Laparoscopic approach of pediatric adrenal tumors.

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.

Síndrome de Cushing por carcinoma adrenocortical en lactante de 5 meses

RESUMEN Introducción: Las causas adrenales del síndrome de Cushing como el carcinoma son las etiologías comunes en el niño, si bien su incidencia es baja. Objetivo: Describir los procedimientos diagnósticos y terapéuticos seguidos en el caso de una lactante de cinco meses con síndrome de Cushing como consecuencia de un carcinoma adrenocortical. Presentación del caso: Lactante femenina de cinco meses consultada por aumento excesivo de peso a partir del segundo mes de nacida a pesar de no alimentarse adecuadamente, además de presentar un retraso en el crecimiento. Se constató obesidad centrípeta y facie pletórica, ligero eritema facial y livedo reticularis, tensión arterial de 130/80 mmHg (>95 percentil). Presentaba agenesia de labios menores, sin vello axilar o pubiano. En el estudio de la función suprarrenal se encontró cifras elevadas de cortisol plasmático. En la tomografía computarizada abdominal se observó imagen hiperdensa compatible con tumor suprarrenal. Se diagnosticó síndrome de Cushing de etiología suprarrenal. Se le realizó adrenalectomía unilateral y se tomó muestra para biopsia posoperatoria y se emitió el diagnóstico de carcinoma adrenocortical. La paciente presenta una evolución desfavorable y finalmente fallece a consecuencia de un fallo multiorgánico al mes de realizada la cirugía. Conclusiones: Ante la sospecha clínica de síndrome de Cushing en la lactante se realizaron las determinaciones hormonales pertinentes y precisó la etiología mediante pruebas de imagen, confirmada por biopsia posquirúrgica. La cirugía constituye la terapia más adecuada ante un paciente con síndrome de Cushing de causa tumoral adrenal, aunque estos pacientes por lo general cursan con mal pronóstico.

ABSTRACT Introduction: The adrenal causes of Cushing's syndrome such as carcinoma are the common etiologies in the child, although their incidence is low. Objective: Describe the diagnostic and therapeutic procedures followed in the case of a five-month-old infant with Cushing's syndrome as a result of adrenocortical carcinoma. Case presentation: Five-month-old female infant consulted due to excessive weight gain from the second month of birth despite not eating properly, in addition to presenting a stunted growth. Centripetal and facial plethora obesity, slight facial erythema and livedo reticularis, blood pressure of 130/80 mmHg (>95 percentile) were found. She had agenesis of labia minora, without axillary or pubic hair. In the study of adrenal function, elevated plasma cortisol levels were found. Abdominal CT scan showed hyperdense image compatible with adrenal tumor. Cushing's syndrome of adrenal etiology was diagnosed. Unilateral adrenalectomy was performed and a sample was taken for postoperative biopsy and the diagnosis of adrenocortical carcinoma was made. The patient presented an unfavorable evolution and she finally died as a result of a multi-organ failure a month after the surgery. Conclusions: Given the clinical suspicion of Cushing's syndrome in the infant, the pertinent hormonal determinations were made and the etiology was specified by imaging tests, confirmed by post-surgical biopsy. Surgery is the most appropriate therapy for a patient with Cushing's syndrome with an adrenal tumor cause, although these patients usually have a bad prognosis.

Genitourinary Tract Tumors in Children: An Update.

BACKGROUND: Genitourinary tract tumors in children are less common than in adults. Most of these tumors have different genetic backgrounds, clinical presentation, and oncologic behavior than their adult counterpart. As a result of low prevalence in children, some of the treatment approaches and recommendations are based on treatment experience in adult patients. However, thanks to scientific and technological development, survival rates have risen considerably. OBJECTIVE: This paper presents a review of the principal features of the tumors involving the genitourinary tract in children and an update in genetic background, diagnosis, and treatment. METHODS: A narrative review was performed on published literature about genitourinary tract tumors in pediatric patients. Papers presented in English and Spanish literature were reviewed. PubMed, Science Direct, and SciELO databases were used to collect information and present this article. RESULTS: Kidney tumors are the most common type of genitourinary tumors in children. Among those, Wilms tumor represents the majority of cases and shows the successful work of clinical trial groups studying this tumor type. Other tumors involving the genitourinary tract in children include Rhabdomyosarcoma, Transitional cell carcinoma, Testicular, and Adrenal tumors. CONCLUSION: Genitourinary tract tumors in children represent significant morbidity and economic burden, so awareness in early diagnosis represents improvement in treatment, clinical, and oncological outcomes.

