Enhancement patterns of adrenal nodules on magnetic resonance imaging

ABSTRACT Objective: To compare enhancement patterns of typical adrenal adenomas, lipid-poor adenomas, and non-adenomas on magnetic resonance imaging (MRI). Materials and Methods: Evaluation of adrenal nodules larger than 1.0 cm, with at least 2-year follow-up, evaluated on MRI in January 2007 and December 2016. Two different protocols were included - upper abdomen MRI (delayed phase after 3 minutes) and abdomen and pelvis MRI (delayed phase after 7 minutes) - and nodules were divided in typical adenomas (characterized on out-of-phase MRI sequence), lipid-poor adenomas (based on follow-up imaging stability) and non-adenomas (based on pathological finding or follow-up imaging). T2-weighted and enhancement features were analyzed (absolute and relative washout and enhancement curve pattern), similarly to classic computed tomography equations. Results: Final cohort was composed of 123 nodules in 116 patients (mean diameter of 1.8 cm and mean follow up time of 4 years and 3 months). Of them, 98 (79%) nodules had features of typical adenomas by quantitative chemical shift imaging, and demonstrated type 3 curve pattern in 77%, mean absolute and relative washout of 29% and 16%, respectively. Size, oncologic history and T2-weighted features showed statistically significant differences among groups. Also, a threshold greater than 11.75% for absolute washout on MRI achieved sensitivity of 71.4% and specificity of 70.0%, in differentiating typical adenomas from non-adenomas. Conclusion: Calculating absolute washout of adrenal nodules on MRI may help identifying proportion of non-adenomas.

Mielolipoma suprarrenal, revisión de literatura en México a propósito de dos casos / Adrenal myelolipoma, review of the literature in Mexico apropos of two cases

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

Challenging risk factors for right and left laparoscopic adrenalectomy: A single centre experience with 272 cases

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.

Limited significance of repeated long-term radiological and hormonal examination in nonfunctioning adrenal incidentalomas

ABSTRACT Purpose: The purposes of the present study were to evaluate growth rate of nonfunctioning adrenal incidentalomas (AIs) and their development to hormonal hypersecretion on follow-up. Materials and methods: A retrospective study was conducted from the electronic medical records. A total of 314 patients were diagnosed with adrenal tumors between 2000 and 2016. After excluding patients who had overt adrenal endocrine disorders or whose adrenal tumors were clinically diagnosed as metastatic malignancies, we investigated 108 patients with nonfunctioning AIs including characteristics, the treatment, the way of follow-up and pathology. Results: Fifteen patients received immediate adrenalectomy because of the initial tumor size or patient's preference. Pathological examination revealed malignancy in 2 patients. In the remaining 93 patients, radiological examinations were performed periodically. Tumor enlargement of ≥ 1.0cm was observed in 8.6% of the patients who were followed up as nonfunctioning AIs with a median follow-up period of 61.5 months (range: 4-192). Eleven patients underwent adrenalectomy. On the pathological examinations, all of the tumors, which showed a size increase, were diagnosed as benign tumors. Regarding the followed up patients without adrenalectomy, only 2.4% of the patients had tumor enlargement during the prolonged follow-up. Furthermore, none of the patients developed hormonal hypersecretion or clinical signs such as obesity, glucose intolerance or poorly controlled hypertension. Conclusions: Tumor enlargement of AIs did not correlate with malignancy. The value of repeat radiological and hormonal examinations may be limited in the long-term follow-up of patients whose AIs are not enlarged.

