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1.
In. Soeiro, Alexandre de Matos; Leal, Tatiana de Carvalho Andreucci Torres; Accorsi, Tarso Augusto Duenhas; Gualandro, Danielle Menosi; Oliveira Junior, Múcio Tavares de; Caramelli, Bruno; Kalil Filho, Roberto. Manual da residência em cardiologia / Manual residence in cardiology. Santana de Parnaíba, Manole, 2 ed; 2022. p.728-736, tab, ilus.
Monography in Portuguese | LILACS | ID: biblio-1353103
2.
Rev. baiana saúde pública ; 45(Supl. Especial 2): 108-117, 2021/12/28.
Article in Portuguese | LILACS | ID: biblio-1352339

ABSTRACT

As neoplasias endócrinas múltiplas (NEM) são síndromes genéticas autossômicas dominantes implicadas no desenvolvimento de neoplasias benignas ou malignas, envolvendo ao menos duas glândulas endócrinas. Entre seus subtipos, está a NEM2A, que consiste em carcinoma medular de tireoide (CMT), feocromocitoma e hiperparatireoidismo. Este texto apresente o relato de caso de um paciente de 40 anos, previamente hígido, que passou a apresentar episódios de cefaleia associada a sudorese profusa, vômitos e taquicardia. Evoluiu com distensão abdominal intensa após alimentação por via oral, perda ponderal, desnutrição, astenia, obstipação, humor deprimido e picos pressóricos. Exames laboratoriais evidenciaram alterações dos hormônios tireoidianos, PTH e hormônios da adrenal. Foi levantada a suspeita clínica de NEM2A, posteriormente corroborada pelos diagnósticos anatomopatológicos de feocromocitoma e CMT, associados à presença de hiperparatireoidismo. Foi possível concluir que, a despeito de sua baixa prevalência na população geral, a NEM é uma síndrome clínica de grande relevância, tendo em vista os impactos para os pacientes e famílias acometidas. Dessa forma, é necessário que os profissionais de saúde tenham conhecimento acerca da síndrome e que o Sistema Único de Saúde (SUS) esteja apto a assistir aos pacientes portadores de NEM, possibilitando diagnóstico precoce e tratamento adequado.


Multiple endocrine neoplasias (MEN) are dominant autosomal genetic syndromes involved in the development of benign or malignant tumors in at least two endocrine glands. MEN2A is one of its subtypes, which consists of medullary thyroid carcinoma (MTC), pheochromocytoma, and hyperparathyroidism. This study reports the case of a healthy 40-year-old male patient presenting with episodes of headache associated with profuse sweating, vomiting, and tachycardia. The patient evolved with severe abdominal distension after oral feeding, weight loss, malnutrition, asthenia, constipation, depressed mood, and pressure peaks. Laboratory tests showed abnormalities in thyroid, parathyroid (PTH), and adrenal hormones ­ thus raising the hypothesis of MEN2A, which was later corroborated by the histological diagnosis of pheochromocytoma and MTC, associated with hyperparathyroidism. The results indicate that, despite its low prevalence in the general population, MEN has a great impact on affected patients and families, thus being a relevant clinical syndrome. For enabling early diagnosis and adequate treatment, health professionals must be familiarized with such a condition, and the Brazilian Unified Health System (SUS) must be able to assist affected patients.


Las neoplasias endocrinas múltiples (NEM) son síndromes genéticos autosómicos dominantes involucrados en el desarrollo de neoplasias benignas o malignas, que afectan al menos dos glándulas endocrinas. Entre sus subtipos se encuentra NEM2A, que consiste en carcinoma medular de tiroides (CMT), feocromocitoma e hiperparatiroidismo. Este es un reporte de caso de un paciente de 40 años de edad, previamente sano, que comenzó a presentar episodios de cefalea asociada a sudoración profusa, vómitos y taquicardia. Evolucionó con distensión abdominal severa después de alimentarse, pérdida de peso, desnutrición, astenia, estreñimiento, estado de ánimo deprimido y picos de presión. Las pruebas de laboratorio mostraron alteraciones en las hormonas tiroideas, PTH y hormonas suprarrenales. Se planteó la hipótesis de MEN2A, posteriormente corroborada por el diagnóstico anatomopatológico de feocromocitoma y CMT, asociado a hiperparatiroidismo. Se pudo concluir que, a pesar de su baja prevalencia en la población general, el NEM es un síndrome clínico de gran relevancia, dado el impacto que tiene en los pacientes y familiares afectados. Por tanto, es necesario que los profesionales sanitarios tengan conocimiento sobre el síndrome y que el Sistema Único de Salud (SUS) sea capaz de asistir a los pacientes con NEM, posibilitándoles diagnóstico precoz y tratamiento adecuado.


