RESUMEN
El carcinoma suprarrenal es una enfermedad muy poco frecuente de presentación clínica poco específica, donde los síntomas por la propia neoplasia son los predominantes. El objetivo de esta publicación es presentar un caso clínico jerarquizando los estudios de imagen para el diagnóstico y la planificación de la táctica para la cirugía.
Adrenal carcinoma is a very rare disease with nonspecific clinical presentation, where symptoms from the neoplasia itself are predominant. The aim of this publication is to present an uncommon clinical case, emphasizing the role of imaging studies in diagnosis and surgical strategy planning.
O carcinoma adrenal é uma doença muito rara, com apresentação clínica inespecífica onde predominam os sintomas decorrentes da própria neoplasia. O objetivo desta publicação é apresentar um caso clínico infrequente priorizando exames de imagem para diagnóstico e planejamento de táticas cirúrgicas.
Asunto(s)
Carcinoma , Neoplasias de las Glándulas Suprarrenales/cirugía , Neoplasias de las Glándulas Suprarrenales/diagnóstico por imagenRESUMEN
INTRODUCTION: In patients with history of cancer adrenal metastases can be found in up to 70% of adrenal tumors detected during follow-up. Currently, laparoscopic adrenalectomy (LA) is considered the gold standard approach for benign adrenal tumors but is still controversial in malignant disease. Depending on the patient's oncological status, adrenalectomy might be a possible treatment option. Our objective was to analyze the results of LA for adrenal metastasis from solid tumors in two referral centers. METHODS: Retrospective analysis of 17 patients with non-primary adrenal malignancy treated with LA between 2007 and 2019 was performed. Demographic and primary tumor data, type of metastasis, morbidity, disease recurrence and evolution were evaluated. Patients were compared according to type of metastases: synchronous (< 6 months) vs metachronous (≥ 6 months). RESULTS: 17 patients were included. Median metastatic adrenal tumor size was 4 cm (IQR, 3-5.4). We had one conversion to open surgery. Recurrence was found in 6 patients with one recurring in the adrenal bed. The median OS was 24 (IQR, 10.5-60.5) months and 5-year OS was 61.4% (95%CI: 36.7%-81.4%). Patients with metachronous metastases had better overall survival vs. patients with synchronous metastases (87% vs. 14%, p = 0.0037). CONCLUSION: LA for adrenal metastases is a procedure associated with low morbidity and acceptable oncologic outcomes. Based on our results, seems reasonable to offer this procedure to carefully selected patients, mainly those with metachronous presentation. Indication of LA must be done on a case by case evaluation in the context of a multidisciplinary tumor board.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Laparoscopía , Humanos , Adrenalectomía/métodos , Estudios Retrospectivos , Resultado del Tratamiento , Recurrencia Local de Neoplasia/cirugía , Neoplasias de las Glándulas Suprarrenales/cirugíaRESUMEN
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/cirugíaRESUMEN
PURPOSE: To analyze the role of laparoscopic surgery for the management of pediatric adrenal tumors (AT). METHODS: Retrospective analysis of children diagnosed with AT, operated laparoscopically during 2003-2020. The strategy differed according to tumor extension. AT < 6 cm were resected. Locally advanced tumors (L2) or > 6 cm were biopsied. RESULTS: N = 28. Complete tumor resection (R0) in 20 (71%), tumor biopsy in 8 (R2). Age (median): 28.8 months (2 months-18 years). 14/28 left-sided, 2 bilateral. Median operating time: 78 min (45-180). Mean tumor size (for resections): 4 cm (2.5-6). Tumor pathology: neuroblastoma (n = 17), Ganglioneuroma (n = 7), Adrenocortical carcinoma (n = 1), Osteosarcoma metastasis (n = 1), Pheochromocytoma (n = 1), Venous malformation (n = 1). Mean hospital stay: 2.5 days (1-3). Mean follow up: 65.5 months (24-192). Overall survival and event-free survival were 86 and 75%, respectively (5 years event-free survival for neuroblastoma: 33% [intermediate risk], 16.6% [high risk]. No surgery-related mortality. CONCLUSION: Laparoscopic surgery for adrenal tumors is safe. Laparoscopic biopsy is useful for unresectable tumors when a percutaneous approach is not possible. With the proposed selection criteria, the laparoscopic approach should be the first option for resection of small and localized AT in pediatrics.