroendocrine cells of the adrenal medulla and produce high secretion of catecholamines. They generate severe cardiovascular complications that are often associated with hypertensive crises. It is important to assess the cardiovascular impact of this entity. OBJECTIVE. To perform an exhaustive review of the various manifestations of pheochromocytomas as a cause of arterial hypertension, their cardiovascular impact, diagnostic and therapeutic conduct. MATERIALS AND METHODS. Bibliographic review and analysis of 141 scientific articles that included topics on the cardiovascular impact, diagnostic and therapeutic behavior of pheochromocytoma as a cause of arterial hypertension. The following databases were used: Medline, Embase, Scopus, Pubmed, Google Scholar. Search criteria in DECS, MeSH: "pheochromocytoma OR hypertension arterial AND cardiomyopathy", in English-Spanish. The following were selected: 13 full-text publications, 10 retrospective articles, 2 clinical practice guidelines, and 1 review. A total of 128 scientific articles were excluded. RESULTS. A review of the clinical manifestations of pheochromocytoma as a cause of arterial hypertension was performed and the cardiovascular impact was related to catecholamine production. For diagnosis, the sensitivity of MRI is 93-100%; the specificity of MRI or computed tomography in combination with 123I-methiodobenzylguanidine scintigraphy is close to 100%. Resection of pheochromocytoma has curative potential. CONCLUSION. Pheochromocytomas present clinical variability, are associated with severe cardiovascular and cerebrovascular complications due to catecholamine production. Timely and effective diagnosis should be made by MRI and scintigraphy in case of high clinical suspicion. Surgical treatment is the treatment of choice.


Asunto(s)
Humanos , Feocromocitoma/complicaciones , Neoplasias de las Glándulas Suprarrenales/complicaciones , Hipertensión/etiología , Feocromocitoma/cirugía , Feocromocitoma/diagnóstico , Catecolaminas/metabolismo , Neoplasias de las Glándulas Suprarrenales/cirugía , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Corazón/fisiopatología , Cardiopatías/etiología
6.
Rev Med Inst Mex Seguro Soc ; 59(4): 352-357, 2021 Aug 02.
Artículo en Español | MEDLINE | ID: mdl-35019754

RESUMEN

BACKGROUND: Adrenal incidentalomas are tumors that are usually detected in imaging studies indicated without suspicion of adrenal disease. Pheochromocytoma is a neuroendocrine tumor that can occur sporadically or associated with genetic syndromes, having been described in 0.1-5% of patients with type 1 neurofibromatosis, which is a progressive multisystemic disease of autosomal dominant inheritance that affects 1 / 2600-3000 individuals . CLINICAL CASE: We present the case of a 50-year-old patient who was admitted to the Emergency Department for acute appendicitis and whose CT scan showed the presence of bilateral adrenal masses is reported. After the surgical intervention, he was hospitalized for study. The patient denied headache, sweating, hypertension, palpitations, and weight loss; Physical examination revealed the presence of multiple lentigines, café-au-lait spots > 15 mm, fixed and pedunculated nodular lesions, compatible with focal neurofibromas, without signs suggestive of hypo or hypercortisolism. In the analysis, total metanephrines and vanillyl-mandelic acid were identified in urine, they were found in very high values, being subjected to laparoscopic adrenalectomy in 2 stages, after adequate preparation that included alpha and beta adrenergic blockade. CONCLUSIONS: In patients with adrenal masses, it is necessary to recognize the importance of a comprehensive clinical evaluation to guide an adequate diagnostic study, as well as the performance of an optimal preoperative study that includes hormonal tests to rule out functionality.