Primary neuroendocrine prostate cancer with adrenal gland metastasis.

Primary neuroendocrine differentiation in prostate cancer occurs infrequently and represents a therapeutic challenge at present due to the poor prognosis involved. We present the case of a patient with de novo neuroendocrine prostate cancer who later developed metastases to adrenals bilaterally which were initially managed surgically.

A case of behavioral changes in a castrated male cat due to a functional adrenocortical adenoma producing testosterone and androstenedione.

CASE SUMMARY: This case report describes a 9-year-old neutered male cat with a functional adrenal tumor showing aggression toward other cats in the household, excessive meowing and urine spraying. The diagnosis was made based on the clinical signs, an enlarged right adrenal gland on ultrasound, penile spines and elevated sex hormones on an adrenocorticotropic hormone stimulation test. The cat was submitted to celiotomy and adrenalectomy. Histopathological examination confirmed the adrenocortical adenoma. Three months after surgery the penile spines disappeared and urine spraying, as well as excessive meowing, had greatly decreased; however, aggressive behavior took much longer to stop and required behavior therapy. RELEVANCE AND NOVEL INFORMATION: Functional adrenal tumors producing only sex hormones and behavioral changes are uncommon. The cat reported herein started showing behavioral changes before spines appeared on the penis, and structural alterations in the adrenal gland at the abdominal ultrasonography were detected. Considering all the possible implications resulting from severe behavior problems such as cat-cat aggression, from a permanent separation of the cats to relinquishment, a detailed investigation of underlying medical mechanisms in these animals is crucial from the start. Examinations may need to be repeated later in the course.

High Prevalence of Alterations in DNA Mismatch Repair Genes of Lynch Syndrome in Pediatric Patients with Adrenocortical Tumors Carrying a Germline Mutation on TP53.

Adrenocortical cancer is a rare malignant neoplasm associated with a dismal prognosis. Identification of the molecular pathways involved in adrenal tumorigenesis is essential for a better understanding of the disease mechanism and improvement of its treatment. The aim of this study is to define the prevalence of alterations in DNA mismatch repair (MMR) genes in Lynch syndrome among pediatric patients with adrenocortical neoplasia from southern Brazil, where the prevalence of a specific TP53 germline mutation (p.Arg337His) is quite high. Thirty-six pediatric patients were retrospectively evaluated. Immunohistochemistry (IHC) for the MMR enzymes MLH1, MSH2, MSH6, and PMS2, as well as next-generation sequencing (NGS) were performed. For IHC, 36 pediatric tumors were tested. In all of them, the expression of all evaluated MMR proteins was well-preserved. For NGS, 35 patients with pediatric tumor were tested. Three patients (8.57%) with the TP53 p.Arg337His germline mutation presented pathogenic and likely pathogenic variants in the MMR genes (two in MLH1 and one in MSH6). The prevalence of altered MMR genes among pediatric patients was elevated (8.57%) and higher than in colorectal and endometrial cancer cohorts. Pediatric patients with adrenocortical tumors should, thus, be strongly considered as at genetic risk for Lynch syndrome.

A Rare Case of an Adrenal Ganglioneuroma Treated Laparoscopically in a Patient with Left-Sided Inferior Vena Cava.

Background: Ganglioneuroma is a rare tumor derived from the neural crest that can occur in any sympathetic tissue. It corresponds to 0.3% to 2% of incidental adrenal tumors and <250 have been reported in the literature so far. Case Presentation: We present a case of a 30-year-old Caucasian woman presented with a large bilobed adrenal tumor found on a CT scan during the investigation of acute abdominal pain. The image also showed a rare anatomic variation of a left-sided inferior vena cava. Biochemical work-up for adrenal incidentaloma showed normal markers. Since we could not rule out malignancy, the patient was subjected to laparoscopic adrenalectomy and the pathology report showed an adrenal ganglioneuroma, a rare nonfunctioning tumor of the adrenal. Conclusion: Ganglioneuroma can present as a large bilobed adrenal tumor. The laparoscopic approach is feasible and safe. Preoperative planning is needed and vascular variations can be challenging during the procedure.