Carcinoma adrenal em crianças: estudo longitudinal em minas gerais, brasil / Adrenal carcinoma in children: longitudinal study in minas gerais, brazil

RESUMO Objetivo: Analisar as características clínicas, laboratoriais e histopatológicas e o percurso até o estabelecimento do diagnóstico e do tratamento de pacientes com carcinoma de suprarrenal (CSR). Métodos: Estudo retrospectivo com 13 pacientes tratados no serviço de oncologia pediátrica do Hospital das Clínicas da Universidade Federal de Minas Gerais (HC-UFMG) entre 2004 e 2015. Resultados: A idade ao diagnóstico variou de 1,0 a 14,8 anos (mediana: 2,0 anos). As manifestações de hipercortisolismo foram identificadas em todos os casos, e as de virilização, em todas as meninas. Todos os pacientes preencheram os critérios de Weiss para diagnóstico histopatológico de CSR. A imuno-histoquímica foi realizada em 61,5% dos casos. A maioria dos pacientes apresentou doença em estádio I (76,9%). Todos foram submetidos à ressecção tumoral total. Dois pacientes (estádios III e IV) receberam quimioterapia associada ao mitotano. O único óbito observado foi do paciente com doença em estádio IV. A probabilidade de sobrevida global para todo o grupo aos 5,0 anos foi de 92,3±7,4%. A mediana de tempo entre o início dos sintomas e o diagnóstico foi de 9,5 meses, e de 6,0 meses entre a primeira consulta e o início do tratamento. Conclusões: A baixa idade ao diagnóstico, o predomínio de casos com doença localizada e a ressecção tumoral completa - com apenas um caso de ruptura de cápsula tumoral - são possivelmente a explicação para a evolução favorável da população estudada. O longo percurso entre o início dos sintomas e o diagnóstico sugere a importância da capacitação dos pediatras para o reconhecimento precoce dos sinais e dos sintomas do CSR.

ABSTRACT Objective: To analyze clinical, laboratory and histopathological features and the path to diagnosis establishment and treatment of patients with adrenal carcinoma (AC). Methods: Retrospective study with 13 patients assisted at the pediatric oncology service of Hospital das Clínicas, Universidade Federal de Minas Gerais, Brazil, between 2004 and 2015. Results: Age at diagnosis ranged from 1.0 to 14.8 years (median: 2.0 years). Manifestations of hypercortisolism were identified in all cases and virilization in all girls. All patients met the Weiss criteria to AC histopathological diagnosis. Immunohistochemistry was performed in 61.5% of the cases. Most patients had stage I disease (76.9%). All subjects were submitted to total tumor resection. Two patients (stages III and IV disease) received chemotherapy associated to mitotane. The only death case was that of a patient with stage IV disease. The probability of overall survival for the entire group up to 5.0 years was 92.3±7.4%. The median time between the onset of symptoms and diagnosis was 9.5 months, and 6.0 months between first visit and start of treatment. Conclusions: Low age at diagnosis, predominance of cases with localized disease and complete tumor resection - with only one case of tumor capsule rupture - can possibly explain the favorable evolution of the studied population. The long period between onset of symptoms and diagnosis highlights the importance of training pediatricians for early recognition of AC signs and symptoms.

Robotic excision of complex adrenal mass with retrocaval extension and encasement of renal hilum with renal preservation

ABSTRACT Objective: The purpose of this video is to present robotic excision of a complex adrenal mass with retrocaval extension and encasement of renal hilum in a 16 year old boy. Biochemical screening was negative for metabolically active component. Computerized tomographic scan with contrast revealed a homogenous mass of approximately 10.8 cm x 6.2 cm x 4.2 cm in the suprarenal area on right side that was extend-ing behind inferior vena cava and encasing renal hilar vessels. Imaging findings were that of a classical ganglioneuroma. Material and methods: Robot assisted laparoscopic adrenalectomy with sparing of renal hilar vasculature was performed. With patient in lateral position, five ports were used, including one for liver retraction. Da Vinci® system with four arms was docked from over the right shoulder. The displaced renal hilar structures were identified by opening Gerota's fascia. Mass was dissected completely and removed through Pfan-nensteil incision. Results: Duration of procedure was 345 minutes and console time was 290 minutes. Blood loss was 250 mL. Post-operative renal doppler showed normal blood flow. He was discharged on post-operative day three. Histopathologic examination of specimen revealed ganglioneuroma arising from adrenal gland. Conclusion: Ganglioneuroma is a rare adrenal tumor with good prognosis on surgical removal. The advent of robotic surgery has made complex surgical procedures involving vital structures like inferior vena cava be performed using minimally invasive techniques without compromising oncologic principles.

