Feocromocitoma maligno. A propósito de tres casos / Malignant Pheochromocytoma. As Regards of Three Cases

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)

Adrenalectomía laparoscópica en 40 pacientes para el tratamientodel feocromocitoma adrenal / Laparoscopic adrenalectomy in 40 patients for the treatment of adrenal pheochromocytoma

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)

Características de incidentaloma suprarrenal durante 10 años en un hospital terciario de Uruguay / Characteristics of adrenal incidentaloma during 10 years in a tertiary hospital in Uruguay

Introducción: la prevalencia de incidentaloma suprarrenal está aumentando por el envejecimiento de la población y el uso generalizado de técnicas de imagen de alta resolución. Objetivo: analizar los casos con incidentaloma suprarrenal en nuestra población. Materiales y métodos: estudio observacional, descriptivo y retrospectivo. Fueron pacientes que se encontraban en seguimiento, desde 2005 al 2015 en Endocrinología del Hospital de Clínicas. En el análisis estadístico se utilizaron medidas de resumen (media, mediana y frecuencias); se realizó análisis bivariados definiendo el punto de significación de 0.05. Resultados: encontramos 42 individuos con incidentaloma suprarrenal. La media de edad: 57.8 años, mayoría mujeres (76.2%). El diagnóstico se realizó por tomografía en 76.2% y el motivo de solicitud fue por causa digestiva en 47.6%. La mediana de tamaño al diagnóstico fue 17 mm, 6 pacientes tuvieron lesiones bilaterales. Se calculó las Unidades Hounsfield a 52.1% de las lesiones y a 18.7% se les realizó washout. Al diagnóstico inicial fueron catalogados 31 pacientes con adenoma no funcionante, 4 con Cushing subclínico, 3 feocromocitomas y 4 quedaron sin catalogar. Se siguieron a los pacientes una mediana de tiempo de 24 meses. De los 4 Cushing subclínicos, 3 se presentaron en lesiones bilaterales, ningún paciente evolucionó a Cushing manifiesto. Crecieron un 18.7% de las lesiones. Se operaron 4 pacientes. Tras la anatomía patológica, uno de los feocromocitomas resultó ser un adenoma. Durante el seguimiento se diagnosticó un feocromocitoma aunque tenía características sospechosas desde el inicio. Ninguno de los bilaterales recibió tratamiento quirúrgico. Conclusiones: se confirmó aumento en el número de casos de incidentaloma suprarrenal del 2005 al 2015. La gran mayoría fueron adenomas no funcionantes. De los Cushing subclínicos tres cuartas partes fueron bilaterales. En la mayoría la conducta fue conservadora.

Introduction: the prevalence of adrenal incidentaloma is increasing due to aging of the population and the widespread use of high-imaging techniques resolution. Objective: to analyze cases with adrenal incidentaloma in our population. Materials and methods: observational, descriptive and retrospective study. They were patients who were in follow-up, from 2005 to 2015 in Endocrinology of the Hospital of Clinics. In the statistical analysis, summary measures were used (mean, median and frequencies); bivariate analyzes were performed defining the significance point of 0.05. Results: we found 42 individuals with adrenal incidentaloma. The average of age: 57.8 years, mostly women (76.2%). The diagnosis was made by tomography in 76.2% and the reason for request was due to digestive causes in 47.6%. The median size at Diagnosis was 17 mm, 6 patients had bilateral lesions. The Units were calculated Hounsfield 52.1% of the injuries and 18.7% had a washout. At the initial diagnosis 31 patients with non-functioning adenoma were classified, 4 with subclinical Cushing, 3 pheochromocytomas and 4 were not cataloged. The patients were followed a median of 24 months' time. Of the 4 subclinical Cushing, 3 were presented in bilateral lesions, no patient evolved to manifest Cushing. They grew 18.7% of the injuries. He 4 patients operated. After the pathological anatomy, one of the pheochromocytomas resulted be an adenoma During the follow-up a pheochromocytoma was diagnosed although suspicious characteristics from the beginning. None of the bilaterals received treatment surgical. Conclusions: an increase in the number of incidentaloma cases was confirmed adrenal from 2005 to 2015. The vast majority were non-functioning adenomas. Of the Cushing subclinical three quarters were bilateral. In the majority, the behavior was conservative.