Carcinoma adrenocortical incidental em cão: aspectos clínicos, tomográficos e histopatológicos / Incidental adrenocortical carcinoma in dog: clinical, tomographic and histopathological aspects

Background: Adrenal incidentalomas are masses accidentally discovered during imaging examinations performed whenthere is no suspicion of adrenal disease. Even with a low prevalence, it is important to perform a reliable evaluation observing biological behavior and determining whether the hormonal activity is stimulated. Frequently, these masses are notfunctional, but in some cases, there is increased cortisol activity, and patients with adrenal incidentalomas may presenthyperadrenocorticism. This report aims to describe the clinical, tomographic, and histopathological aspects of a case ofadrenal incidentaloma detected in a routine abdominal ultrasound.Case: An 8-year-old, male, maltese dog with occasional emesis, halitosis, claudication of the left pelvic limb, and previous compensated and untreated cardiomyopathy was treated at the Veterinary Hospital of the Federal University of MatoGrosso (HOVET-UFMT). Physical examination revealed arterial hypertension and claudication, with the positive posteriordrawer test suggesting rupture of the cruciate ligament. The blood count showed no alteration, and the serum biochemistryrevealed a slight increase in the activity of alanine aminotransferase. In order to investigate this increase, an abdominalultrasound was performed. Slight hepatomegaly and a heterogeneous mass of irregular edges were observed with a moderate and difficult-to-delimit vascularization of the adrenal gland. Left knee radiography raised the suspicion of rupture ofthe cranial cruciate ligament due to the cranial displacement of the tibia in relation to the femur. Computed tomographywas performed to define the extent and delimitation of the mass, which was compatible with a tumor of the right adrenalgland characterized by the visualization of an abdominal mass...(AU)

Oncocitoma suprarrenal / Oncocytic adrenocortical carcinoma

El oncocitoma suprarrenal es un tumor extremadamente infrecuente, con apenas unos 150 casos descriptos. Paciente de sexo masculino, de 48 años, con adrenalectomía derecha por incidentaloma suprarrenal de 7 cm, no funcionante. El oncocitoma suprarrenal no puede distinguirse desde el punto de vista clínico-imagenológico de otros tumores adrenales más frecuentes, por lo que su confirmación es histológica. Rara vez son malignos, y el tratamiento aceptado es la suprarrenalectomía, idealmente por vía laparoscópica. En nuestro caso, la presencia de dos criterios histológicos menores lo clasifican como un tumor con potencial maligno.

Oncocytic adrenocortical carcinoma is an extremely rare tumor, with only 150 cases reported in the literature. A 48-year-old male patient, with right adrenalectomy due to a 7-cm non-functional adrenal incidentaloma. From a clinical imaging point of view, oncocytic adrenocortical carcinoma cannot be distinguished from other more common adrenal tumors; therefore, the diagnostic confirmation is histological. It is rarely malignant, and the accepted treatment is laparoscopic adrenalectomy. In our case, two minor histological criteria classify it as a potentially malignant tumor.

A rare case of virilizing adult ectopic adrenal tumor.

Ectopic adrenal gland is an atypical medical condition with a prevalence of less than 1%. It occurs due to an abnormal gland development during embryonic life and can be found in different parts of the body. Rarely, these ectopic glands can be hormonally active and present as bulky masses. Herein we report a case of a patient who underwent laparoscopic surgery for a large myelolipoma associated with an androgen producing adenoma in an ectopic adrenal gland.

Diagnóstico diferencial y cirugía mínimamente invasiva de un tumor suprarrenal de hallazgo antenatal / Differential diagnosis and minimally invasive surgery of an antenatal adrenal mass

Resumen: Introducción: Las masas suprarrenales en recién nacidos son infrecuentes. El diagnóstico diferen cial incluye masas benignas (hemorragia suprarrenal o secuestro pulmonar extralobar) y malignas (neuroblastoma), y pueden ser un hallazgo durante la ecografía obstétrica. El uso de imágenes com plementarias en el periodo postnatal permite una mejor aproximación diagnóstica, con implicancias en el manejo de estos pacientes. Objetivos: comunicar el caso de una recién nacida portadora de una masa suprarrenal, discutir los diagnósticos diferenciales y el manejo de lesiones suprarrenales en recién nacidos. Caso Clínico: Lactante de 2 meses de edad, derivada para estudio de tumor supra rrenal de diagnóstico antenatal a las 22 semanas de edad gestacional. El estudio imagenológico con ecografía postnatal mostró un tumor compatible con neuroblastoma. Paciente asintomática, estudios de laboratorios sin hallazgos relevantes. Se realizó resección laparoscópica de la lesión. El estudio histológico confirmó un secuestro pulmonar. Conclusión: El secuestro pulmonar extralobar debe ser considerado en el diagnóstico diferencial de una masa suprarenal del recién nacido. La cirugía mínimamente invasiva debiera considerarse como el abordaje de elección en casos como este, donde existe factibilidad técnica y beneficios en la recuperación y secuelas cosméticas del paciente.