Subject(s)
Humans , Pheochromocytoma , Multiple Endocrine Neoplasia , Thyroid Neoplasms , Carcinoma, Medullary , Endocrine Glands , Hyperparathyroidism
3.
Arch. argent. pediatr ; 119(6): 401-407, dic. 2021. tab
Article in English, Spanish | LILACS, BINACIS | ID: biblio-1342846

ABSTRACT

Introducción. Los ensayos clínicos cooperativos han aumentado el conocimiento sobre los tumores pediátricos; sin embargo, no es el caso de los tumores raros (TR). Objetivo. Describir prevalencia, características clínicas y evolución de los TR en la edad pediátrica diagnosticados en el Hospital Garrahan. Material y métodos. Estudio descriptivo retrospectivo de pacientes entre 0 y 18 años con diagnóstico de TR ingresados entre enero de 2007 y diciembre de 2017.Resultados. De 1 657 pacientes con diagnóstico de tumores sólidos, 164 (9,9 %) correspondieron a TR, 71,95 % (118) eran menores de 14 años y 81,7 % (130) eran varones. En orden de frecuencia, los TR fueron: carcinoma de tiroides (60), carcinoma suprarrenal (14), tumores pulmonares (14), melanoma (13), carcinoma de glándulas salivales (11), tumores gastrointestinales (8), tumores gonadales no germinales (7), tumores pancreáticos (7), carcinomas renales (6), carcinomas nasofaríngeos (5), feocromocitoma y paraganglioma (5) y carcinoma de timo en 1 paciente. El tratamiento recibido dependió del tipo de tumor y del estadio. Con una mediana de seguimiento de 34,9 meses (rango: 1-128,5 meses), 133 pacientes (78,7 %) están vivos y solo 10 pacientes (6 %) se perdieron durante el seguimiento. Conclusión. La prevalencia de TR fue del 9,9 %. El 27 % se presentaron en adolescentes. Los tumores más frecuentemente diagnosticados fueron carcinoma de tiroides, carcinoma suprarrenal y melanoma. El tratamiento y la evolución varió según el tipo histológico. Se hallaron alteraciones moleculares predisponentes en el 5,3 % de los pacientes, el 3,5 % tenían antecedente de patología oncológica.


Introduction. Collaborative clinical trials have enlarged the knowledge base about pediatric tumors; however, this is not the case for rare tumors (RT). Objective. To describe the prevalence, clinical characteristics, and course of RT in pediatric patients diagnosed at Hospital Garrahan. Material and methods. Descriptive, retrospective study of patients aged 0-18 years diagnosed with a RT and admitted between January 2007 and December 2017. Results. Out of 1657 patients diagnosed with solid tumors, 164 (9.9 %) were RT; 71.95 % (118) of patients were younger than 14 years and 81.7 % (130) were males. In order of frequency, RT were thyroid carcinoma (60), adrenal carcinoma (14), lung tumors (14), melanoma (13), salivary gland cancer (11), gastrointestinal tumors (8), non-germ cell gonadal tumors (7), pancreatic tumors (7), renal carcinomas (6), nasopharyngeal carcinomas (5), pheochromocytoma and paraganglioma (5), and thymic carcinoma in 1 patient. Treatment depended on tumor type and stage. The median follow-up was 34.9 months (range: 1-128.5 months);133 patients (78.7 %) are alive and only 10 patients (6 %) were lost-to-follow-up. Conclusion. The prevalence of RT was 9.9 %. Twenty-seven percent occurred in adolescents. The most frequent tumors included thyroid carcinoma, adrenal carcinoma, and melanoma. Treatment and course varied based on tumor histology. Predisposing molecular alterations were found in 5.3 % of patients; 3.5 % had a history of cancer.


Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Pediatrics , Pheochromocytoma , Adrenal Gland Neoplasms , Argentina/epidemiology , Epidemiology, Descriptive , Prevalence , Retrospective Studies
4.
Rev. colomb. anestesiol ; 49(3): e301, July-Sept. 2021. tab
Article in English | LILACS, COLNAL | ID: biblio-1280180

ABSTRACT

Abstract Pheochromocytomas are rare neuroendocrine neoplasms that require adequate preoperative evaluation in order to prevent and lessen the serious complications of catecholamine hypersecretion. Preoperative management contributes to reducing morbidity and mortality rates in patients who have not been diagnosed with this condition and undergo any surgery. However, current mortality seems to be lower, a fact attributed to preoperative management with alpha blockers.


Resumen Los feocromocitomas son neoplasias neuroendocrinas poco frecuentes que requieren una evaluación preoperatoria adecuada, con el fin de prevenir y disminuir las complicaciones graves de la hipersecreción de catecolaminas. El manejo preoperatorio contribuye a disminuir las tasas de morbimortalidad en los pacientes que no han sido diagnosticados con esta entidad y son sometidos a cualquier cirugía. Sin embargo, la mortalidad actual parece ser más baja, hecho atribuido a un manejo preoperatorio con α-bloqueadores.


Subject(s)
Humans , Male , Female , Paraganglioma , Pheochromocytoma , Surgical Clearance , Neoplasms , Postoperative Care , Catecholamines , Indicators of Morbidity and Mortality , Morbidity , Mortality
5.
Rev. cuba. endocrinol ; 32(1): e258, 2021. tab
Article in Spanish | LILACS, CUMED | ID: biblio-1289387

ABSTRACT

Introducción: Los feocromocitomas son tumores que se desarrollan a partir de las células cromafines localizadas en la médula suprarrenal, ganglios simpáticos y parasimpáticos. La distinción entre tumores benignos y malignos es compleja, pues no existen marcadores que puedan discriminar esta diferencia con alta sensibilidad y especificidad. Objetivo: Presentar tres casos clínicos de feocromocitoma maligno, diagnosticados y/o tratados en la sala de hospitalización de adultos del INEN. Método: Se revisaron las historias clínicas y se resumieron los datos. Presentación de los casos: Los 3 pacientes eran del sexo masculino, con edades entre 41 y 51 años al diagnóstico del tumor primario. De ellos, dos tenían una hipertensión arterial controlada y el tercero se comenzó a estudiar por crisis paroxísticas de hipertensión; dos tenían antecedentes de diabetes mellitus y no se demostraron evidencias en ningún caso de lesiones en órganos diana por la hipertensión. En los tres pacientes el tumor se localizó en la suprarrenal derecha. Solo en uno de los casos, la etiología maligna fue informada en el estudio anatomopatológico. En los otros dos pacientes, la malignidad se diagnosticó a punto de partida de lesiones metastásicas, años después de la adrenalectomía, en un caso de cadera y en el otro, de pulmón. Conclusiones: El feocromocitoma maligno es una enfermedad infrecuente de la edad mediana y el diagnóstico de su naturaleza es complejo. Se debe sospechar la aparición de enfermedad metastásica ante la reaparición de los síntomas que motivaron el estudio inicial en el paciente, incluso, años después de la adrenalectomía(AU)


Introduction: Pheochromocytomas are tumors developed from chromaffin cells located in the suprarenal medulla, as well as in sympathetic and parasympathetic ganglia. The distinction between benign and malignant tumors is complex, since there are no markers to discriminate this difference with high sensitivity and specificity. Objective: To present three clinical cases of malignant pheochromocytoma, diagnosed and/or treated in the adult hospitalization ward at the National Institute of Endocrinology. Method: The medical records were reviewed and the data was summarized. Case presentation: The three patients were male and aged between 41 and 51 years at the diagnosis of the primary tumor. Of them, two had controlled arterial hypertension and the third began to be studied for paroxysmal hypertension crises; two had a history of diabetes mellitus and no evidence of target-organ lesions due to hypertension was shown in any case. In the three patients, the tumor was located in the right suprarenal gland. Only in one of the cases, malignant etiology was reported in the pathological study. In the other two patients, the malignancy was diagnosed at the starting point of metastatic lesions, years after adrenalectomy: two respective hip and lung cases. Conclusions: Malignant pheochromocytoma is a rare disease that appears at middle age and whose diagnosis is complex, due to its etiological nature. The appearance of metastatic disease should be suspected, given the reappearance of the symptoms that motivated the initial study in the patient, even years after adrenalectomy(AU)