INTRODUCCIÓN: los incidentalomas adrenales son tumores que suelen ser detectados en estudios de imágenes indicados sin sospecha de enfermedad adrenal. El feocromocitoma es un tumor neuroendocrino que puede presentarse esporádicamente o asociado a síndromes genéticos, habiendo sido descrito en 0.1-5% de pacientes con neurofibromatosis tipo 1, que es una enfermedad progresiva multisistémica de herencia autosómica dominante que afecta a 1/2600-3000 individuos. CASO CLÍNICO: se reporta el caso de un paciente de 50 años quien ingresó al departamento de Emergencia por apendicitis aguda y cuya tomografía mostró la presencia de masas adrenales bilaterales. Luego de la intervención quirúrgica fue hospitalizado para estudio. El paciente negó cefalea, diaforesis, hipertensión arterial, palpitaciones y pérdida de peso; en el examen físico se evidenció presencia de múltiples lentígines, manchas café con leche > 15 mm, lesiones nodulares fijas y pedunculadas, compatibles con neurofibromas focales, sin signos sugestivos de hipo o hipercortisolismo. En la analítica se identificaron metanefrinas totales y ácido vanilil-mandélico en orina encontrándose en valores muy elevados, siendo sometido a adrenalectomía laparoscópica en 2 tiempos, luego de una adecuada preparación que incluyó bloqueo alfa y beta adrenérgicos. CONCLUSIONES: en los pacientes con masas adrenales es necesario reconocer la importancia de una evaluación clínica integral para guiar un adecuado estudio diagnóstico, así como la realización de un óptimo estudio preoperatorio que incluye las pruebas hormonales para descartar funcionalidad.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Apendicitis , Feocromocitoma , Neoplasias de las Glándulas Suprarrenales/complicaciones , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Neoplasias de las Glándulas Suprarrenales/cirugía , Adrenalectomía , Apendicitis/complicaciones , Apendicitis/diagnóstico , Apendicitis/cirugía , Humanos , Masculino , Persona de Mediana Edad , Feocromocitoma/complicaciones , Feocromocitoma/diagnóstico , Feocromocitoma/cirugía
7.
Medwave ; 20(2): e7830, 2020 Mar 04.
Artículo en Español | MEDLINE | ID: mdl-32191683

RESUMEN

Pheochromocytomas and paragangliomas are rare neuroendocrine tumors, characterized by a high morbidity rate due to catecholamine excess. These high levels are independent of physiologic stressors. For the diagnosis, a biochemical workup is paramount. The most widely used are plasma-free metanephrines and urinary fractionated metanephrines. Imaging studies should be initiated once the biochemical diagnosis is established. Evaluation of the patient with pheochromocytomas and paragangliomas must be done taking into account the leading causes of perioperative morbidity and mortality. The two primary interventions that have reduced perioperative mortality are alpha-adrenergic blockade and intravascular volume normalization. Another significant advance has been the establishment of laparoscopic surgery as the gold standard for the surgical approach. No anesthetic technique has been found to be superior to another. Intraoperative hemodynamic instability has been correlated with poorer outcomes; thus one of the main intraoperative goals is maintaining hemodynamic stability. Lower morbidity and almost zero mortality rates due to preoperative and intraoperative management improvements have led to a focus on the immediate and long-term postoperative care. Anual lifelong follow-up is recommended to detect recurrent disease.


Los feocromocitomas y paragangliomas son tumores neuroendocrinos raros, caracterizados por una alta tasa de morbilidad debida a un exceso de niveles de catecolaminas. Este exceso de catecolaminas es independiente de los estresores fisiológicos. Para el diagnóstico de un feocromocitoma-paraganglioma son fundamentales las pruebas bioquímicas. Las más utilizadas son las metanefrinas fraccionadas urinarias o metanefrinas libres plasmáticas. Seguido del diagnóstico bioquímico, debe realizarse un estudio imagenológico. La evaluación del paciente con diagnóstico de feocromocitoma-paraganglioma debe realizarse teniendo presente sus principales causas de morbimortalidad perioperatoria. Las dos grandes intervenciones que han disminuido la mortalidad perioperatoria son la introducción del α bloqueo y la restauración de la volemia. El otro gran avance ha sido la introducción de la cirugía laparoscópica como el estándar de oro para el abordaje quirúrgico. En relación con el manejo intraoperatorio, no se ha identificado que alguna técnica anestésica sea superior a otra. Sí se ha logrado establecer criterios de inestabilidad hemodinámica que se correlacionan con mayor morbilidad, por lo que los principales objetivos intraoperatorios son mantener estabilidad hemodinámica. El avance en el manejo preoperatorio e intraoperatorio con la consecuente disminución en la mortalidad relacionada a esta patología ha llevado el foco al manejo postoperatorio tanto agudo como a largo plazo. También se debe considerar el riesgo de recurrencia tumoral, por lo que estos pacientes deben tener un control anual de por vida.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales/cirugía , Paraganglioma/cirugía , Feocromocitoma/cirugía , Humanos , Laparoscopía
8.
Acta sci. vet. (Online) ; 48(suppl.1): Pub. 490, 10 mar. 2020. ilus
Artículo en Portugués | VETINDEX | ID: vti-744819