Abstract: 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.

Carcinoma adrenocortical incidental em cão: aspectos clínicos, tomográficos e histopatológicos / Incidental adrenocortical carcinoma in dog: clinical, tomographic and histopathological aspects

Background: Adrenal incidentalomas are masses accidentally discovered during imaging examinations performed whenthere is no suspicion of adrenal disease. Even with a low prevalence, it is important to perform a reliable evaluation observing biological behavior and determining whether the hormonal activity is stimulated. Frequently, these masses are notfunctional, but in some cases, there is increased cortisol activity, and patients with adrenal incidentalomas may presenthyperadrenocorticism. This report aims to describe the clinical, tomographic, and histopathological aspects of a case ofadrenal incidentaloma detected in a routine abdominal ultrasound.Case: An 8-year-old, male, maltese dog with occasional emesis, halitosis, claudication of the left pelvic limb, and previous compensated and untreated cardiomyopathy was treated at the Veterinary Hospital of the Federal University of MatoGrosso (HOVET-UFMT). Physical examination revealed arterial hypertension and claudication, with the positive posteriordrawer test suggesting rupture of the cruciate ligament. The blood count showed no alteration, and the serum biochemistryrevealed a slight increase in the activity of alanine aminotransferase. In order to investigate this increase, an abdominalultrasound was performed. Slight hepatomegaly and a heterogeneous mass of irregular edges were observed with a moderate and difficult-to-delimit vascularization of the adrenal gland. Left knee radiography raised the suspicion of rupture ofthe cranial cruciate ligament due to the cranial displacement of the tibia in relation to the femur. Computed tomographywas performed to define the extent and delimitation of the mass, which was compatible with a tumor of the right adrenalgland characterized by the visualization of an abdominal mass...

Feocromocitoma maligno em cão: relato de caso / Malignant pheochromocytoma in a dog: case report / Feocromocitoma maligno en perro: relato de caso

O feocromocitoma é um tumor secretor de catecolaminas de difícil diagnóstico, pois as manifestações clínicas nem sempre estão presentes, já que a secreção de epinefrina e norepinefrina ocorre de maneira episódica. O objetivo deste trabalho é relatar o caso de um cão maltês macho, de nove anos de idade, assintomático, que apresentava uma massa adrenal identificada ao acaso durante um exame ultrassonográfico. Foi indicado o tratamento cirúrgico, que no entanto não foi realizado inicialmente. Após oito meses, o animal apresentou trombo neoplásico em veia cava caudal, sendo submetido a adrenalectomia e trombectomia. A análise histopatológica revelou um feocromocitoma maligno e trombo neoplásico. Ressaltase aqui a importância do diagnóstico e do tratamento cirúrgico dos tumores adrenais, mesmo quando identificados ao acaso em animais assintomáticos. Atualmente, cinco anos após o tratamento cirúrgico, o paciente encontra-se estável, saudável e sem recidiva ou evidências de metástase do tumor.

Pheochromocytoma is a catecholamine secreting tumor that may be difficull to diagnose because clinical signs are not always present due to the episodic secretion of epinephrine and norepinephrine. We report a case of pheochromocytoma in an asymptomatic 9-year-old intact male maltese, The adrenal mass was an incidental finding during an abdominal ultrasonographic exam. Surgical treatment was declined by the owner. Eight months laler the animal was presented with neoplastic thrombus in the caudal vena cava, and was submitted to adrenalectomy with venotomy and thrombectomy. The histopathological exam confirmed the diagnosis malignant pheochromocytoma and neoplastic thrombus. The importance of diagnosis and surgical treatment of adrenal tumors, including cases of incidental finding in asymptomatic animais is emphasized here. Currently, five years after surgery, the dog is stable, healthy and without recurrence or evidences of tumor metastasis.