Subject(s)
Humans , Male , Adult , Middle Aged , Pheochromocytoma/diagnosis , Sensitivity and Specificity , Adrenal Gland Neoplasms/epidemiology , Adrenalectomy/methods , Rare Diseases/etiology , Neoplasm Metastasis/physiopathology
6.
Rev. bras. anal. clin ; 52(4): 395-399, 20201230. tab
Article in Portuguese | LILACS | ID: biblio-1248361

ABSTRACT

Pheochromocytomas are tumors of chromaffin cells in the adrenal medulla that produce, store, metabolize, and secrete catecholamines. Due to increased secretion the patient may present with various symptoms, but elevation of blood pressure is the most common manifestation, and can cause serious complications if not recognized and treated in time. The present report mentions a case of a young male patient who started the picture with severe hypertension, tachycardia and headache, diagnosed with Pheochromocytoma during the investigation of paroxysmal arterial hypertension. It should be noted that early diagnosis, followed by surgical treatment of tumor removal, makes it possible to cure the disease and remission of symptoms.


Os feocromocitomas são tumores das células cromafins da medula adrenal que produzem, armazenam, metabolizam e secretam catecolaminas. Devido à secreção aumentada, o paciente pode apresentar vários sintomas, porém a elevação da pressão arterial é a manifestação mais comum e pode causar sérias complicações se não for reconhecida e tratada a tempo. O presente relato menciona o caso de paciente do sexo masculino, jovem, com início de quadro com hipertensão severa, taquicardia e cefaleia, sendo diagnosticado com feocromocitoma durante a investigação do quadro de hipertensão arterial paroxística. Ressalva-se que o diagnóstico precoce seguido do tratamento cirúrgico de retirada do tumor possibilita a cura da patologia e a remissão dos sintomas.


Subject(s)
Humans , Male , Middle Aged , Pheochromocytoma , Catecholamines , Arterial Pressure
7.
Rev. colomb. anestesiol ; 48(4): e400, Oct.-Dec. 2020. tab, graf
Article in English | LILACS, COLNAL | ID: biblio-1144318

ABSTRACT

Abstract Introduction Laparoscopic adrenalectomy is the treatment of choice of pheochromocytoma. During the first surgical phase (pneumoperitoneum insufflation, tumor and veins handling), there is a risk of hypertensive crisis due to catecholamine release. After tumor excision, patients can suffer relative vasodilation and the residual effect of antihypertensive drugs, which results in arterial hypotension. For that reason, antihypertensive drugs used in the first phase should have a rapid onset of action, short half-life and no residual effect. Methods We report a series of three cases of patients with pheochromocytoma who were treated with laparoscopic adrenalectomy. They all received clevidipine infusion from the beginning of the surgery, before they had presented hypertension, to treat and try to minimize hypertensive peaks. Results In all patients, hypertensive peaks were controlled in a few minutes. After tumor resection, clevidipine infusion was stopped in all cases, and any patient required infusion of vasopressors. Discussion Clevidipine could be a first choice antihypertensive drug in pheochromocytoma surgery. Starting the infusion of clevidipine before the hypertensive peaks could help to make them less pronounced.