RESUMEN

Background: In dogs pheochromocytoma usually compress and invade into the adjacent vessels and structures, whichmay lead to the occurrence of caudal vena caval tumor thrombi. The surgical approach in these cases requires adrenalectomy with caval thrombectomy. Conventionally, tourniquets and partial occlusion clamp are used to reduce intraoperativeblood loss, but in dogs with a large thrombus associated to a great local tumor invasion, the traditional technique may behindered. The aim of this study was to report the use of a purse-string suture during venotomy of the caudal vena cava forthrombectomy, as an alternative to tourniquet and partial occlusion clamp.Case: An 11-year-old male Daschound breed dog, was referred for evaluation because of the appearance of small blackenednodules in the ventral abdomen. During a search for metastatic lesions with abdominal ultrasound, the right adrenal wasidentified with significant volume increase, and presence of tumor-associate circular formation that appeared to invade thecaudal vena cava, suggestive of neoplastic invasion or thrombus. Abdominal computed tomography showed caudal venacava in its hepatic portion presenting considerable increase in diameter, with caudal dilation and presence of hypoattenuatingmaterial in its lumen. Right adrenal gland with rounded appearance and regular borders, heterogeneous parenchyma andjuxtaposed caudal vena cava suggesting invasion of this, confirming the sonographic findings. During exploratory celiotomy,adherence of the adrenal mass to the right renal vessels and invasion of the caudal vena cava were observed, leading to theneed of nephrectomy and venotomy for thrombectomy. To perform the venotomy, a Rumel tourniquet was placed looselyaround the vena cava only caudal to the invasion point of the tumor thrombus, cranially, tourniquet application was notpossible due to the presence of a large thrombus extending into the intrahepatic cava...(AU)


Asunto(s)
Animales , Masculino , Perros , Feocromocitoma/cirugía , Feocromocitoma/veterinaria , Neoplasias de las Glándulas Suprarrenales/veterinaria , Flebotomía/veterinaria
9.
Medwave ; 20(2): e7830, 31-03-2020.
Artículo en Inglés, Español | LILACS | ID: biblio-1097789

RESUMEN

Los feocromocitomas y paragangliomas son tumores neuroendocrinos raros, caracterizados por una alta tasa de morbilidad debida a un exceso de niveles de catecolaminas. Este exceso de catecolaminas es independiente de los estresores fisiológicos. Para el diagnóstico de un feocromocitoma-paraganglioma son fundamentales las pruebas bioquímicas. Las más utilizadas son las metanefrinas fraccionadas urinarias o metanefrinas libres plasmáticas. Seguido del diagnóstico bioquímico, debe realizarse un estudio imagenológico. La evaluación del paciente con diagnóstico de feocromocitoma-paraganglioma debe realizarse teniendo presente sus principales causas de morbimortalidad perioperatoria. Las dos grandes intervenciones que han disminuido la mortalidad perioperatoria son la introducción del α bloqueo y la restauración de la volemia. El otro gran avance ha sido la introducción de la cirugía laparoscópica como el estándar de oro para el abordaje quirúrgico. En relación con el manejo intraoperatorio, no se ha identificado que alguna técnica anestésica sea superior a otra. Sí se ha logrado establecer criterios de inestabilidad hemodinámica que se correlacionan con mayor morbilidad, por lo que los principales objetivos intraoperatorios son mantener estabilidad hemodinámica. El avance en el manejo preoperatorio e intraoperatorio con la consecuente disminución en la mortalidad relacionada a esta patología ha llevado el foco al manejo postoperatorio tanto agudo como a largo plazo. También se debe considerar el riesgo de recurrencia tumoral, por lo que estos pacientes deben tener un control anual de por vida.