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Laparoscopía , Neuroblastoma , Neoplasias de las Glándulas Suprarrenales/patología , Neoplasias de las Glándulas Suprarrenales/cirugía , Adrenalectomía , Niño , Humanos , Neuroblastoma/cirugía , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: Since Gagner performed the first laparoscopic adrenalectomy in 1992, laparoscopy has become the gold-standard procedure in the treatment of adrenal surgical diseases. A review of the literature indicates that the rate of intra- and postoperative complications are not negligible. This study aims to describe the single-center experience of adrenalectomies; and explore the associations between body mass index (BMI) and tumor volume in main postoperative outcomes. METHODS: Retrospective observational study with a prospective database in which we described patients who underwent adrenalectomy between January 2015 and December 2020. Operative time, intraoperative blood loss, conversion rate, complications, length of hospital stay, and comparison of the number of antihypertensive drugs used before and after surgery were analyzed. Analysis of BMI and tumor volume with postoperative outcomes such as anti-hypertensive change (AHC) in drug usage and pre-operative conditions were performed. RESULTS: Forty-five adrenalectomies were performed, and all of them were carried out laparoscopically. Four were performed as a robot-assisted laparoscopy approach. Nineteen were women and 26 were men. Mean age was 54.9 ± 13.8 years. Mean tumor volume was 95.698 mm3 (3.75-1010.87). Mean operative time was shorter in right tumors (2.64 ± 0.75 h) than in left tumors (3.33 ± 2.73 h). Pearson correlation was performed to assess the relationship between BMI and AHC showing a direct relationship between increased BMI and higher change in anti-hypertensive drug usage at postoperative period r(45) = 0.92, p > 0.05 CI 95%. Higher tumor volume showed a longer operative time, r(45) = 0.6 (p = 0.000 CI 95%). CONCLUSIONS: Obese patients could have an increased impact with surgery with an increased change in postoperative anti-hypertensive management. Tumor volume is associated with increased operative time and blood loss, our data suggest that it could be associated with increased rates of morbidity. However, further prospective studies with larger sample sizes are needed to validate our results.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Laparoscopía , Neoplasias de las Glándulas Suprarrenales/patología , Neoplasias de las Glándulas Suprarrenales/cirugía , Adrenalectomía/métodos , Adulto , Anciano , Antihipertensivos , Pérdida de Sangre Quirúrgica , Índice de Masa Corporal , Femenino , Humanos , Laparoscopía/métodos , Tiempo de Internación , Masculino , Persona de Mediana Edad , Estudios Observacionales como Asunto , Complicaciones Posoperatorias/etiología , Periodo Posoperatorio , Estudios Prospectivos , Estudios RetrospectivosRESUMEN
Background: Adrenal myelolipomas (ML) are rare benign neoplasms compound of adipose and myeloid tissue. Clinically they are usually asymptomatic, being diagnosed generally by incident. In Mexico, there are only 32 published cases of ML, these occur between 37 and 65 years, with the male-female ratio being 1:1.1, clinically they present with abdominal or lumbar pain, open surgery being the main surgical approach (89%). Clinical case: We made a literature review of ML in Mexico and present two clinical cases: a 67-year-old man in followup for diverticular disease and a 40-year-old woman with pain in the left upper quadrant. In both cases, tumor resection was performed measuring 9.5 cm and 13.3 cm long respectively. Conclusions: We present two new cases in our country that correspond to incidentalomas. In both cases, surgery was performed to confirm the diagnosis, as well as to prevent possible complications.