Resumen Introducción La adrenalectomía laparoscópica es el tratamiento de elección del feocromocitoma. Durante la primera fase quirúrgica (insuflación de neumoperitoneo, manipulación del tumor y de las venas implicadas), existe el riesgo de que se desencadenen crisis hipertensivas debido a la liberación de catecolaminas. Después de la extirpación del tumor, los pacientes pueden sufrir una vasodilatación relativa y el efecto residual de los fármacos antihipertensivos usados previamente, lo que resulta en hipotensión arterial. Por esa razón, los fármacos antihipertensivos utilizados en la primera fase quirúrgica deben tener rápido inicio de acción, vida media corta y mínimo efecto residual. Métodos Se describe una serie de casos de tres pacientes con feocromocitoma que fueron tratados con adrenalectomía laparoscópica. Todos recibieron infusión de clevidipino desde el comienzo de la cirugía, antes de presentar hipertensión arterial, para así intentar minimizar y tratar rápidamente los posibles picos hipertensivos. Resultados En todos los pacientes los picos hipertensivos se controlaron en pocos minutos. Después de la resección del tumor, la infusión de clevidipino se detuvo en todos los casos y ningún paciente requirió perfusión de vasopresores. Discusión El clevidipino podría ser un fármaco antihipertensivo de primera elección en la cirugía de feocromocitoma. Iniciarlo antes de que ocurran los picos hipertensivos podría ayudar a que sean más leves.


Subject(s)
Humans , Male , Female , Middle Aged , Aged , Antihypertensive Agents , Pheochromocytoma , Catecholamines , Adrenalectomy , Hypotension
8.
Rev. méd. Maule ; 36(2): 61-67, dic. 2020. tab
Article in Spanish | LILACS | ID: biblio-1344688

ABSTRACT

30 to 40% of the adult population worldwide has been diagnosed with hypertension, among these patients 5 to 10% of them could have a possibly curable condition. In order to recognize this special population, the clinician must perform a complete work up and be aware of the main underlying causes of secondary hypertension. Often this could be a goal difficult to accomplish. The purpose of this article is to discuss the most frequent causes of secondary hypertension and offer a diagnostic approach for these patients. Clinicians should never forget that drug-related hypertension is a common cause that is discovered only with the help of a good medical history.


Subject(s)
Humans , Hypertension/prevention & control , Hypertension, Renovascular/etiology , Pheochromocytoma , Sleep Apnea Syndromes , Blood Pressure Monitoring, Ambulatory , Hyperaldosteronism , Hypertension/diagnosis , Hypertension/etiology , Hypertension, Renovascular/diagnosis , Antihypertensive Agents/therapeutic use
9.
Medwave ; 20(2): e7830, 31-03-2020.
Article in English, Spanish | LILACS | ID: biblio-1097789

ABSTRACT

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.


Subject(s)
Humans , Paraganglioma/surgery , Pheochromocytoma/surgery , Adrenal Gland Neoplasms/surgery , Laparoscopy
10.
Article in English | WPRIM | ID: wpr-816625

ABSTRACT

No abstract available.


Subject(s)
Epidemiology , Korea , Paraganglioma , Pheochromocytoma
11.
Article in English | WPRIM | ID: wpr-816617

ABSTRACT

BACKGROUND: Pheochromocytomas and paragangliomas (PPGLs) are rare endocrine tumors originating from chromaffin cells. PPGLs are associated with a high mortality rate and several complications. To date, no epidemiological studies have been conducted on PPGLs in Asia. This study aimed to investigate the epidemiology and prognosis of PPGLs in Korea using nationwide data.METHODS: Using the National Health Insurance Service Database, subjects with a principal diagnosis of PPGLs on two or more occasions between 2003 and 2014 who satisfied the operational definition of PPGLs were included. Incidence, prevalence, complications, metastasis, and mortality were investigated.RESULTS: In total, 1048 subjects with a mean age of 47.6±16.1 years were included. There was no sex preponderance. The overall prevalence of PPGLs was 2.13 per 100,000 persons, and the overall age-standardized incidence rate was 0.18 per 100,000 person-years. Malignant PPGLs accounted for 17.7% (185 of 1,048) of cases, and 94 subjects exhibited metastasis at the time of diagnosis. Among initially non-metastatic PPGLs, 9.5% (nine of 954) eventually metastasized after a mean duration of 78.1±41.4 months. The 5-year survival rates for non-metastatic and metastatic PPGLs at diagnosis were 97% and 84%, respectively. Multivariable Cox regression models adjusted for covariates showed that metastatic PPGLs were associated with a 2.40-fold higher risk of mortality than non-metastatic PPGLs (95% confidence interval, 1.38 to 4.17; P=0.002).CONCLUSION: PPGLs are rare in Korea, and the prognosis of these endocrine tumors varies depending on whether they are benign or malignant. This epidemiological study paves the way for further research on PPGLs.