Pheochromocytomas and paragangliomas are rare neuroendocrine tumors, characterized by a high morbidity rate due to catecholamine excess. These high levels are independent of physiologic stressors. For the diagnosis, a biochemical workup is paramount. The most widely used are plasma-free metanephrines and urinary fractionated metanephrines. Imaging studies should be initiated once the biochemical diagnosis is established. Evaluation of the patient with pheochromocytomas and paragangliomas must be done taking into account the leading causes of perioperative morbidity and mortality. The two primary interventions that have reduced perioperative mortality are alpha-adrenergic blockade and intravascular volume normalization. Another significant advance has been the establishment of laparoscopic surgery as the gold standard for the surgical approach. No anesthetic technique has been found to be superior to another. Intraoperative hemodynamic instability has been correlated with poorer outcomes; thus one of the main intraoperative goals is maintaining hemodynamic stability. Lower morbidity and almost zero mortality rates due to preoperative and intraoperative management improvements have led to a focus on the immediate and long-term postoperative care. Anual lifelong follow-up is recommended to detect recurrent disease.


Asunto(s)
Humanos , Paraganglioma/cirugía , Feocromocitoma/cirugía , Neoplasias de las Glándulas Suprarrenales/cirugía , Laparoscopía
10.
Acta sci. vet. (Impr.) ; 48(suppl.1): Pub.490-4 jan. 2020. ilus
Artículo en Portugués | VETINDEX | ID: biblio-1458317

RESUMEN

Background: In dogs pheochromocytoma usually compress and invade into the adjacent vessels and structures, whichmay lead to the occurrence of caudal vena caval tumor thrombi. The surgical approach in these cases requires adrenalectomy with caval thrombectomy. Conventionally, tourniquets and partial occlusion clamp are used to reduce intraoperativeblood loss, but in dogs with a large thrombus associated to a great local tumor invasion, the traditional technique may behindered. The aim of this study was to report the use of a purse-string suture during venotomy of the caudal vena cava forthrombectomy, as an alternative to tourniquet and partial occlusion clamp.Case: An 11-year-old male Daschound breed dog, was referred for evaluation because of the appearance of small blackenednodules in the ventral abdomen. During a search for metastatic lesions with abdominal ultrasound, the right adrenal wasidentified with significant volume increase, and presence of tumor-associate circular formation that appeared to invade thecaudal vena cava, suggestive of neoplastic invasion or thrombus. Abdominal computed tomography showed caudal venacava in its hepatic portion presenting considerable increase in diameter, with caudal dilation and presence of hypoattenuatingmaterial in its lumen. Right adrenal gland with rounded appearance and regular borders, heterogeneous parenchyma andjuxtaposed caudal vena cava suggesting invasion of this, confirming the sonographic findings. During exploratory celiotomy,adherence of the adrenal mass to the right renal vessels and invasion of the caudal vena cava were observed, leading to theneed of nephrectomy and venotomy for thrombectomy. To perform the venotomy, a Rumel tourniquet was placed looselyaround the vena cava only caudal to the invasion point of the tumor thrombus, cranially, tourniquet application was notpossible due to the presence of a large thrombus extending into the intrahepatic cava...


Asunto(s)
Masculino , Animales , Perros , Feocromocitoma/cirugía , Feocromocitoma/veterinaria , Flebotomía/veterinaria , Neoplasias de las Glándulas Suprarrenales/veterinaria
11.
Braz J Cardiovasc Surg ; 33(5): 522-524, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30517262

RESUMEN

This paper presents a case study of a patient that underwent surgery for a ruptured abdominal aneurysm. The postoperative course was complicated by resistant hypertension and tachycardia. A suprarenal mass was detected in the computed tomography scan with radiological suspicion of pheochromocytoma. Few cases of pheochromocytoma coexisting with aneurysms have been reported. Management of cardiovascular stability is crucial in such cases. Despite the lack of evidence, pheochromocytomas might have a role in the etiology of aortic aneurysms.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales/cirugía , Aneurisma de la Aorta Abdominal/cirugía , Hipertensión/etiología , Feocromocitoma/cirugía , Neoplasias de las Glándulas Suprarrenales/complicaciones , Neoplasias de las Glándulas Suprarrenales/diagnóstico por imagen , Aneurisma de la Aorta Abdominal/complicaciones , Humanos , Masculino , Persona de Mediana Edad , Feocromocitoma/complicaciones , Feocromocitoma/diagnóstico por imagen , Tomografía Computarizada por Rayos X
12.
Int. braz. j. urol ; 44(5): 1032-1035, Sept.-Oct. 2018. graf
Artículo en Inglés | LILACS | ID: biblio-975632