Síndrome del dedo azul como manifestación de feocromocitoma / Blue toe syndrome as a clinical finding of pheochromocytoma

Los feocromocitomas son tumores que proceden de las células cromafines del sistema nervioso simpático y actúan sintetizando y liberando catecolaminas. Suelen presentarse entre la cuarta y quinta década de la vida y tienen presentaciones clínicas muy diversas. Ocurren solamente en 0.1-0.2% de la población hipertensa, constituyen una causa tratable y curable de hipertensión arterial, así como de otras manifestaciones derivadas de la liberación incontrolada de catecolaminas. La isquemia arterial periférica secundaria a la liberación masiva de aminas por un feocromocitoma es muy infrecuente. Aquí se presenta un caso clínico de feocromocitoma manifestado como síndrome del dedo azul en un paciente con pulsos distales conservados y el antecedente de mal control tensional a pesar de tratamiento con dos fármacos.

Pheochromocytomas are tumors that arise from chromaffin cells of the sympathetic nervous system and act by synthesizing and releasing catecholamines. They usually occur between the fourth and fifth decade of life and have a very wide clinical presentation. They occur only in 0.1-0.2% of the hypertensive population and represent a treatable and curable cause of arterial hypertension, as well as other symptoms derived from the uncontrolled secretion of catecholamines. Peripheral arterial ischemia secondary to massive amines release by a pheochromocytoma is a very uncommon condition. Here we report a case of pheochromocytoma manifested as blue finger syndrome in a patient with palpable distal pulses and history of poor blood pressure control despite treatment with two drugs.

Primary malignant tumors of the adrenal glands

Malignancy must be considered in the management of adrenal lesions, including those incidentally identified on imaging studies. Adrenocortical carcinomas (ACCs) are rare tumors with an estimated annual incidence of 0.7-2 cases per year and a worldwide prevalence of 4-12 cases per million/year. However, a much higher incidence of these tumors (>15 times) has been demonstrated in south and southeastern Brazil. Most ACCs cause hypersecretion of steroids including glucocorticoids and androgens. ACC patients have a very poor prognosis with a 5-year overall survival (OS) below 30% in most series. Pheochromocytoma or paraganglioma (PPGL) is a metabolically active tumor originating from the chromaffin cells of the adrenal medulla. The incidence of PPGL is 0.2 to 0.9 cases per 100,000 individuals per year. Pheochromocytomas are present in approximately 4-7% of patients with adrenal incidentalomas. Classically, PPGL manifests as paroxysmal attacks of the following 4 symptoms: headaches, diaphoresis, palpitations, and severe hypertensive episodes. The diagnosis of malignant PPGL relies on the presence of local invasion or metastasis. In this review, we present the clinical and biochemical characteristics and pathogenesis of malignant primary lesions that affect the cortex and medulla of human adrenal glands.

Feocromocitoma quístico gigante: reporte de un caso / Pheochromocytoma giant cystic: a case report

El feocromocitoma quístico gigante es tumor adrenal raro en el que predomina el curso asintomático; por lo que muchos de los casos no son diagnosticados hasta el momento de la cirugía. La simple movilización del tumor se asocia con el paso a la sangre de grandes cantidades de catecolaminas y a una elevada morbimortalidad.; por esta razón la cirugía per se y su manejo perioperatorio constituyen un enorme desafío. En este artículo se presenta el caso de un feocromocitoma gigante maligno (35 cm) que ocupaba todo el hemiabdomen derecho. Aun con el diagnóstico preoperatorio de feocromocitoma, el bloqueo farmacológico preoperatorio y las medidas intraoperatorias, el paciente falleció poco antes de que finalizara la cirugía.

The giant cystic pheochromocytoma is a rare adrenal tumor in the predominantly asymptomatic course; so many cases are not diagnosed until the time of surgery. The simple mobilization of the tumor is associated with the passage to the blood of large amounts of catecholamines and high morbidity and mortality. So the surgery itself and perioperative management are a huge challenge. This article describes the case of a malignant giant pheochromocytoma (35 cm) which occupied the entire right abdomen. Even with the preoperative diagnosis of pheochromocytoma, pharmacological blockade preoperative and intraoperative measures, the patient died shortly before the end of surgery.