Subject(s)
Asia , Chromaffin Cells , Diagnosis , Epidemiologic Studies , Epidemiology , Humans , Incidence , Korea , Mortality , National Health Programs , Neoplasm Metastasis , Paraganglioma , Pheochromocytoma , Prevalence , Prognosis , Survival Rate
13.
Rev. cuba. cir ; 58(3): e851, jul.-set. 2019. tab, graf
Article in Spanish | LILACS, CUMED | ID: biblio-1098973

ABSTRACT

RESUMEN Introducción: D esde el año 1997 se comenzó a realizar la adrenalectomía laparoscópica en nuestro centro. Objetivo: Analizar los resultados de la adrenalectomía laparoscópica en el tratamiento quirúrgico del feocromocitoma en el servicio de cirugía general del Hospital Clínico Quirúrgico "Hermanos Ameijeiras". Métodos: Desde noviembre de 1997 a junio del año 2019 se realizaron 192 adrenalectomías por vía laparoscópica en 190 pacientes y en 41 fueron realizadas por feocromocitoma adrenal, en el servicio de cirugía general del Hospital Clínico Quirúrgico "Hermanos Ameijeiras". Se empleó la técnica laparoscópica con abordaje lateral intraperitoneal en la mayoría de los casos, y el abordaje en decúbito supino en un caso para la adrenalectomía bilateral. Resultados: Se realizaron 41 adrenalectomías laparoscópicas en 40 pacientes por feocromocitoma adrenal, en 1 pacientes se realizó adrenalectomía bilateral en un tiempo. La edad promedio fue de 44 años. Predominaron las lesiones del lado derecho en 26 pacientes, 14 del lado izquierdo y uno bilateral. Fueron convertidos a cirugía convencional 1 pacientes (0,41 por ciento). El tiempo quirúrgico promedio fue de 80 minutos. La estadía postoperatoria promedio fue de 2,5 días. Todos los pacientes se curaron de la hipertensión arterial. Conclusiones: La adrenalectomía laparoscópica es una técnica reproducible y segura en el tratamiento del feocromocitoma adrenal(AU)


ABSTRACT Introduction: Since 1997, laparoscopic adrenalectomy began to be performed in our center. Objective: To analyze the results of laparoscopic adrenalectomy in the surgical treatment of pheochromocytoma in the general surgery service of the "Hermanos Ameijeiras" Surgical Clinical Hospital. Methods: From November 1997 to June 2019, 192 adrenalectomies were performed laparoscopically in 190 patients and in 41 they were performed for adrenal pheochromocytoma, in the general surgery service of the "Hermanos Ameijeiras" Surgical Clinical Hospital. The laparoscopic technique with an intraperitoneal lateral approach was used in most cases, and the supine approach in one case for bilateral adrenalectomy. Results: 41 laparoscopic adrenalectomies were performed in 40 patients for adrenal pheochromocytoma, in 1 patients bilateral adrenalectomy was performed at one time. The average age was 44 years. Lesions on the right side predominated in 26 patients, 14 on the left side and one bilateral. 1 patients (0.41 pèrcent) were converted to conventional surgery. The average surgical time was 80 minutes. The average postoperative stay was 2.5 days. All patients were cured of high blood pressure. Conclusions: Laparoscopic adrenalectomy is a reproducible and safe technique in the treatment of adrenal pheochromocytoma(AU)


Subject(s)
Humans , Female , Adult , Pheochromocytoma/etiology , Laparoscopy/methods , Adrenal Gland Neoplasms/epidemiology , Adrenalectomy/methods
14.
Arch. endocrinol. metab. (Online) ; 63(4): 369-375, July-Aug. 2019. tab, graf
Article in English | LILACS | ID: biblio-1019359