Mielolipoma, una lesión quirúrgica poco frecuente de la glándula adrenal / Myelolipoma, a rare surgical lesion of the adrenal gland

El mielolipoma adrenal es un tumor benigno poco frecuente, compuesto de tejido adiposo maduro y elementos hematopoyéticos diversos. Estos tumores pueden localizarse en las glándulas adrenales aunque también pueden encontrarse en sitios extradrenales. Las manifestaciones clínicas son variadas tales como sangramientos digestivos, dolor abdominal en los flancos, masa abdominal palpable, hematuria, hemorragia retroperitoneal y pérdida de peso. Entre los métodos diagnósticos figuran el ultrasonido, la tomografía axial computarizada y la resonancia magnética. La resección quirúrgica es el tratamiento de elección, sobre todo cuando haya sintomatología o en tumores mayores de 4 cm, por el riesgo de ruptura espontánea con hemorragia retroperitoneal. Presentamos la historia clínica de una paciente de 42 años de edad con antecedentes de dolores en hipocondrio derecho de aproximadamente un año de evolución, la cual sufrió un cólico nefrítico que cedió con tratamiento médico. Posteriormente en la consulta externa, en el examen fisico, se constata una masa tumoral en hipocondrio derecho, la cual fue diagnosticada por ultrasonido y tomografía axial computarizada como un tumor adrenal. Se intervino la paciente y se confirma el diagnóstico. La evolución posoperatoria fue buena y la paciente continúa asintomática. El objetivo de este trabajo es presentar un nuevo caso de esta enfermedad, poco frecuente, la cual fue diagnosticada en una paciente de 42 años de edad y realizar una breve revisión de la literatura(AU)

Mielolipoma is a rare benign tumor of the adrenal gland, which is formed by mature adipose tissue and several hematopoietic elements. They are generally located in the adrenal glands, although they can occur in other sites. The clinical manifestations are varied such as gastrointestinal bleeding, abdominal pain in the flanks, abdominal mass, retroperitoneal hemorrhage, hematuria and loss of weight. Among the diagnosing methods are ultrasound, computerized axial tomography and magnetic imaging resonance. The surgical excision is the treatment of choice, mainly when symptoms are present or in case of over 4 cm tumors, because of the spontaneous rupture risk with retroperitoneal hemorrhage. The objective of this paper was to present a new case of this rare disease together with a brief literature review. A 42 years-old female with a history of abdominal pain located in the right upper quadrant of the abdomen, who suffered nephritic colic that was successfully treated. The physical exam at the outpatient service revealed a tumorous mass diagnosed as adrenal tumor by means of ultrasound and axial computerized tomography. She was operated on and the diagnosis was confirmed. Her postoperative recovery was good and the patient is still asymptomatic(AU)

Pheochromocytoma in children and adolescents.

Eleven subjects aged <20 yr with histologically proven pheochromocytoma between 1987 and 2006 were analyzed. Family history was present in 18%. In 2 patients, pheochromocytoma was part of VHL and in one it was associated with MEN 2. Twenty four hour urine VMA level was elevated in 100% and metanephrine level in 73%. CT/ MRI were showing the tumor in all. Prazosin extended release tablets (maximum 30 mg/day) were used in 73% and doxazosin (maximum 12 mg/ day) in 27%. Intraoperative BP fluctuations were seen in 27%. All were biochemically cured after surgery. Preoperative á blockade with extended release prazosin and doxazosin were effective in controlling perioperative BP fluctuations. Hence these drugs can be used in children and adolescents without fear of postoperative hypotension.

Endocrine cancer in Iran: based on cancer registry system.

