Carcinoma corticosuprarrenal virilizante en un niño / Virilizing adrenocortical carcinoma in a child

Introducción: Los tumores suprarrenales en niños son poco frecuentes y el carcinoma suprarrenal representa menos de un 10 %. En el prepúber, la manifestación más típica es el desarrollo de pubertad precoz. Objetivo: Describir las características clínicas, los procederes diagnósticos y terapéuticos de un paciente con carcinoma adrenal en edad pediátrica. Presentación de caso: Paciente de 8 años, masculino y de piel blanca con antecedentes de salud. Acude a la consulta por crecimiento de vello pubiano y aumento del pene en longitud y grosor de aproximadamente 2 años de evolución. En el examen físico se constatan aumento de la velocidad de crecimiento y signos sugestivos de virilización (voz gruesa, vello axilar, vello sexual púbico y genitales externos estadio III de Tanner). Se realizaron estudios hormonales que corroboraron el hiperandrogenismo por secreción endógena autónoma, con niveles de gonadotropinas suprimidas, niveles de testosterona y dehidroepiandrosterona elevados. También se realizaron estudios imagenológicos que evidenciaron edad ósea acelerada y la existencia de un tumor. Se realizó una adrenalectomía izquierda y se confirmó por anatomía patológica el carcinoma corticosuprarrenal virilizante izquierdo en estadío 2. Inició un tratamiento con quimioterapia por dicho diagnóstico y actualmente se mantiene en seguimiento. Conclusiones: Los carcinomas corticosuprarrenales en niños son mayoritariamente funcionantes y constituyen una de las causas de pubertad precoz periférica. Estos son infrecuentes y agresivos, por lo que la realización de estudios genéticos en familias con síndromes hereditarios contribuiría a su diagnóstico precoz para un adecuado tratamiento y mejor pronóstico(AU)

Introduction: Adrenal tumors in children are rare and adrenal carcinoma represents less than the 10 percent. In the prepubescent, the most typical manifestation is the development of early puberty. Objective: Describe the clinical characteristics and diagnostic and therapeutic procedures of a patient with adrenal carcinoma in a pediatric age. Case presentation: 8-year-old male, white-skinned patient with a history of health conditions. He attentds to the consultation due to pubic hair growth and penis enlargement in length and thickness of approximately 2 years of evolution. Physical examination shows increased growth rate and signs suggestive to virilization (deep voice, axillary hair, pubic sexual hair and external genitalia in Tanner's stage III). Hormonal studies were carried out that corroborated hyperandrogenism by autonomic endogenous secretion, with suppressed gonadotropin levels, elevated testosterone and dehydroepiandrosterone levels. Imaging studies were also performed that showed accelerated bone age and the existence of a tumor. A left adrenalectomy was performed and stage 2 left virilizing adrenocrotical carcinoma was confirmed by pathological anatomy studies. He began chemotherapy treatment for this diagnosis and is currently being followed up. Conclusions: Adrenocortical carcinomas in children are mostly functioning and are one of the causes of peripheral early puberty. These are uncommon and aggressive, so genetic studies in families with hereditary syndromes would contribute to their early diagnosis for adequate treatment and better prognosis(AU)

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.

Escore clínico-patológico para predizer o risco de metástases e recorrência local em pacientes com carcinoma cortical adrenal e papel do algoritmo da reticulina na distinção entre adenomas e carcinomas corticais adrenais / Clinicopathological score for predicting the risk of metastases and local recurrence in patients with adrenal cortical carcinoma and role of the reticulin algorithm in distinguishing between adrenal cortical adenomas and carcinomas