Introducción: los mielolipomas suprarrenales (ML) son neoplasias benignas poco frecuentes constituidas por tejido adiposo y mieloide. Clínicamente asintomáticas, suelen ser diagnosticados incidentalmente. En México existen solo 32 casos publicados de ML, presentándose en pacientes de entre 37 a 65 años, siendo la relación hombre-mujer 1:1.1, el síntoma más comúnmente reportado es dolor abdominal inespecífico, y la cirugía abierta es el principal abordaje quirúrgico empleado en nuestro país (89%). Caso clínico: presentamos una recopilación de la literatura actual sobre ML en México, además de dos casos clínicos de pacientes con ML: un hombre de 67 años con enfermedad diverticular y una mujer de 40 años con dolor en hipocondrio; en ambos se realizó resección tumoral, midiendo 9.5 cm y 13.3 cm, respectivamente. Conclusiones: presentamos dos casos nuevos en nuestro país que corresponden a incidentalomas. En ambos casos la cirugía se realizó para confirmar el diagnóstico, así como para prevenir posibles complicaciones.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Lipoma , Dolor de la Región Lumbar , Mielolipoma , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Neoplasias de las Glándulas Suprarrenales/patología , Neoplasias de las Glándulas Suprarrenales/cirugía , Adulto , Anciano , Femenino , Humanos , Masculino , México , Mielolipoma/diagnóstico , Mielolipoma/patología , Mielolipoma/cirugíaRESUMEN
The aim of the study was to clarify the relationship and the time of aldosterone and renin recoveries at immediate and long-term follow-up in aldosterone-producing adenoma (APA) patients who underwent adrenalectomy. Prospective and longitudinal protocol in a cohort of APA patients was followed in a single center. Among 43 patients with primary aldosteronism (PA), thirteen APA patients were enrolled in this study. Blood was collected for aldosterone, renin, potassium, creatinine, cortisol, and ACTH before and 1, 3, 5, 7, 15, 30, 60, 90, 120, 180, 270, 360 days after adrenalectomy. At diagnosis, most patients (84%) had hypokalemia and high median aldosterone levels (54.8; 24.0-103 ng/dl) that decreased to undetectable (<2.2) or very low (<3.0) levels between fifth to seventh days after surgery; then, between 3-12 months, its levels gradually increased to the lower normal range. The suppressed renin (2.3; 2.3-2.3 mU/l) became detectable between the fifteen and thirty days after surgery, remaining normal throughout the study. The aldosterone took longer than renin to recover (60 vs.15 days; p<0.002) and patients with higher aldosterone had later recovery (p=0.03). The cortisol/ACTH levels remained normal despite the presence of a post-operative hypoaldosteronism. Blood pressure and antihypertensive requirement decreased after adrenalectomy. In conclusion, our prospective study shows the borderline persistent post-operative hypoaldosteronism in the presence of early renin recovery indicating incapability of the zona glomerulosa of the remaining adrenal gland to produce aldosterone. These findings contribute to the comprehension of differences in renin and aldosterone regulation in APA patients, although both are part of the same interconnected system.
Asunto(s)
Adenoma , Neoplasias de las Glándulas Suprarrenales , Adenoma Corticosuprarrenal , Hiperaldosteronismo , Hipertensión , Hipoaldosteronismo , Adenoma/cirugía , Neoplasias de las Glándulas Suprarrenales/cirugía , Adrenalectomía , Hormona Adrenocorticotrópica , Aldosterona , Humanos , Hidrocortisona , Hiperaldosteronismo/cirugía , Estudios Prospectivos , ReninaRESUMEN
Introducción: los mielolipomas suprarrenales (ML) son neoplasias benignas poco frecuentes constituidas por tejido adiposo y mieloide. Clínicamente asintomáticas, suelen ser diagnosticados incidentalmente. En México existen solo 32 casos publicados de ML, presentándose en pacientes de entre 37 a 65 años, siendo la relación hombre-mujer 1:1.1, el síntoma más comúnmente reportado es dolor abdominal inespecífico, y la cirugía abierta es el principal abordaje quirúrgico empleado en nuestro país (89%). Caso clínico: presentamos una recopilación de la literatura actual sobre ML en México, además de dos casos clínicos de pacientes con ML: un hombre de 67 años con enfermedad diverticular y una mujer de 40 años con dolor en hipocondrio; en ambos se realizó resección tumoral, midiendo 9.5 cm y 13.3 cm, respectivamente. Conclusiones: presentamos dos casos nuevos en nuestro país que corresponden a incidentalomas. En ambos casos la cirugía se realizó para confirmar el diagnóstico, así como para prevenir posibles complicaciones.