El feocromocitoma es un tumor secretor de catecolaminas de difícil diagnóstico debido a que pocas veces presenta signos clínicos, dado que la secreción de epinefrina y norepinefrina no son constantes. El objetivo de este trabajo fue relatar el caso de un perro Maltés macho de nueve años, asintomático, que presentaba una masa adrenal encontrada casualmente durante un examen ecográfico. Se indicó el tratamiento quirúrgico, pero no fue realizado en forma inmediata. Ocho meses después, el paciente presentó un trombo neoplásico en vena cava caudal, por lo que se realizó una adrenalectomía y trombectomía. En el examen histopatológico fue diagnosticado un feocromocitoma maligno y presencia de trombo neoplásico. Destacamos la importancia del diagnóstico y del tratamiento quirúrgico de los tumores adrenales, aún cuando se los identifica en forma casual en animales asintomáticos. Actualmente, cinco años después de la cirugía, el paciente se encuentra estable, sin alteraciones clínicas ni evidencias de metástasis del tumor.

Feocromocitoma maligno em cão: relato de caso / Malignant pheochromocytoma in a dog: case report / Feocromocitoma maligno en perro: relato de caso

O feocromocitoma é um tumor secretor de catecolaminas de difícil diagnóstico, pois as manifestações clínicas nem sempre estão presentes, já que a secreção de epinefrina e norepinefrina ocorre de maneira episódica. O objetivo deste trabalho é relatar o caso de um cão maltês macho, de nove anos de idade, assintomático, que apresentava uma massa adrenal identificada ao acaso durante um exame ultrassonográfico. Foi indicado o tratamento cirúrgico, que no entanto não foi realizado inicialmente. Após oito meses, o animal apresentou trombo neoplásico em veia cava caudal, sendo submetido a adrenalectomia e trombectomia. A análise histopatológica revelou um feocromocitoma maligno e trombo neoplásico. Ressaltase aqui a importância do diagnóstico e do tratamento cirúrgico dos tumores adrenais, mesmo quando identificados ao acaso em animais assintomáticos. Atualmente, cinco anos após o tratamento cirúrgico, o paciente encontra-se estável, saudável e sem recidiva ou evidências de metástase do tumor.(AU)

Pheochromocytoma is a catecholamine secreting tumor that may be difficull to diagnose because clinical signs are not always present due to the episodic secretion of epinephrine and norepinephrine. We report a case of pheochromocytoma in an asymptomatic 9-year-old intact male maltese, The adrenal mass was an incidental finding during an abdominal ultrasonographic exam. Surgical treatment was declined by the owner. Eight months laler the animal was presented with neoplastic thrombus in the caudal vena cava, and was submitted to adrenalectomy with venotomy and thrombectomy. The histopathological exam confirmed the diagnosis malignant pheochromocytoma and neoplastic thrombus. The importance of diagnosis and surgical treatment of adrenal tumors, including cases of incidental finding in asymptomatic animais is emphasized here. Currently, five years after surgery, the dog is stable, healthy and without recurrence or evidences of tumor metastasis.(AU)

El feocromocitoma es un tumor secretor de catecolaminas de difícil diagnóstico debido a que pocas veces presenta signos clínicos, dado que la secreción de epinefrina y norepinefrina no son constantes. El objetivo de este trabajo fue relatar el caso de un perro Maltés macho de nueve años, asintomático, que presentaba una masa adrenal encontrada casualmente durante un examen ecográfico. Se indicó el tratamiento quirúrgico, pero no fue realizado en forma inmediata. Ocho meses después, el paciente presentó un trombo neoplásico en vena cava caudal, por lo que se realizó una adrenalectomía y trombectomía. En el examen histopatológico fue diagnosticado un feocromocitoma maligno y presencia de trombo neoplásico. Destacamos la importancia del diagnóstico y del tratamiento quirúrgico de los tumores adrenales, aún cuando se los identifica en forma casual en animales asintomáticos. Actualmente, cinco años después de la cirugía, el paciente se encuentra estable, sin alteraciones clínicas ni evidencias de metástasis del tumor.(AU)

Case Report: An incidentaloma that catches your eye - adrenal myelolipoma.