RESUMEN

ABSTRACT Introduction: Paraganglioma is an extremely rare catecholamine-producing tumor during pregnancy. Paraganglioma carries high risks of fetal and maternal mortality during pregnancy. We report a pregnant woman with paraganglioma in the second trimester. Case Description: A 24-year-old pregnant woman presented with severe hypertension in the 17th week of gestation. Hormonal examination and Magnetic Resonance Imaging (MRI) confirmed the diagnosis of extra adrenal pheochromocytoma (paraganglioma). She underwent laparoscopic tumor excision successfully. Conclusions: A high index of suspicion is needed to diagnose paraganglioma in a pregnant patient with hypertension. Laparoscopic tumor removal for paraganglioma seems to be a feasible and safe procedure during pregnancy.


Asunto(s)
Humanos , Femenino , Embarazo , Adulto Joven , Feocromocitoma/cirugía , Complicaciones Neoplásicas del Embarazo/cirugía , Feocromocitoma/diagnóstico por imagen , Complicaciones Neoplásicas del Embarazo/diagnóstico por imagen , Segundo Trimestre del Embarazo , Imagen por Resonancia Magnética , Laparoscopía , Hipertensión/etiología
13.
Rev. bras. cir. cardiovasc ; Rev. bras. cir. cardiovasc;33(5): 522-524, Sept.-Oct. 2018. tab, graf
Artículo en Inglés | LILACS | ID: biblio-977446

RESUMEN

Abstract This paper presents a case study of a patient that underwent surgery for a ruptured abdominal aneurysm. The postoperative course was complicated by resistant hypertension and tachycardia. A suprarenal mass was detected in the computed tomography scan with radiological suspicion of pheochromocytoma. Few cases of pheochromocytoma coexisting with aneurysms have been reported. Management of cardiovascular stability is crucial in such cases. Despite the lack of evidence, pheochromocytomas might have a role in the etiology of aortic aneurysms.


Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Feocromocitoma/cirugía , Aneurisma de la Aorta Abdominal/cirugía , Neoplasias de las Glándulas Suprarrenales/cirugía , Hipertensión/etiología , Feocromocitoma/complicaciones , Feocromocitoma/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Aneurisma de la Aorta Abdominal/complicaciones , Neoplasias de las Glándulas Suprarrenales/complicaciones , Neoplasias de las Glándulas Suprarrenales/diagnóstico por imagen
14.
Int. braz. j. urol ; 44(3): 629-633, May-June 2018. graf
Artículo en Inglés | LILACS | ID: biblio-954044

RESUMEN

ABSTRACT A 32-year-old 22-week pregnant hypertensive woman with sporadic episodes of headaches, sweating, and facial flushing was diagnosed with pheochromocytoma through biochemical and imaging tests. Perioperative management included a multidisciplinary approach, symptom stabilization with α blockade followed by β blockade, and tumor resection by laparoscopic adrenalectomy at 24 weeks gestation. The diagnosis was confirmed by histopathological examination and immuno-histochemistry tests. The decision for surgical removal of the tumor was based on maternal symptoms, tumor size, gestational age, the possibility of doing a laparos-copy, and the expertise of the surgical team.