Mielolipoma adrenal gigante: Adrenalectomía derecha laparoscópica / Giant adrenal myelolipoma. Right laparoscopic adrenalectomy

El mielolipoma es un tumor adrenal poco frecuente. Se trata de un tumor benigno no funcionante compuesto por tejido adiposo y hematopoyético. Presentamos el caso de una paciente de 33 años de edad con diagnóstico de incidentaloma adrenal de 14 cm de diámetro no funcionante. Los estudios imagenológicos presentaban características sugestivas de mielolipoma. Debido a la naturaleza benigna del tumor se decidió el abordaje laparoscópico. La anatomía patológica informó un mielolipoma de 444 gramos. La paciente evolucionó favorablemente en el postoperatorio. El abordaje laparoscópico para la resección del mielolipoma gigante fue factible y exitoso.

Adrenal myelolipoma is a rare, benign, non-functioning tumor. It is composed by fat and hematopoietic tissues. We present the case of a 33-year-old woman with diagnosis of a 14 cm diameter non-functioning right adrenal incidentaloma, with imaging features suggestive of myelolipoma. Based on the benign nature of the tumor, laparoscopic resection was performed. Histopathology showed a myelolipoma, weighting 444 grams. The patient evolved with an uneventful postoperative period. Laparoscopic adrenalectomy for a giant myelolipoma was feasible and successful.

Adenoma negro de la glándula suprarrenal (black adenoma) / Black adrenal adenoma

Introduction: the black adenoma is a rare tumor of the adrenal gland. Clinical case: a male patient treated for lung carcinoma, was found to have an incidental adrenal mass. Due to a suspicious of metastatic disease, a laparoscopic adrenalectomy was done. The biopsy showed a black adenoma. After 11 years of follow-up, there is no evidence of recurrence. Conclusion: the black adenoma of the adrenal gland is a rare, benign and non-functioning tumor. The accurate diagnosis is done only by histological studies.

Introducción: el adenoma negro de la glándula suprarrenal, o black adenoma, es una patología de baja frecuencia dentro las masas suprarrenales. Caso clínico: presentamos el caso de un paciente tratado por un cáncer pulmonar, con el diagnóstico incidental de una masa suprarrenal izquierda que requirió extirpación laparoscópica por la sospecha de metástasis. La biopsia confirmó la presencia de un adenoma negro. El paciente se encuentra vivo 11 años después. Conclusión: el adenoma negro es un tumor suprarrenal raro, benigno, no funcionante, cuyo diagnóstico es solamente histológico.

Nasal paraganglioma: A case report and literature review

Introduction: Paragangliomas are neuroendocrine tumors that most commonly originate in the adrenal gland, a type that is called pheochromocytoma; however, 5-10% of paragangliomas are extra-adrenal and may arise in any area between the neck and pelvic region along the sympathetic nervous system. Those located in the head and neck comprise 3% of extra-adrenal tumors, with the majority originating in the tympanic-jugular region and carotid body. Objective: To present a rare case of nasal paraganglioma and review the literature. Case report: The patient was submitted to medial subtotal maxillectomy, and her clinical findings, diagnostic data, and treatment outcome were recorded. Conclusion: Paragangliomas are considered benign tumors, but they occasionally display a malignant character. The most important finding in this case was the need for total resection of the tumor to avoid recurrence...