Background: Adrenal incidentaloma refers to the incidental finding of a tumor in the adrenal gland, where nonfunctional forms are the most common variant. Myelolipoma is a rare (0.08-0.4%) occurrence characterized by adipose and hematopoietic tissue. The aim of this case report is to describe the diagnosis and appropriate management of a myelolipoma in an asymptomatic patient, which was originally considered an incidental hepatic hemangioma prior to being identified as a giant adrenal adenoma. Case description: The patient was a 54 year old obese female with a recent diagnosis of diabetes type II and dyslipidemia with recent ultrasound imaging suggestive of a hepatic hemangioma. An MRI was performed revealing a 7x6cm lesion in the right adrenal area indicating a giant adrenal adenoma. An adrenalectomy was performed without complications. The pathology report identified a myelolipoma. Discussion: The incidence of myelolipoma has recently increased due to advances in radiological techniques. Its etiology is unclear and the most accepted theories support a myeloid cell metaplasia in the embryonic stage as a result of stress, infections, or adrenocorticotropic hormone or erythropoietin stimulus. Contributing components may include bone morphogenetic protein 2 and ß-catenin, as well as the presence of the chromosomal translocation (3, 21) (q25; p11). Despite its benign nature, the association with other adrenal lipomas must be ruled out. A biochemical evaluation is essential for detecting subclinical states, such as Cushing syndrome and pheochromocytoma. Conclusion: Adrenal myelolipomas are rare benign tumors that are generally asymptomatic. Uncertainty still exists surrounding their etiology. Surgical management depends on hormone production, tumor size, high risk features on imaging and patient consent.  Additional information is needed to better understand myelolipomas, their etiology, and clinical management.  Incidentalomas may confuse the physician and patient. Ensuring proper multidisciplinary management based on the clinical guidelines of endocrinology allowed a satisfactory resolution of this case.

Primary hyperaldosteronism in a cat / Hiperaldosteronismo primário em um gato

Background: Primary hyperaldosteronism in cats occurs as a consequence of excessive hormone production by adrenal glands, mainly in adrenocortical tumors. Excessive aldosterone secretion leads to hypokalemia, hypernatremia and systemic hypertension, with consequent clinical signs of muscle weakness, cervical ventroflexion and ocular abnormalities. This report describes a cat diagnosed with primary hyperaldosteronism successfully treated with unilateral adrenalectomy.Case: A female mixed breed 10-year-old cat was presented with dehydration, severe cervical ventroflexion, bilateral mydriasis and ocular hemorrhage. Systolic blood pressure was 300 mmHg. Anti hypertensive drugs (amlodipine and benazepril) were started. The animal was referred to ophthalmologist, with diagnosis of bilateral partial retinal detachment without blindness. Biochemical results showed severe hypokalemia (2.7 mEq / L) and mild azotemia, BUN 71 mg / dL and creatinine 2.2 mg / dL. Total T4 was within the reference range. Oral potassium citrate (100 mg / kg orally every 12 h) was administered. However, after five days, hypokalemia was more intense (2.4 mEq / L), and systolic blood pressure was 280 mmHg. Due to severe hypertension associated with refractory hypokalemia, hyperaldosteronism was suspected. Serum aldosterone was 1239.75 pg / mL. Abdominal ultrasound revealed a large heterogeneous mass in the...(AU)

O hiperaldosteronismo primário (HAP) é uma doença endócrina caracterizada pela secreção autônoma excessiva de aldosterona pela zona glomerulosa da glândula adrenal. O aumento da secreção de aldosterona resulta na retenção de líquido, hipernatremia, hipocalemia e hipertensão arterial. Como consequência, a maior parte dos gatos acometidos apresenta fraqueza muscular e sinais oculares associados à hipertensão sistêmica [17].As causas do HAP incluem neoplasia adrenal uni ou bilateral, e hiperplasia idiopática da zona glomerulosa da adrenal. A neoplasia é o achado mais frequente em felinos, incluindo adenomas e carcinomas [1,5,6,13,15,18].Os sinais clínicos mais frequentes incluem sinais de hipocalemia, como letargia, fraqueza muscular, ventroflexão cervical, ataxia e mialgia; poliúria, polidipsia, perda de peso, hipertensão arterial e anormalidades oculares associadas à hipertensão, como midríase, cegueira e descolamento de retina [8,11,12].O diagnóstico definitivo é realizado através da dosagem do nível sérico de aldosterona e concentração plasmática de renina. A ultrassonografia e tomografia computadorizada são úteis na localização e estadiamento do tumor [8,12]. O tratamento cirúrgico é o indicado para as neoplasias sem a presença de metástases detectáveis [8,11].O objetivo deste trabalho é relatar o caso de uma gata diagnosticada com hiperaldosteronismo primário e tratada...(AU)