Asunto(s)
Humanos , Femenino , Embarazo , Adulto , Feocromocitoma/cirugía , Complicaciones Neoplásicas del Embarazo/cirugía , Laparoscopía/métodos , Neoplasias de las Glándulas Suprarrenales/cirugía , Adrenalectomía/métodos , Feocromocitoma/complicaciones , Resultado del Embarazo , Reproducibilidad de los Resultados , Edad Gestacional , Resultado del Tratamiento , Neoplasias de las Glándulas Suprarrenales/complicaciones , Hipertensión/etiología
15.
Urology ; 119: 140-142, 2018 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-29545044

RESUMEN

Pheochromocytoma is a rare chromaffin cell tumor that may be associated with a genetic predisposition, such as von Hippel-Lindau (VHL) disease. VHL is an autosomal dominant disorder that is characterized by a predisposition to multiple tumors, including retinal and central nervous system hemangioblastomas, renal cell carcinoma, and pheochromocytomas. The classic presentation of pheochromocytoma is episodic hypertension, headaches, palpitations, and diaphoresis. In the pediatric population, 40% of pheochromocytomas have a hereditary basis. We present a case of metastatic pheochromocytoma in a child with VHL and discuss the relevant current medical literature.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales/patología , Neoplasias Pulmonares/secundario , Feocromocitoma/secundario , Enfermedad de von Hippel-Lindau , Neoplasias de las Glándulas Suprarrenales/etiología , Neoplasias de las Glándulas Suprarrenales/cirugía , Enfermedades Asintomáticas , Niño , Humanos , Neoplasias Pulmonares/etiología , Neoplasias Pulmonares/cirugía , Masculino , Feocromocitoma/etiología , Feocromocitoma/cirugía , Enfermedad de von Hippel-Lindau/complicaciones
16.
Int Braz J Urol ; 44(5): 1032-1035, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-29570256

RESUMEN

INTRODUCTION: Paraganglioma is an extremely rare catecholamine-producing tumor during pregnancy. Paraganglioma carries high risks of fetal and maternal mortality during pregnancy. We report a pregnant woman with paraganglioma in the second trimester. CASE DESCRIPTION: A 24-year-old pregnant woman presented with severe hypertension in the 17th week of gestation. Hormonal examination and Magnetic Resonance Imaging (MRI) confirmed the diagnosis of extra adrenal pheochromocytoma (paraganglioma). She underwent laparoscopic tumor excision successfully. CONCLUSIONS: A high index of suspicion is needed to diagnose paraganglioma in a pregnant patient with hypertension. Laparoscopic tumor removal for paraganglioma seems to be a feasible and safe procedure during pregnancy.


Asunto(s)
Feocromocitoma/cirugía , Complicaciones Neoplásicas del Embarazo/cirugía , Femenino , Humanos , Hipertensión/etiología , Laparoscopía , Imagen por Resonancia Magnética , Feocromocitoma/diagnóstico por imagen , Embarazo , Complicaciones Neoplásicas del Embarazo/diagnóstico por imagen , Segundo Trimestre del Embarazo , Adulto Joven
17.
Int Braz J Urol ; 44(3): 629-633, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-29412551

RESUMEN

A 32-year-old 22-week pregnant hypertensive woman with sporadic episodes of headaches, sweating, and facial flushing was diagnosed with pheochromocytoma through biochemical and imaging tests. Perioperative management included a multidisciplinary approach, symptom stabilization with ɑ blockade followed by ß blockade, and tumor resection by laparoscopic adrenalectomy at 24 weeks gestation. The diagnosis was confirmed by histopathological examination and immunohistochemistry tests. The decision for surgical removal of the tumor was based on maternal symptoms, tumor size, gestational age, the possibility of doing a laparoscopy, and the expertise of the surgical team.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales/cirugía , Adrenalectomía/métodos , Laparoscopía/métodos , Feocromocitoma/cirugía , Complicaciones Neoplásicas del Embarazo/cirugía , Neoplasias de las Glándulas Suprarrenales/complicaciones , Adulto , Femenino , Edad Gestacional , Humanos , Hipertensión/etiología , Feocromocitoma/complicaciones , Embarazo , Resultado del Embarazo , Reproducibilidad de los Resultados , Resultado del Tratamiento
18.
Ann Surg ; 268(1): 172-178, 2018 07.
Artículo en Inglés | MEDLINE | ID: mdl-28257320