Miocardiopatía dilatada y feocromocitoma / Dilated myocardiopathy and pheochromocytoma

El feocromocitoma, un tumor de baja incidencia con un comportamiento benigno en la mayoría de los casos, puede ser el responsable de extensas manifestaciones cardiovasculares. Las manifestaciones sobre el miocardio pueden ser de profundas consecuencias, pues llegan a producir una miocardiopatía dilatada con insuficiencia cardiaca severa. La exéresis del tumor suele ser curativa en el 90 por ciento de los casos y el manejo perioperatorio muy complejo. Se presenta un paciente joven portador de un feocromocitoma, que sometido a la larga acción de las catecolaminas llegó a desarrollar una miocardiopatía dilatada con criterio de trasplante cardiaco. La cirugía adrenal fue curativa en nuestro paciente(AU)

Pheochromocytoma, a low incidence tumor of benign behavior in most of cases, can cause extensive cardiovascular manifestations. These manifestations on the myocardium can have deep consequences since they may lead to dilated myocardiopathy with severe heart failure. The excision of tumor may be the curative solution for 90 percent of cases but the perioperative management is very complex. This is the case of a young patient who carries pheochromocytoma and after a long-acting catecholemine-based treatment, he developed dilated myocardiopathy, and the medical criterion was that he required heart transplantation. The adrenal surgery was successfully healing in our patient(AU)

Síndrome de Cushing durante el embarazo: manifestaciones cutáneas secundarias a un tumor suprarrenal / Cushing's syndrome secondary to adrenal tumor during pregnancy

El síndrome de Cushing secundario a tumor suprarrenal es una patología infrecuente en el embarazo. Su presencia se traduce en un aumento significativo de la morbimortalidad materna y fetal. Muchas de sus características clínicas son enmascaradas por los cambios propios de una gestación fisiológica, lo que dificulta el diagnóstico. En este contexto las manifestaciones dermatológicas pueden ser de gran utilidad en la sospecha clínica precoz. Se presenta el caso de una paciente de 26 años de edad que en el segundo trimestre de embarazo inicia un cuadro caracterizado por acné inflamatorio severo e hirsutismo. La gestación se interrumpe a las 35 semanas por síndrome hipertensivo del embarazo y rotura prematura de membranas. Durante el puerperio consulta al dermatólogo presentando aún las lesiones descritas, además de estrías violáceas gruesas en abdomen, facie de luna, obesidad centrípeta y máculas purpúricas en zonas de presión. Se diagnóstica síndrome de Cushing. El estudio confirmatorio y etiológico dirigido evidenció un adenoma de corteza suparrenal. El diagnóstico de laboratorio es difícil debido a los cambios físicos y de laboratorio habituales del embarazo que pueden mimetizar los hallazgos propios de la enfermedad. Los hallazgos dermatológicos pueden ser de gran valor para un diagnóstico y tratamiento precoz. Las lesiones dermatológicas secundarias al hipercortisolismo fueron de difícil manejo...

Cushing's syndrome secondary to adrenal tumor is a rare pathology in pregnancy. Its presence results in a significant increase in maternal and fetal morbimortality. Many of its clinical features are masked by the typical changes of a physiological pregnancy, which make difficult the diagnosis. Indeed, skin manifestations may be useful in early clinical suspicion. We report a case of a 26 year old in the second trimester with severe inflammatory acne and hirsutism. The pregnancy is interrupted at 35 weeks for pregnancy-induced hypertension syndrome and premature rupture ovular membranes. During the puerperium, patient consulted a dermatologist presenting still the injuries described, as well as thick purple striae throughout the abdomen, moon face, truncal obesity and purpuric macules in pressure zones. Cushing's syndrome is diagnosed. The study showed a cortex suprarenal adenoma. Laboratory diagnosis is difficult because the pregnancy physical changes and routine laboratory findings that can mimic the disease themselves. Dermatological findings may be valuable for diagnosis and early treatment. The skin lesions secondary to hypercortisolism were difficult to manage...

Left-sided giant adrenal myelolipoma secreting catecholamine.