Primary hyperaldosteronism in a cat / Hiperaldosteronismo primário em um gato

Background: Primary hyperaldosteronism in cats occurs as a consequence of excessive hormone production by adrenal glands, mainly in adrenocortical tumors. Excessive aldosterone secretion leads to hypokalemia, hypernatremia and systemic hypertension, with consequent clinical signs of muscle weakness, cervical ventroflexion and ocular abnormalities. This report describes a cat diagnosed with primary hyperaldosteronism successfully treated with unilateral adrenalectomy.Case: A female mixed breed 10-year-old cat was presented with dehydration, severe cervical ventroflexion, bilateral mydriasis and ocular hemorrhage. Systolic blood pressure was 300 mmHg. Anti hypertensive drugs (amlodipine and benazepril) were started. The animal was referred to ophthalmologist, with diagnosis of bilateral partial retinal detachment without blindness. Biochemical results showed severe hypokalemia (2.7 mEq / L) and mild azotemia, BUN 71 mg / dL and creatinine 2.2 mg / dL. Total T4 was within the reference range. Oral potassium citrate (100 mg / kg orally every 12 h) was administered. However, after five days, hypokalemia was more intense (2.4 mEq / L), and systolic blood pressure was 280 mmHg. Due to severe hypertension associated with refractory hypokalemia, hyperaldosteronism was suspected. Serum aldosterone was 1239.75 pg / mL. Abdominal ultrasound revealed a large heterogeneous mass in the...

O hiperaldosteronismo primário (HAP) é uma doença endócrina caracterizada pela secreção autônoma excessiva de aldosterona pela zona glomerulosa da glândula adrenal. O aumento da secreção de aldosterona resulta na retenção de líquido, hipernatremia, hipocalemia e hipertensão arterial. Como consequência, a maior parte dos gatos acometidos apresenta fraqueza muscular e sinais oculares associados à hipertensão sistêmica [17].As causas do HAP incluem neoplasia adrenal uni ou bilateral, e hiperplasia idiopática da zona glomerulosa da adrenal. A neoplasia é o achado mais frequente em felinos, incluindo adenomas e carcinomas [1,5,6,13,15,18].Os sinais clínicos mais frequentes incluem sinais de hipocalemia, como letargia, fraqueza muscular, ventroflexão cervical, ataxia e mialgia; poliúria, polidipsia, perda de peso, hipertensão arterial e anormalidades oculares associadas à hipertensão, como midríase, cegueira e descolamento de retina [8,11,12].O diagnóstico definitivo é realizado através da dosagem do nível sérico de aldosterona e concentração plasmática de renina. A ultrassonografia e tomografia computadorizada são úteis na localização e estadiamento do tumor [8,12]. O tratamento cirúrgico é o indicado para as neoplasias sem a presença de metástases detectáveis [8,11].O objetivo deste trabalho é relatar o caso de uma gata diagnosticada com hiperaldosteronismo primário e tratada...

Conducta ante un incidentaloma suprarrenal / Behavior to be adopted to face adrenal incidentaloma

El incidentaloma suprarrenal en la actualidad es uno de los dilemas diagnóstico-terapéuticos mas frecuentes en la Endocrinología. Tiene un pico de aparición entre los 50 y los 70 años de edad, y se asocia a una mayor frecuencia de obesidad, diabetes mellitus e hipertensión arterial. La etiología es diversa, pero alrededor del 80 por ciento son adenomas benignos no funcionantes. A pesar de ello, la aparición de un carcinoma (por su agresividad) y disfunción hormonal (por la repercusión desfavorable sobre algunos factores de riesgo cardiovascular y el sistema óseo), pueden ensombrecer el pronóstico. La conducta estará basada en las definiciones mencionadas: naturaleza y función, y las 2 modalidades más empleadas son la cirugía adrenal (de preferencia laparoscópica, excepto si el tumor tiene criterios imagenológicos de malignidad); y la conservadora, que conlleva un seguimiento de, al menos, 5 años. El conocimiento de sus características, puede favorecer el mejor tratamiento a los pacientes con diagnóstico de un tumor suprarrenal incidental, y la garantía de una mejor calidad de vida(AU)