RESUMEN

OBJECTIVE: To determine whether primary tumor resection in patients with metastatic pheochromocytoma or paraganglioma (PPG) is associated with longer overall survival (OS). BACKGROUND: Patients with metastatic PPG have poor survival outcomes. The impact of surgical resection of the primary tumor on OS is not known. METHODS: We retrospectively studied patients with metastatic PPG treated at the University of Texas, MD Anderson Cancer Center from January 2000 through January 2015. Kaplan-Meier analysis with log-rank tests was used to compare OS among patients undergoing primary tumor resection and patients not treated surgically. Propensity score method was applied to adjust for selection bias using demographic, clinical, biochemical, genetic, imaging, and pathologic information. RESULTS: A total of 113 patients with metastatic PPG were identified. Eighty-nine (79%) patients had surgery and 24 (21%) patients did not. Median OS was longer in patients who had surgery than in patients who did not [148 months, 95% confidence interval (CI) 112.8-183.2 months vs 36 months, 95% CI 27.2-44.8 months; P < 0.001].Fifty-three (46%) patients had synchronous metastases; of these patients, those who had surgery had longer OS than those who did not (85 months, 95% CI 64.5-105.4 months vs 36 months, 95% CI 29.7-42.3 months; P < 0.001). Patients who had surgery had a similar ECOG performance status to the ones who did not (P = 0.1798, two sample t test; P = 0.2449, Wilcoxon rank sum test). Univariate and propensity score analysis confirmed that patients treated with surgery had longer OS than those not treated surgically irrespective of age, race, primary tumor size and location, number of metastatic sites, and genetic background (log-rank P < 0.001).In patients with hormonally active tumors (70.8%), the symptoms of catecholamine excess improved after surgery. However, the tumor burden was a more important determinant of OS than hormonal secretion. CONCLUSIONS: Primary tumor resection in patients with metastatic PPG appeared to be associated with improved OS. In patients with hormonally active tumors, surgical resection led to better blood pressure control.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales/cirugía , Paraganglioma/cirugía , Adolescente , Neoplasias de las Glándulas Suprarrenales/mortalidad , Neoplasias de las Glándulas Suprarrenales/patología , Adulto , Anciano , Niño , Femenino , Humanos , Masculino , Persona de Mediana Edad , Metástasis de la Neoplasia , Paraganglioma/mortalidad , Paraganglioma/patología , Feocromocitoma/mortalidad , Feocromocitoma/patología , Feocromocitoma/cirugía , Calidad de Vida , Estudios Retrospectivos , Análisis de Supervivencia , Adulto Joven
19.
Nosso clínico ; 20(120): 32-34, nov.-dez. 2017. ilus
Artículo en Portugués | VETINDEX | ID: biblio-1486010

RESUMEN

Foi atendida uma fêmea, canina, de raça Chow Chow, com oitos anos de idade com histórico de anorexia há dois dias. Apresentou ao exame físico pelo opaco, foi solicitado hemograma que apresentou anemia,hiperproteinemia e eosinofilia, foi realizado exame bioquímico ALT, FA, ureia e creatinina que não obtiveram alterações.Na ultrassonografia abdominal encontrou-se adenomegalia por hiperadrenocorticismo devido a neoplasia em glândula adrenal direita. Sendo indicado como tratamento adrenalectomia unilateral direita, no exame histopatológico foi caracterizado o Feocromocitoma maligno. O feocromocitoma maligno é um tumor característico de glândula adrenal, ocorre normalmente de forma unilateral. Os tumores adrenocorticais são responsáveis por 15 a 20% do hiperadrenocorticismo (HAC) espontâneo em cães, sendo mais comuns os adenomas e os carcinomas.


An eight-year-old female, canine Chow Chow breed with an anorexia history of two days old was seen.He was submitted to a physical examination for the opaque, he was asked for a blood count that presented anemia,hyperproteinemia and eosinophilia, a biochemical ALT, AF, urea and creatinine test were performed, which did not change. In abdominal ultrasonography adenomegaly was observed due to hyperadrenocorticism due to neoplasia in the right adrenal gland. Being indicated as a right unilateral adrenalectomy treatment, histopathological examination characterized the malignant pheochromocytoma.Malignant pheochromocytoma is a tumor characteristic of the adrenal gland, usually occurs unilaterally. Adrenocortical tumors account for 15 to 20% of spontaneous hyperadrenocorticism (HAC) in dogs, with adenomas and carcinomas being more common.