BACKGROUND: A population-based registry of endocrine cancer cases in four Iranian provinces, was performed for the years 1996-2000. MATERIALS AND METHODS: Patients in each province were grouped according to age, gender and tumor specifics (site, morphology, behavior) and the data was coded according to the international classification of diseases for oncology. STATISTICAL ANALYSIS USED: Person-years of population at risk were calculated and the results were presented as incidence rates by sex, age, age specific rates and age standard rate (ASR) per 100,000 person-years, using direct method of standardization to the world population. RESULTS: A total of 319 cases of primary endocrine cancer were found and registered, including 313 cases of thyroid carcinoma and 6 cases of adrenal cancer. The thyroid carcinoma group cases consisted of papillary (82.7%), follicular (8.6%), medullary (7.0%) and anaplastic (1.6%) carcinomas. The ASR for thyroid carcinoma was 1.289 (0.627 for men, 1.59 for women), with the highest incidence rate in Kerman (ASR 1.643) and the lowest incidence rate in Golestan (ASR 0.735). For the 6 cases of adrenal cancer, 4 were neuroblastoma and 2 were pheochromocytoma. CONCLUSIONS: Iran was considered as an endemic, iodine-deficient area, until fairly recently. Iodinization of salt has been started about 12 years ago, in the nation. Considering the effect of improvement in the iodine intake in previously deficient communities, which is associated with an increase in the incidence of papillary carcinoma compared to other histologic types, the frequency and distribution of histologic types of thyroid carcinoma was closer to what can be seen in iodine-rich areas.

Prevalência de lesão adrenal incidental em pacientes submetidos a tomografia computadorizada de tórax e abdome em um hospital geral brasileiro / Prevalence of adrenal incidentaloma at computed tomography (chest and abdominal) in a general hospital in Brazil

INTRODUÇÃO: Determinar prospectivamente a prevalência de lesão adrenal incidental a tomografia computadorizada (TC) em um hospital geral universitário brasileiro. PACIENTES E MÉTODO: Estudo transversal, prospectivo, avaliando pacientes consecutivos sem doença adrenal conhecida, submetidos a TC de tórax e abdome no Hospital de Clínicas de Porto Alegre, em um período de 10 meses. Incidentalomas adrenais foram definidos pela presença de alteração no contorno, tamanho ou densidade da glândula. Valores de kappa foram calculados, avaliando a concordância intra e inter-observador. RESULTADOS: Identificaram-se 101 lesões adrenais incidentais em 83 de 3.382 pacientes, correspondendo a uma prevalência de 2,5 por cento (IC 95 por cento: 1,0 por cento-3,2 por cento). A prevalência de lesões adrenais aumentou com a idade e foi significativamente mais alta em pacientes do sexo masculino (3,3 por cento vs. 1,5 por cento; p= 0,01). Na avaliação das concordâncias intra e inter-observador, obteve-se um kappa de 0,76 (boa concordância) e 0,83 (excelente concordância) respectivamente. CONCLUSÕES: A prevalência de incidentaloma adrenal detectada por TC foi semelhante à de diversas séries clínicas retrospectivas publicadas. Os dois fatores que influenciaram de forma independente a distribuição dos incidentalomas foram aumento na idade e sexo masculino.

A comprehensive approach to adrenal incidentalomas

O aperfeiçoamento das técnicas de imagem abdominal tem aumentado a detecção de massas adrenais clinicamente não aparentes, ou incidentalomas (IA), cujo diagnóstico e manuseio apropriados têm se tornado um problema clínico comum para os profissionais da saúde. Uma vez detectada uma massa adrenal, o clínico é obrigado a formular duas questões: 1) o tumor é hormonalmente ativo? e 2) existe alguma possibilidade de a massa ser maligna?. A maioria dos IA são adenomas corticais não hipersecretores, mas uma avaliação endócrina pode resultar na identificação de um excesso hormonal sutil. Como passos preliminares de screening são recomendados um teste de supressão overnight com doses baixas de dexametasona, a dosagem de metanefrinas em urina fracionada ou no plasma e, em pacientes hipertensos, estabelecer a relação da aldosterona plasmática/atividade plasmática de renina na posição ortostática. Massas maiores que 4cm têm risco maior de malignidade. Achados morfológicos de imagem podem ser valiosos na distinção entre formas benignas e malignas. A biópsia de aspiração com agulha fina é um procedimento importante na avaliação de pacientes oncológicos para se estabelecer qualquer doença metastática. Adrenalectomia está indicada na evidência de uma massa adrenal funcionante, ou na suspeita de uma forma maligna. Nós recomendamos adrenalectomia para casos de hipercortisolismo sutil, especialmente em presença de hipertensão, obesidade, diabetes ou osteoporose, potencialmente agravados pelo excesso de glicocorticóides. Um acompanhamento rigoroso é necessário, particularmente no primeiro ano após o diagnóstico.