Adrenal myelolipoma (AML) is a rare benign tumor composed of mature adipose and hematopoietic tissue. Most of these patients are asymptomatic and the tumors are non-secreting. We present a case with a large functional adrenal myelolipoma, wherein the patient was hypertensive and biochemistry revealed increase in 24 hours urinary Vanillylmandelic Acid (VMA), a metabolite of catecholamine. The mass was removed surgically and diagnosed as adrenal myelolipoma on histopathological examination. Both his blood pressure and urinary VMA returned to normal following surgery, which suggested that the mass was functioning and was secreting catecholamine. To the best of our knowledge, a catecholamine secreting adrenal myelolipoma has been reported in the literature only once previously. The association of hypertension and adrenal myelolipoma may not be entirely coincidental, as it may be associated with secreting catecholamine, as seen in our case. We also review the literature on functioning adrenal myelolipoma.

Adrenal ganglioneuroma / Ganglioneuroma de adrenal

Adrenal incidentalomas (AI) are unsuspected adrenal masses discovered during investigation of unrelated diseases, and are increasing in frequency. The majority of the AI is non-secretory adenomas, although it can also represent primary or metastatic malignant neoplasia. However, less frequent diseases should not be forgotten in the differential diagnosis. We describe a case of a young woman with an adrenal mass without clinical or laboratorial signs of hormonal hyperfunction. Diagnosis was performed after an episode of acute pyelonephritis in which the imaging study was carried out in order to exclude local complications. During the surgical procedure, the fish flesh aspect of the mass raised the suspicion of a neurogenic tumor, which was diagnosed in the histophatological analysis. The patient presented the most common manifestation of adrenal ganglioneuroma, an incidentaloma.

Incidentalomas adrenais (IA) são massas descobertas ao acaso na investigação de distúrbios não relacionados a patologias das glândulas adrenais e tem se tornado cada vez mais frequentes. A maioria dos casos é constituída por adenomas não secretores, embora possa também representar uma neoplasia maligna primária ou metastática. Porém, no diagnóstico diferencial, doenças menos prevalentes não podem ser esquecidas. Relatamos um caso de massa adrenal em mulher jovem, sem sinais de hiperfunção clínica ou laboratorial. O diagnóstico foi feito após episódio de pielonefrite aguda, em que o exame de imagem foi realizado para excluir complicações locais. Durante a abordagem cirúrgica, o aspecto em carne de peixe da lesão fez suscitar a hipótese de uma lesão de origem neural, a qual foi prontamente definida pelo exame histopatológico. A paciente se apresentava com a manifestação mais comum do ganglioneuroma de adrenal, um incidentaloma.

Manejo quirúrgico del incidentaloma suprarrenal / Surgical management of adrenal incidentaloma

Background: The adrenal incidentaloma is a lesion found on imaging studies for diagnosis of non-adrenal disorders. Most of these patients are not of surgical treatment. Our objective was to describe the clinical features and results of surgical management of adrenal incidentalomas in the Hospital de la Universidad de Chile. Material and Methods: Retrospective descriptive study. Period 2000 to 2009. Information was gathered from medical records and biopsies registers of patients with operated adrenal incidentaloma. Results: We evaluated 24 patients undergoing surgery, 66.7 percent female, 58.3 percent between 41 and 70 years. 58.3 percent were found in the study of abdominal pain. Surgical indications were: size ≥ 4 cm, enlarged in controls, atypical features in the abdominal and pelvic CT and/or functionality. 54.2 percent were ≥ 4 cm, 16.7 percent increase in size in controls, 45.8 percent had atypical features in the abdominal and pelvic CT and 33.3 percent were functioning, being the most frequent hypercortisolism. In 87.5 percent of patients the approach was laparoscopically. The morbidity was 12.6 percent (pneumonia, wound infection and stroke) and perioperative mortality was 0 percent. Discussion: The adrenal incidentaloma is a rare indication for surgery of adrenal tumors. Before the intervention should be studies in order to evaluate functionality and suspicion of malignancy. Functionating tumors and suspicious of malignancy should be resected, being the laparoscopic approach the election, likely in most cases.