La edad de ocho años de edad, canina Chow Chow crianza con una anorexia historia de dos días de edad. En el caso de que se produzca una hemorragia, la hiperproteinemia y la eosinophilia, el bioquímico ALT, el AF, la urea y la creatinina test fueron realizadas, que no cambió. In abdominal ultrasonografía adenomegaly fue observado debido a hiperadrenocorticismo debido a la neoplasia en el derecho adrenal gland. El uso de las a la derecha unilateral adrenalectomy del tratamiento, el histopathological examina el maligno pheochromocytoma.Malignantpheochromocytoma es un tumor característico de la adrenal gland, generalmente ocurre unilateralmente.Adrenocortical tumors account para 15 a 20% de espontaneous hyperadrenocorticism (HAC) en perros, con adenomas y carcinomas ser más comunes.


Asunto(s)
Animales , Perros , Adrenalectomía/veterinaria , Feocromocitoma/cirugía , Feocromocitoma/veterinaria , Hiperfunción de las Glándulas Suprarrenales/veterinaria , Síndrome de Cushing/veterinaria
20.
Nosso Clín. ; 20(120): 32-34, nov.-dez. 2017. ilus
Artículo en Portugués | VETINDEX | ID: vti-728642

RESUMEN

Foi atendida uma fêmea, canina, de raça Chow Chow, com oitos anos de idade com histórico de anorexia há dois dias. Apresentou ao exame físico pelo opaco, foi solicitado hemograma que apresentou anemia,hiperproteinemia e eosinofilia, foi realizado exame bioquímico ALT, FA, ureia e creatinina que não obtiveram alterações.Na ultrassonografia abdominal encontrou-se adenomegalia por hiperadrenocorticismo devido a neoplasia em glândula adrenal direita. Sendo indicado como tratamento adrenalectomia unilateral direita, no exame histopatológico foi caracterizado o Feocromocitoma maligno. O feocromocitoma maligno é um tumor característico de glândula adrenal, ocorre normalmente de forma unilateral. Os tumores adrenocorticais são responsáveis por 15 a 20% do hiperadrenocorticismo (HAC) espontâneo em cães, sendo mais comuns os adenomas e os carcinomas.(AU)


An eight-year-old female, canine Chow Chow breed with an anorexia history of two days old was seen.He was submitted to a physical examination for the opaque, he was asked for a blood count that presented anemia,hyperproteinemia and eosinophilia, a biochemical ALT, AF, urea and creatinine test were performed, which did not change. In abdominal ultrasonography adenomegaly was observed due to hyperadrenocorticism due to neoplasia in the right adrenal gland. Being indicated as a right unilateral adrenalectomy treatment, histopathological examination characterized the malignant pheochromocytoma.Malignant pheochromocytoma is a tumor characteristic of the adrenal gland, usually occurs unilaterally. Adrenocortical tumors account for 15 to 20% of spontaneous hyperadrenocorticism (HAC) in dogs, with adenomas and carcinomas being more common.(AU)


La edad de ocho años de edad, canina Chow Chow crianza con una anorexia historia de dos días de edad. En el caso de que se produzca una hemorragia, la hiperproteinemia y la eosinophilia, el bioquímico ALT, el AF, la urea y la creatinina test fueron realizadas, que no cambió. In abdominal ultrasonografía adenomegaly fue observado debido a hiperadrenocorticismo debido a la neoplasia en el derecho adrenal gland. El uso de las a la derecha unilateral adrenalectomy del tratamiento, el histopathological examina el maligno pheochromocytoma.Malignantpheochromocytoma es un tumor característico de la adrenal gland, generalmente ocurre unilateralmente.Adrenocortical tumors account para 15 a 20% de espontaneous hyperadrenocorticism (HAC) en perros, con adenomas y carcinomas ser más comunes.(AU)


Asunto(s)
Animales , Perros , Adrenalectomía/veterinaria , Feocromocitoma/cirugía , Feocromocitoma/veterinaria , Hiperfunción de las Glándulas Suprarrenales/veterinaria , Síndrome de Cushing/veterinaria
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