Feocromocitoma y anestesia: Revisión del tema a propósito de un caso / Phechromocytoma and anestesia. A subject review for one-case purpose

Se reporta el caso de un paciente al cual se le realizó resección de un feocromocitoma en el Instituto de Nefrología. Se describen sus antecedentes, anestesia general orotraqueal, control hemodinámico con lidocaína y nitropusiato de sodio y monitoreo transoperatorio empleado. Se realizó una revisión bibliográfica a través del Medline, a la luz de los conocimientos actuales de la atención perioperatoria, métodos anestésicos, terapéutica con vasodilatadores, bloqueadores alfa y beta adrenérgicos, sulfato de magnesio y bloqueadores de los canales de calcio utilizados en el paciente con feocromocitoma(AU)

The case is reported from a patient to which was carried out resection of a feocromocitoma in the Institute of Nefrología. Their antecedents are described, it anesthetizes general orotraqueal, hemodynamic control with lidocaína and nitropusiato of sodium and monitoreo used transoperatorio. He/she was carried out a bibliographical revision through the Medline, by the light of the current knowledge of the attention perioperatoria, anesthetic methods, therapy with vasodilatadores, blocking alpha and beta adrenérgicos, sulfate of magnesium and blocking of the channels of calcium used in the patient with feocromocitoma(AU)

Incidentalomas de supra-renal: análise de uma casuística / Adrenal incidentalomas: analysis of a casuistic

Relatamos 12 casos de incidentalomas de supra-renal, identificados durante dez anos no Serviço de Endocrinologia do Hospital de Clínicas de Porto Alegre. A prevalência estimada em relaçao ao número total de ultra-sonografias e de tomografias computadorizadas é de apenas O,08 por cento. A avaliaçao laboratorial incluiu verificaçao dos níveis de andrógenos, calemia, acido vanililmandélico urinário (VMA), teste de supressao overnight com dexametasona e teste de ACTH para cortisol e l7 alpha-hidroxiprogesterona, quando indicados. Os incidentalomas foram descobertos na sua maioria (9/12 casos) pela ultra-sonografia, indicada por dor abdominal ou lombar. Os dados de investigaçao que se revelaram mais importantes foram o VMA e as características da lesao à tomografia (tamanho, impregnaçao heterogênea ao contraste, hipodensidades na lesao e invasao de tecidos vizinhos). Os diagnósticos mais freqüentes foram feocromocitoma (4/12), linfoma nao-Hodgkin (2/12) e de neoplasias (2/12, um Schwannoma e um carcinoma metastático). Nossos dados sugerem que os incidentalomas de supra-renal sao geralmente manifestaçoes de uma doença grave, em desacordo com a maioría das séries de casos na literatura, indicando a necessidade de cuidadoso manejo destes casos.

Tumor adrenal virilizante productor de testosterona

Los tumores adrenales virilizantes son una rara patología que se desarrolla especialmente en la infancia. Generalmente son productores de dehidroepiandrosterona (DHEA) y androsternidiona (A2)(1); ocasionalmente, sin embargo, se han descrito tumores de este tipo productores de testosterona en mujeres adultas (2-20). Presentamos la historia clínica y los hallazgos endocrinológicos, mediante diversas pruebas, en una paciente de 58 años con un tumor virilizante productora de testosterona que fue estudiada por nuestro grupo en el Hospital Militar Central. Igualmente se muestran los hallazgos histopatológicos y se comenta la literatura reciente sobre el tema.