Introducción: El incidentaloma suprarrenal es aquella lesión encontrada como hallazgo en estudios imagenológicos para el diagnóstico de desórdenes no suprarrenales. La gran mayoría de esos pacientes no son de resorte quirúrgico. Nuestro objetivo es describir las características clínicas y resultados del manejo quirúrgico de los incidentalomas suprarrenales operados en el Hospital Clínico de la Universidad de Chile. Material y Método: Estudio descriptivo-retrospectivo. Período 2000-2009. Se obtuvo información de fichas clínicas y registro de biopsias de los pacientes operados por incidentaloma suprarrenal. Resultados: Se evaluaron 24 pacientes intervenidos quirúrgicamente, el 66,7 por ciento sexo femenino, el 58,3 por ciento entre los 41 y los 70 años. El 58,3 por ciento se pesquisaron durante el estudio de dolor abdominal. Las indicaciones quirúrgicas fueron: tamaño ≥ 4 cm, aumento de tamaño en controles, características atípicas en la TC abdomino-pelviana y/o la funcionalidad. El 54,2 por ciento fueron ≥ de 4 cm, 16,7 por ciento aumento de tamaño en controles, 45,8 por ciento tenía características atípicas en la TC abdomino-pelviana y 33,3 por ciento fueron funcionantes, siendo lo más frecuente el hipercortisolismo. El 87,5 por ciento de los pacientes se abordaron por vía laparoscópica. La morbilidad fue de 12,6 por ciento (neumonía, infección de herida operatoria y AVE) y la mortalidad peri operatoria fue de 0 por ciento. Discusión: El incidentaloma suprarrenal es una indicación infrecuente de cirugía de tumores suprarrenales. Antes de la intervención deben estudiarse con el propósito de evaluar funcionalidad y sospecha de malignidad. Los tumores funcionantes y sospechosos de malignidad deben ser resecados, siendo la vía laparoscópica la de elección, factible en la gran mayoría de los casos.

MENX and MEN4

Multiple endocrine neoplasias are autosomal dominant disorders characterized by the occurrence of tumors in at least two endocrine glands. Two MEN syndromes have long been known and are well characterized: the MEN type 1 (MEN1) and type 2 (MEN2). These syndromes are caused by germline mutations in the MEN1 and RET genes, respectively, and have a different tumor spectrum. Recently, a variant of the MEN syndromes arose spontaneously in a rat colony and was named MENX. Affected animals consistently develop multiple endocrine tumors, with a spectrum that shares features with both MEN1 and MEN2 human syndromes. Genetic studies identified a germline mutation in the Cdkn1b gene, encoding the p27 cell cycle inhibitor, as the causative mutation for MENX. Capitalizing on these findings, heterozygous germline mutations in the human homologue, CDKN1B, were searched for and identified in patients with multiple endocrine tumors. As a consequence of this discovery, a novel human MEN syndrome, named MEN4, was recognized, which is caused by mutations in p27. Altogether, these studies identified Cdkn1b/CDKN1B as a novel tumor susceptibility gene for multiple endocrine tumors in both rats and humans. Here we review the characteristics of the MENX and MEN4 syndromes and we briefly address the main function of p27 and how they are affected by MENX/4-associated mutations.

Retroperitoneoscopic adrenalectomy in pheochromocytoma

Since the first laparoscopic adrenalectomy, the technique has evolved and it has become the standard of care for many adrenal diseases, including pheochromocytoma. Two laparoscopic accesses to the adrenal have been developed: transperitoneal and retroperitoneal. Retroperitoneoscopic adrenalectomy may be recommended for the treatment of pheochromocytoma with the same peri-operative outcomes of the transperitoneal approach because it allows direct access to the adrenal glands without increasing the operative risks. Although technically more demanding than the transperitoneal approach, retroperitoneoscopy can shorten the mean operative time, which is critical for cases with pheochromocytoma where minimizing the potential for intra-operative hemodynamic changes is essential. Blood loss and the convalescence time can be also shortened by this approach. There is no absolute indication for either the transperitoneal or retroperitoneal approach; however, the latter procedure may be the best option for patients who have undergone previous abdominal surgery and obese patients. Also, retroperitoneoscopic adrenalectomy is a good alternative for treating cases with inherited pheochromocytomas, such as multiple endocrine neoplasia type 2A, in which the pheochromocytoma is highly prevalent and frequently occurs bilaterally.