INTRODUÇÃO: o padrão-ouro para o diagnóstico histológico dos tumores corticais adrenais (TCAs) e sua diferenciação entre adenomas e carcinomas é o sistema de Weiss, cuja aplicação é limitada pela baixa reprodutibilidade de alguns dos critérios que o compõe. Recentemente foi proposto e validado um algoritmo diagnóstico para os TCAs baseado na integridade do arcabouço de reticulina e da membrana basal. Os carcinomas adrenais são tumores raros e apresentam prognóstico reservado, mesmo nos pacientes com doença aparentemente localizada. Além do estadiamento e da extensão da ressecção cirúrgica, outros dados foram reportados na literatura como tendo importância prognóstica, tais como idade ao diagnóstico, padrão funcional, tamanho tumoral, extensão local do tumor primário e alguns achados histológicos e imuno-histoquímicos, com destaque à taxa mitótica e ao índice de Ki-67. O sistema de Weiss, embora permita o diagnóstico diferencial entre adenomas e carcinomas, não foi testado completamente como uma ferramenta para distinguir os carcinomas com boa evolução clínica daqueles com desfecho desfavorável. OBJETIVOS: o presente estudo teve como objetivo primário construir um nomograma para estimar o risco de metástases e recorrência local em portadores de carcinoma adrenal, a partir de dados clínico-patológicos. O objetivo secundário foi avaliar o desempenho do algoritmo da reticulina no diagnóstico diferencial entre adenomas e carcinomas do córtex adrenal. MÉTODOS: para a construção do nomograma, foram analisados dados clínico-patológicos de 129 portadores de carcinomas adrenais atendidos no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo entre 1976 e 2010. A avaliação do desempenho do algoritmo da reticulina para o diagnóstico histológico dos TCAs foi feita a partir do exame de 89 lâminas (45 adenomas e 44 carcinomas adrenais)...

INTRODUCTION: The gold standard for the histological diagnosis of adrenal cortical tumors (ACTs) and for the differentiation between adenomas and carcinomas is the Weiss system, whose application is limited by poor reproducibility of some of its criteria. Recently, a diagnostic algorithm for ACT diagnosis based on the integrity of the reticulin network and the basal membrane has been proposed and validated. Adrenal carcinomas are rare tumors and have a poor prognosis, even in patients with apparently localized disease. Besides tumor staging and extent of surgical resection, other data have been reported in the literature as having prognostic importance, such as age at diagnosis, the functional pattern, tumor size, local extension of the primary tumor and some histological and immunohistochemical findings, such as the mitotic rate and the Ki-67 index. The Weiss system, while allowing the differential diagnosis between adrenal cortical adenomas and carcinomas, has not been fully tested as a tool for distinguishing carcinomas with favorable clinical outcome from those with unfavorable outcome. OBJECTIVES: The primary objective of this study was to construct a nomogram for estimating the risk of metastasis and local recurrence in patients with adrenal cortical carcinoma, based on clinical and pathological data. The secondary objective was to evaluate the performance of the reticulina algorithm in the differential diagnosis between adenomas and carcinomas of the adrenal cortex. METHODS: For the construction of the nomogram, clinical and pathological data from 129 patients with adrenal cortical carcinomas treated at the Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo between 1976 and 2010 were analyzed. The evaluation of the performance of the reticulin algorithm for the histological diagnosis of ACTs was made from the examination of 89 slides (45 adenomas and 44 adrenal carcinomas)...

Adrenal cortical carcinoma in Thailand: A review on the previous reported case.

Background: Adrenal cortical carcinoma (ACC) is an uncommon cancer. Materials and Methods: The author performed a literature review on the reports of ACC in Thailand in order to summarize the characteristics of this rare cancer among Thai patients. Results: According to this study, there have been at least seven reports in the literature of nine individual cases of ACC, of which none was lethal directly to ACC. Conclusions: Here, the ACC is sporadically reported in Thailand. The diagnosis of ACC is usually by detection adrenal mass using imaging technique and the confirmation for the nature of cancer is usually by histopathology. Nevertheless, among the Thai population, the prognostic outcome is usually good.

Tumores adrenocorticais na criança: da abordagem clínica à avaliação molecular / Childhood adrenocortical tumors: from a clinical to a molecular approach

Tumores do córtex adrenal (TCA) são mais frequentes em crianças, mas podem ocorrer em qualquer faixa etária. São classificados como funcionantes, não funcionantes (predominam no adulto), e mistos. O diagnóstico é baseado na avaliação clínica, hormonal e exames de imagem. Em crianças, o método de escolha para diferenciar entre benigno ou maligno é a classificação baseada no estadiamento do tumor. Alguns marcadores moleculares merecem destaque: além de mutações inativadoras no gene supressor tumoral TP53, há evidências de envolvimento do IGF2 em 90 por cento de TAC malignos, e mutações no éxon 3 do gene CTNNB1 foram encontradas em 6 por cento dos TAC pediátricos. Além disso, microRNAs podem atuar como reguladores negativos da expressão gênica e participar da tumorigênese adrenocortical. Métodos para análise da expressão gênica permitem identificar TCA com prognóstico bom ou ruim, e espera-se que esses estudos possam facilitar o desenvolvimento de drogas para tratar pacientes de acordo com as vias de sinalização específicas que estiverem alteradas.