Background: Adrenal myelolipomas (ML) are rare benign neoplasms compound of adipose and myeloid tissue. Clinically they are usually asymptomatic, being diagnosed generally by incident. In Mexico, there are only 32 published cases of ML, these occur between 37 and 65 years, with the male-female ratio being 1:1.1, clinically they present with abdominal or lumbar pain, open surgery being the main surgical approach (89%). Clinical case: We made a literature review of ML in Mexico and present two clinical cases: a 67-year-old man in followup for diverticular disease and a 40-year-old woman with pain in the left upper quadrant. In both cases, tumor resection was performed measuring 9.5 cm and 13.3 cm long respectively. Conclusions: We present two new cases in our country that correspond to incidentalomas. In both cases, surgery was performed to confirm the diagnosis, as well as to prevent possible complications
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Anciano , Mielolipoma/diagnóstico , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Mielolipoma/cirugía , Mielolipoma/patología , Neoplasias de las Glándulas Suprarrenales/cirugía , Neoplasias de las Glándulas Suprarrenales/patología , Glándulas Suprarrenales/patología , Glándulas Suprarrenales/diagnóstico por imagen , MéxicoRESUMEN
INTRODUCTION AND OBJECTIVES: Laparoscopic surgery is the standard approach for the treatment of adrenal glands. Bilateral synchronous adrenalectomy is rarely performed, and evidence about this procedure is limited. Our objective is to report our 13-year experience with synchronous laparoscopic bilateral adrenalectomy, evaluating its feasibility, safety, and perioperative outcomes. PATIENTS AND METHODS: A total of 23 consecutive patients undergoing synchronous bilateral laparoscopic adrenalectomy between 2007 and 2020 in a single academic center were included. Variables evaluated were operative time, estimated blood loss, conversion to open surgery, postoperative complications, mortality, and postoperative length of stay. RESULTS: Mean operative time was 189.3⯱â¯48.9â¯min. Mean estimated blood loss was 163.0⯱â¯201.3â¯mL. There were no conversions to open surgery. Five patients had postoperative complications, three of those were major. No patient died in the perioperative period. Median postoperative length of stay was three days (range 1-30). At pathology analysis, 15 patients had bilateral adrenal hyperplasia, 2 unilateral adrenal hyperplasia and a contralateral benign tumor, 1 unilateral adrenal hyperplasia and a normal contralateral gland, 1 unilateral adenoma, 3 bilateral pheochromocytomas and 1 bilateral myelolipoma. CONCLUSION: Synchronous bilateral laparoscopic adrenalectomy is a feasible and safe technique. A multidisciplinary and experienced team involving anesthesiologists and endocrinologists is required.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Laparoscopía , Neoplasias de las Glándulas Suprarrenales/cirugía , Adrenalectomía/efectos adversos , Adrenalectomía/métodos , Humanos , Hiperplasia/etiología , Laparoscopía/métodos , Complicaciones Posoperatorias/etiologíaRESUMEN
Los tumores suprarrenales virilizante son infrecuentes y representan 5-6% de los tumores de esas glándulas1. Pueden secretar diferentes andrógenos como dehidroepiandrosterona sulfato (DHEAS), androstenediona y testosterona. Las características clínicas dependen de la edad de presentación; en niños pueden determinar pubertad precoz y en mujeres en edad fértil ocasionar hirsutismo, amenorrea o ciclos oligomenorreicos y diversos grados de virilización2. Los carcinomas adrenocorticales son tumores raros y la incidencia es aproximadamente uno a dos por millón de habitantes/año3,4. Los exámenes de imagen como la tomografía o la resonancia confirman el origen suprarrenal, valoran la presencia de metástasis y definen la conducta terapéutica5. La presentación inicial en pacientes pediátricos mayoritariamente es con virilización6 y aproximadamente el 50% de los pacientes adultos con carcinoma adrenal tienen un estadio de la enfermedad relativamente avanzado7. El tratamiento de elección es la cirugía y sigue siendo la mejor esperanza para la supervivencia a largo plazo8. El pronóstico habitual para el carcinoma adrenocortical es generalmente malo con una supervivencia global a 5 años de 20 a 25%5 en adultos, pero en niños y adolescentes la supervivencia puede llegar al 100%9. Se presenta el caso de una paciente con fenotipo totalmente masculino con diagnóstico de carcinoma adrenal virilizante que comienza en la infancia y se diagnostica en la adolescencia.