ABSTRACT

ABSTRACT Objective Paraganglioma (PGL) and pheochromocytoma (PCC) are rare neuroendocrine tumors that were considered to be predominantly sporadic. However, with the identification of novel susceptibility genes over the last decade, it is currently estimated that up to 40% of cases can occur in the context of a hereditary syndrome. We aimed to characterize PGL/PCC families to exemplify the different scenarios in which hereditary syndromes can be suspected and to emphasize the importance for patients and their families of making an opportune genetic diagnosis. Materials and methods Retrospective analysis of patients diagnosed with PGL/PCC. Germline mutations were studied using next-generation sequencing panels including SDHA, SDHB, SDHC and SDHD. Clinical data were collected from clinical records, and all patients received genetic counseling. Results We describe 4 families with PGL/PCC and germline mutations in SDH complex genes. 2 families have SDHB mutations and 2 SDHD mutations. The clinical presentation of the patients and their families was heterogeneous, with some being atypical according to the literature. Conclusions PGL/PCC are more commonly associated with a germline mutation than any other cancer type, therefore, all individuals with these types of tumors should undergo genetic risk evaluation. NGS multigene panel testing is a cost-effective approach given the overlapping phenotypes. Individuals with germline mutations associated with PGL/PCC should undergo lifelong clinical, biochemical and imaging surveillance and their families should undergo genetic counseling. For all these reasons, it is critical that all medical staff can suspect and diagnose these inherited cancer predisposition syndromes.


Subject(s)
Humans , Male , Female , Paraganglioma/genetics , Pheochromocytoma/genetics , Adrenal Gland Neoplasms/genetics , Germ-Line Mutation/genetics , Pedigree , Genetic Testing/methods , Retrospective Studies , Sentinel Surveillance , Genetic Predisposition to Disease
15.
Article in English | WPRIM | ID: wpr-786457

ABSTRACT

A 36-year-old male patient initially presented with hypertension, tinnitus, bilateral carotid masses, a right jugular foramen, and a periaortic arch mass with an elevated plasma dopamine level but an otherwise normal biochemical profile. On surveillance MRI 4 years after initial presentation, he was found to have a 2.2-cm T2 hyperintense lesion with arterial enhancement adjacent to the gallbladder, which demonstrated avidity on ⁶⁸Ga-DOTATATE PET/CTand retrospectively on ¹⁸F-FDOPA PET/CT but was nonavid on ¹⁸F-FDG PET/CT. Biochemical work-up including plasma catecholamines, metanephrines, and chromogranin A levels were found to be within normal limits. This lesion was surgically resected and was confirmed to be a paraganglioma (PGL) originating from the gallbladder wall on histopathology. Pheochromocytoma (PHEO) and PGL are rare tumors of the autonomic nervous system. Succinate dehydrogenase subunit D (SDHD) pathogenic variants of the succinate dehydrogenase complex are usually involved in parasympathetic, extra-adrenal, multifocal head, and neck PGLs. We report an unusual location of PGL in the gallbladder associated with SDHD mutation which could present as a potential pitfall on ¹⁸F-FDOPA PET/CT as its normal excretion occurs through biliary system and gallbladder. This case highlights the superiority of ⁶⁸Ga-DOTATATE in comparison to ¹⁸F-FDOPA and ¹⁸F-FDG in the detection of SDHD-related parasympathetic PGL.ClinicalTrials.gov Identifier: NCT00004847.


Subject(s)
Adult , Autonomic Nervous System , Biliary Tract , Catecholamines , Chromogranin A , Dopamine , Gallbladder , Head , Humans , Hypertension , Magnetic Resonance Imaging , Male , Neck , Paraganglioma , Pheochromocytoma , Plasma , Positron Emission Tomography Computed Tomography , Retrospective Studies , Succinate Dehydrogenase , Tinnitus
16.
Article in English | WPRIM | ID: wpr-785323

ABSTRACT

Pheochromocytoma (PCC) is a rare catecholamine-producing tumor with the incidence in hypertension of 0.1-0.6%. PCC crisis is an endocrine emergency that can lead to hemodynamic disturbance and organ failure such as catecholamine-induced cardiomyopathy. The circulatory collapse caused by it often requires mechanical support. The author reports an unusual case in which a patient who previously underwent surgery for malignant PCC developed catecholamine-induced cardiomyopathy, and successfully recovered using extracorporeal membrane oxygenation.