Adrenal incidentaloma is at present one of the commonest diagnostic/therapeutic dilemmas in endocrinology. It mainly occurs in the 50 to 70 years age group and is associated to higher frequency of obesity, diabetes mellitus and blood hypertension. Its etiology is varied, but around 80 percent of them are benign non-functioning adenomas. Despite the above-mentioned, the occurrence of carcinoma (based on aggressiveness) and hormonal dysfunction (negative impact on some cardiovascular risk factors and on the osseous system) can make prognosis gloomy. The behavior to be adopted will consider the stated definitions: nature and function, and the most used variants are adrenal surgery (mainly laparoscopic, except for a tumor with imaging-based malignancy criteria) and conservative surgery that implies at least five-year follow-up of the patient. Knowledge about the characteristics of adrenal incidentaloma may support a best treatment for patients suffering incidental adrenal tumor and assure them a better quality of life(AU)

Actualización en el diagnóstico por imágenes de los tumores adrenales / Update on imaging diagnosis of adrenal tumors

Cada vez es más frecuente el diagnóstico por imágenes de los tumores adrenales. La caracterización y diferenciación de los adenomas benignos y las lesiones malignas es muy importante, sobre todo, en el paciente con enfermedad maligna o funcionante. La tomografía axial computarizada, la resonancia magnética nuclear, y la tomografía con emisión de positrones acoplada a tomografía axial computarizada, permiten, con gran precisión, caracterizar y diferenciar, de forma no invasiva, las masas adrenales. En esta revisión se explican los principios fisiológicos en que se basan estas técnicas, sus ventajas y sus limitaciones. Se describen las principales características de imagen de los tumores suprarrenales más frecuentes, y se propone un algoritmo para el uso de estos medios diagnósticos(AU)

Imaging diagnosis of adrenal tumors is increasingly frequent. Characterization and differentiation of benign adenomas and malignant injures is very important, mainly in the patient with malignant or functioning disease. Computed tomography, magnetic resonance imaging and positron emission tomography/computed tomography allowed greater precision in characterizing and differentiating the adrenal masses in a non-invasive way. This review explained the physiological principles supporting these techniques, its advantages and restrictions. The main characteristics of the most frequent adrenal tumor images were described, in addition to submitting an algorithm for the use of these diagnostic means(AU)

Feocromocitoma maligno: comunicación de 2 casos tratados con cirugía de mínima invasión / Malignant pheochromocytoma treated with minimally invasive surgery

Introducción: El Feocromocitoma es un tumor de células cromafines productor de catecolaminas. Su variedad maligna es muy poco frecuente. Objetivo: Describir 2 casos de Feocromocitoma maligno tratados con cirugía mínimamente invasiva. Casos clínicos: Presentamos 2 pacientes de sexo femenino, de 31 y 51 años respectivamente. La primera con historia de hipertensión arterial de larga data y uso de múltiples fármacos. La segunda paciente debuta con dolor abdominal. En ambos casos los estudios radiológicos y metabólicos confirmaron el diagnóstico de Feocromocitoma. A la primera paciente se le realizó un procedimiento laparoscópico puro, mientras que a la segunda paciente se le realizó un procedimiento laparoscópico asistido por robot. En ambos casos fue necesario realizar adrenalectomía en bloque con el riñón, por compromiso del hilio renal. No hubo complicaciones severas intra ni post-operatorias, como tampoco conversión a cirugía abierta. La histopatología confirmó el diagnóstico de Feocromocitoma maligno con metástasis ganglionares regionales y márgenes quirúrgicos negativos. Ambas se mantienen en control, sin evidencia de recurrencia. Conclusión: El Feocromocitoma maligno puede ser manejado de forma mínimamente invasiva, con morbilidad y resultados oncológicos comparables a cirugía abierta.

Introduction: Pheochromocytoma is a tumor derived from chromaffin cells that produces cathecolamines. Malignant pheochromocytomas are extremely rare. Aim: To describe 2 cases of malignant Pheochromocytoma treated with pure laparoscopic and robot assisted surgery. Clinical cases: Two female patients, 31 and 51 years of age, presented with a history of high blood pressure and abdominal pain. Radiologic and metabolic studies confirmed the diagnosis of Pheochromocytoma in both patients. A pure laparoscopic approach was performed in the first patient while a robotic assisted laparoscopy was done in the second patient. In both cases, adrenalectomy with in bloc nephrectomy was performed due to compromise of the renal sinus. There were no complications or conversion to open surgery. Histopathology confirmed the diagnosis of malignant Pheochromocytoma with metastasis to regional nodes and negative surgical margins. At follow-up, there is no evidence of recurrence. Conclusion: Malignant Pheochromocytoma can be treated with minimally invasive surgery, with morbidity and oncological outcomes similar to open approach.