Virilizing adrenal tumors are uncommon and represent 5-6% on tumors of these glands1. They can secrete different androgens such as dehydroepiandrosterone sulfate (DHEAS), androstenedione, and testosterone. Clinical features depend on the age of presentation; in children they can determine precocious puberty and in women of childbearing age cause hirsutism, amenorrhea or oligomenorrheic cycles and various degrees of virilization2. Diagnosis consists of clinical evidence of hyperandrogenism, accompanied by an increase in androgens in the blood, especially DHEAS, whose origin is mainly adrenal. Adrenocortical carcinomas are rare and the incidence is approximately one to two per million inhabitants/year3,4. Imaging tests such as tomography or resonance confirm the adrenal origin, assess the presence of metastases and define the therapeutic approach5. In initial presentation in most pediatric patients is with virilization6 and approximately 50% adult's patients with adrenal carcinoma have a relatively advanced stage of the disease7. The treatment of choice is surgery and is the best hope for long-term survival. The usual prognosis for adrenocortical carcinoma is generally poor with a 5-year overall survival of 20 to 25%5 in adults, but in children and adolescent's survival can reach 100%9. We present the case of a patient with a totally male phenotype diagnosed with virilizing adrenal carcinoma that begins in childhood and is diagnosed in adolescence.
Asunto(s)
Humanos , Femenino , Adolescente , Virilismo/etiología , Carcinoma/complicaciones , Neoplasias de las Glándulas Suprarrenales/complicaciones , Carcinoma/cirugía , Carcinoma/diagnóstico , Hiperandrogenismo , Neoplasias de las Glándulas Suprarrenales/cirugía , Neoplasias de las Glándulas Suprarrenales/diagnósticoRESUMEN
BACKGROUND: Adrenal metastases are the most common malignant lesions of the adrenal glands and the second most common tumor after adenomas. The location of the primary tumor is described: lung (39%), breast (35%), gastrointestinal tract, among other. Several studies show that surgery improves survival in selected cases. METHOD: Retrospective and single-center observational study of patients operated for adrenal metastasis over a period of 11 years. The characteristics of the disease and surgical results were described. RESULTS: 14 suprarenalectomies were performed. The average age was 65.85 years. The primary tumors described: non-small cell lung carcinoma (42.8%) and clear cell renal carcinoma (14.20%). In 92.8% the injury was unilateral. In 64.2% it was metachronous. An initial laparoscopic approach was performed in 85.71%. The morbidity of our series was 14.28%. The median overall survival was 30 months. Survival was 75% per year, 55.5% at 3 years and 40% at 5 years. CONCLUSIONS: Age, primary location, degree of differentiation, histological type, size greater, laterality, disease-free interval, chemotherapy and surgical technique are not associated with changes in survival. In the presence of a single adrenal mass, surgical evaluation is mandatory and surgery could play a role in patients with metastases in other locations with control of the primary disease.