Subject(s)
Cardiomyopathies , Emergencies , Extracorporeal Membrane Oxygenation , Hemodynamics , Humans , Hypertension , Incidence , Pheochromocytoma , Shock , Shock, Cardiogenic
17.
Article in Chinese | WPRIM | ID: wpr-775980

ABSTRACT

Objective To analyze the clinical features of Von-Hippel-Lindau(VHL)syndrome and explore the diagnostic value of abdominal ultrasound for this disease.Methods The clinical features including age at first diagnosis,symptoms,signs,affected organs,number of operations,and diagnostic examinations of 35 patients with VHL syndrome admitted to our center from January 1994 to December 2017 were retrospectively analyzed.The diagnostic value of abdominal ultrasound for VHL syndrome was analyzed.Results Pheochromocytoma(=14)and nervous system hemangioblastoma(=13)were the common firstly-identified tumors.Nervous system hemangioblastoma(=21),pheochromocytoma(=19),renal carcinoma(=17),and pancreatic mass(=15)were common tumors.The main surgical reasons were nervous system hemangioblastoma(=22),pheochromocytoma(=23)and renal carcinoma(=13).Abdominal organ involvements were found in 33 patients,which were first detected by abdominal ultrasound in 20 patients and were found accidently during routine health checkups in 6 patients.The ultrasound results were accurate in 27 of 33 adrenal gland scans,13 of 16 pancreas scans,and 8 of 19 kidney scans.Conclusions When multiple tumors are detected in the kidney,adrenal gland,and pancreas by ultrasound,the possibility of VHL syndrome should be considered.When the clinical findings suggest the possibility of VHL syndrome,ultrasound can discover and diagnose the abdominal tumors and can also be used for the long-term follow-up of the tumors.Therefore,ultrasound is an important method in the screening and follow-up of patients with VHL syndrome.


Subject(s)
Abdomen , Diagnostic Imaging , Adrenal Gland Neoplasms , Diagnostic Imaging , Hemangioblastoma , Diagnostic Imaging , Humans , Pheochromocytoma , Diagnostic Imaging , Retrospective Studies , Ultrasonography , von Hippel-Lindau Disease , Diagnostic Imaging
18.
Article in English | WPRIM | ID: wpr-760171

ABSTRACT

Although pheochromocytoma usually presents as a solid tumor, it can also present as a cystic lesion in the adrenal glands. Cystic lesions in the adrenal glands, along with hypertension, need attention to exclude pheochromocytoma. If ignored, they may lead to a hypertensive crisis with multi-organ failure. Proper preoperative preparation is important to prevent a hypertensive crisis during and after surgery. We report a case of pheochromocytoma with cystic degeneration that presented as a hypertensive crisis caused by endoscopic ultrasound-guided tissue sampling.


Subject(s)
Adrenal Glands , Biopsy, Fine-Needle , Endosonography , Hypertension , Pheochromocytoma
19.
Int. braz. j. urol ; 44(5): 1032-1035, Sept.-Oct. 2018. graf
Article in English | LILACS | ID: biblio-975632

ABSTRACT

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.


Subject(s)
Humans , Female , Pregnancy , Young Adult , Pheochromocytoma/surgery , Pregnancy Complications, Neoplastic/surgery , Pheochromocytoma/diagnostic imaging , Pregnancy Complications, Neoplastic/diagnostic imaging , Pregnancy Trimester, Second , Magnetic Resonance Imaging , Laparoscopy , Hypertension/etiology
20.
Rev. bras. cir. cardiovasc ; 33(5): 522-524, Sept.-Oct. 2018. tab, graf
Article in English | LILACS | ID: biblio-977446

ABSTRACT

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.


Subject(s)
Humans , Male , Middle Aged , Pheochromocytoma/surgery , Aortic Aneurysm, Abdominal/surgery , Adrenal Gland Neoplasms/surgery , Hypertension/etiology , Pheochromocytoma/complications , Pheochromocytoma/diagnostic imaging , Tomography, X-Ray Computed , Aortic Aneurysm, Abdominal/complications , Adrenal Gland Neoplasms/complications , Adrenal Gland Neoplasms/diagnostic imaging
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