ANTECEDENTES: Las metástasis suprarrenales son las lesiones malignas más frecuentes de las glándulas suprarrenales. Como localización del tumor primario se encuentran el pulmón (39%), la mama (35%), el riñón y el tracto gastrointestinal, entre otros. La cirugía mejora la supervivencia en casos seleccionados. MÉTODO: Estudio observacional, retrospectivo y unicéntrico de pacientes intervenidos de suprarrenalectomía por metástasis suprarrenal en un periodo de 11 años. Se describen las características de la enfermedad y los resultados quirúrgicos. RESULTADOS: Se realizaron 14 suprarrenalectomías. La media de edad de los pacientes fue de 65.85 años. Los tumores primarios fueron carcinoma no microcítico pulmonar (42.8%) y carcinoma renal de células claras (14.20%). En el 92.8% de los casos fue unilateral y en el 64.2% metacrónica. Se realizó laparoscopia inicial en el 85.71%. La morbilidad fue del 14.28%. La mediana de supervivencia global fue de 30 meses. La supervivencia fue del 75% al año, el 55.5% a los 3 años y el 40% a los 5 años. CONCLUSIONES: La edad, la localización primaria, el grado de diferenciación, el tipo histológico, el tamaño, la lateralidad, el intervalo libre de enfermedad, la quimioterapia y la técnica quirúrgica no se asocian a cambios en la supervivencia. Ante una masa suprarrenal única, la valoración quirúrgica es mandatoria y la cirugía podría ocupar un papel en pacientes con metástasis en otras localizaciones con control de la enfermedad primaria.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Neoplasias Pulmonares , Neoplasias Primarias Secundarias , Neoplasias de las Glándulas Suprarrenales/cirugía , Adrenalectomía , Anciano , Humanos , Neoplasias Pulmonares/cirugía , Estudios RetrospectivosRESUMEN
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/etiologíaRESUMEN
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/cirugíaRESUMEN
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 , LaparoscopíaRESUMEN
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 , LaparoscopíaRESUMEN
BACKGROUND: Obese patients may have unrecognized primary aldosteronism due to high rates of concomitant hypertension. We hypothesized that obesity impacts the diagnosis and management of patients with primary aldosteronism. METHODS: We conducted a retrospective analysis of all primary aldosteronism patients (n = 418) who underwent adrenal vein sampling (1997-2017). Patients were classified by body mass index as obese (body mass index ≥35) or nonobese (body mass index <35) and diagnostic evaluation was compared between groups. Within the operative cohort (n = 285), primary outcomes were changes in both blood pressure and antihypertensive medications after adrenalectomy. Secondary outcome was clinical resolution by Primary Aldosteronism Surgery Outcomes criteria. RESULTS: Thirty-five percent of patients were obese. Obese patients were more likely to be male (67.8% vs 56.1%, P = .025), somewhat younger (51.5 vs 54.4 years old, P < .012), and require more preoperative antihypertensive medications (6.7 vs 5.7, P = .04) than nonobese patients. Obese patients had lesser rates of radiologic evidence of adrenal tumors (68.4 vs 77.9%, P = .038) despite similar rates of lateralization on adrenal vein sampling. In the operative subset, obese patients had somewhat smaller tumors on final pathology (1.1 vs 1.5 cm, P = .014) but similar rates of complete and partial clinical resolution (P = 1.000). CONCLUSION: Obese primary aldosteronism patients have lesser rates of localization by imaging, likely due to smaller tumor size, however, experience similar benefit from adrenalectomy.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales/diagnóstico , Adrenalectomía , Antihipertensivos/administración & dosificación , Hiperaldosteronismo/diagnóstico , Hipertensión/terapia , Obesidad/complicaciones , Neoplasias de las Glándulas Suprarrenales/complicaciones , Neoplasias de las Glándulas Suprarrenales/epidemiología , Neoplasias de las Glándulas Suprarrenales/cirugía , Glándulas Suprarrenales/diagnóstico por imagen , Glándulas Suprarrenales/patología , Glándulas Suprarrenales/cirugía , Adulto , Factores de Edad , Anciano , Presión Sanguínea/efectos de los fármacos , Índice de Masa Corporal , Femenino , Humanos , Hiperaldosteronismo/epidemiología , Hiperaldosteronismo/etiología , Hiperaldosteronismo/cirugía , Hipertensión/etiología , Masculino , Persona de Mediana Edad , Obesidad/epidemiología , Estudios Retrospectivos , Factores de Riesgo , Factores Sexuales , Resultado del TratamientoRESUMEN
ABSTRACT Purpose This study aimed to compare perioperative and postoperative results of right and left laparoscopic adrenalectomy (LA), and to evaluate the impact of challenging factors on these outcomes. Materials and Methods A total of 272 patient's medical records that underwent single side LA between October 2006 and September 2017 were retrospectively reviewed. The patients were divided into 2 groups according to operation side. Moreover, pheochromocytoma, metastatic masses and adrenal lesions >5cm in size were considered to be difficult adrenalectomy cases and the outcomes of these cases were compared between two groups. Results 135 patients (49.6%) underwent right LA and 137 patients (50.4%) underwent left LA. Operation time, estimated blood loss (EBL) and hospitalization time were similar between the groups (p=0.415, p=0.242, p=0.741, respectively). Although EBL was higher on the right side than the left (p=0.038) in the first 20 cases, after this learning period has been completed, there was no significant difference between the groups. In patients with pheochromocytoma, metastatic mass and a mass >5cm in size, despite bleeding complications were clinically higher on the right side, this difference was not statistically significant. Conclusions During the learning period of LA, EBL is higher on the right side. Due to the greater risk of bleeding complications on the right side even on the hands of experienced surgeons, extra care and preoperative planning are required in patients with pheochromocytoma, metastatic masses and masses >5cm in size.
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Anciano , Anciano de 80 o más Años , Adulto Joven , Laparoscopía/efectos adversos , Laparoscopía/métodos , Adrenalectomía/efectos adversos , Adrenalectomía/métodos , Periodo Posoperatorio , Valores de Referencia , Estudios Retrospectivos , Factores de Riesgo , Pérdida de Sangre Quirúrgica , Resultado del Tratamiento , Neoplasias de las Glándulas Suprarrenales/cirugía , Neoplasias de las Glándulas Suprarrenales/patología , Glándulas Suprarrenales/cirugía , Glándulas Suprarrenales/patología , Estadísticas no Paramétricas , Medición de Riesgo , Carga Tumoral , Periodo Perioperatorio , Tempo Operativo , Tiempo de Internación , Persona de Mediana EdadRESUMEN
INTRODUCTION: Adrenal masses are uncommon in newborns. The differential diagnosis includes be nign masses (adrenal hemorrhage, extralobar pulmonary sequestration) and malignant ones (neuro blastoma) that may be a finding during an obstetric ultrasound. The use of complementary imaging methods allows a better diagnosis approach during the postnatal period, with implications for the management of these patients. OBJECTIVE: To report the case of a female newborn with diagnosis of an adrenal mass, and to discuss differential diagnoses and management alternatives of adrenal lesions in newborns. CASE REPORT: Two-month-old female infant, referred for adrenal tumor study diagnosed at 22 weeks gestational age. Postnatal ultrasound showed a tumor compatible with neuroblastoma. The patient was asymptomatic, and the laboratory studies showed no relevant findings. The lesion was excised by laparoscopy. A histological study confirmed pulmonary sequestration. CONCLUSIONS: Extralobar pulmonary sequestration should be considered in the differential diagnosis of an adrenal mass in the newborn. Minimally invasive surgery should be the preferred surgical technique choice in these cases, given the technical feasibility and benefits in the recovery and cosmetic issues of the patient.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales/diagnóstico por imagen , Secuestro Broncopulmonar/diagnóstico , Neuroblastoma/diagnóstico por imagen , Neoplasias de las Glándulas Suprarrenales/cirugía , Diagnóstico Diferencial , Femenino , Humanos , Lactante , Recién Nacido , Laparoscopía/métodos , Procedimientos Quirúrgicos Mínimamente Invasivos , Neuroblastoma/cirugía , Embarazo , Ultrasonografía , Ultrasonografía PrenatalRESUMEN
The Guidelines Project, an initiative of the Brazilian Medical Association, aims to combine information from the medical field in order to standardize producers to assist the reasoning and decision-making of doctors. The information provided through this project must be assessed and criticized by the physician responsible for the conduct that will be adopted, depending on the conditions and the clinical status of each patient.