Adrenocortical tumors (ACT) are more frequent during childhood, but they can appear at any age. ACTs can be classified in functioning, nonfunctioning (mainly observed in adults) and mixed. The diagnosis is based on clinical, biochemical findings and imaging. In children, in order to classify ACT as benign or malignant, tumor staging classification is recommended. Regarding molecular markers some studies should be taken into account: besides TP53 mutations, previous studies have also provided evidences of IGF2 involvement in 90 percent of the malignant ACT. Mutations altering exon 3 of CTNNB1 gene have been found in 6 percent of childhood ACTs. In addition, microRNAs can act as negative regulators of gene expression by targeting mRNA controlling cell growth, differentiation and apoptosis and have been implicated in adrenal tumorigenesis. High-throughput methods to analyze genome-wide expression have been developed over the last decade and identified a subset of tumors with good or poor prognosis. In the future, these studies can provide the basis of specific drug development, which can treat patients according to specific altered signaling pathway.

Giant adrenal myelolipoma associated with 21-hydroxylase deficiency: unusual association mimicking an androgen-secreting adrenocortical carcinoma / Mielolipoma adrenal gigante associado à deficiência da 21-hidroxilase: associação não usual simulando um carcinoma adrenocortical secretor de androgênios

The objective of this study was to describe a case of giant myelolipoma associated with undiagnosed congenital adrenal hyperplasia (CAH) due to 21-hydroxylase (21OH) deficiency. Five seven year-old male patient referred with abdominal ultrasound revealing a left adrenal mass. Biochemical investigation revealed hyperandrogenism and imaging exams characterized a large heterogeneous left adrenal mass with interweaving free fat tissue, compatible with the diagnosis of myelolipoma, and a 1.5 cm nodule in the right adrenal gland. Biochemical correlation has brought concerns about differential diagnosis with adrenocortical carcinoma, and surgical excision of the left adrenal mass was indicated. Anatomopathologic findings revealed a myelolipoma and multinodular hyperplasic adrenocortex. Further investigation resulted in the diagnosis of CAH due to 21OH deficiency. Concluded that CAH has been shown to be associated with adrenocortical tumors. Although rare, myelolipoma associated with CAH should be included in the differential diagnosis of adrenal gland masses. Moreover, CAH should always be ruled out in incidentally detected adrenal masses to avoid unnecessary surgical procedures.

O objetivo deste trabalho foi descrever um caso de mielolipoma gigante associado à hiperplasia adrenal congênita (HAC) por deficiência da 21-hidroxilase (21OH). Paciente do sexo masculino, 57 anos de idade, encaminhado por achado ultrassonográfico de massa adrenal esquerda. Investigação bioquímica revelou hiperandrogenismo e exames de imagem revelaram grande lesão sólida em adrenal esquerda de aspecto heterogêneo, entremeada de tecido gorduroso, compatível com diagnóstico de mielolipoma, e um nódulo de 1,5 cm na adrenal direita. Os achados bioquímicos sugeriam o diagnóstico de carcinoma adrenocortical, indicando cirurgia para retirada da massa adrenal esquerda. O anatomopatológico confirmou mielolipoma e hiperplasia multinodular do córtex adrenal. A investigação subsequente diagnosticou HAC por deficiência da 21OH. Concluiu-se que a HAC tem sido descrita em associação com tumores adrenocorticais. Apesar de raro, o mielolipoma associado à HAC deve ser incluído nas possibilidades diagnósticas de massa adrenal. Adicionalmente, a HAC deve ser sempre afastada nos casos de massa adrenal de achado incidental, evitando cirurgias desnecessárias.

Carcinoma suprarrenal: [revisión] / Adrenocortical carcinoma: [review]

El carcinoma suprarrenal es una patología poco frecuente, con una incidencia anual estimada en 1-2 casos por millón de habitantes. Si bien se presenta a cualquier edad tiene una distribución bimodal, presentándose con mayor frecuencia antes de los 5 años y durante la cuarta y quinta décadas de vida. Tiene una leve tendencia a ser más frecuente en mujeres. Cerca del 60 por ciento de los casos se presentan como un tumor funcional asociado a un síndrome clínico reconocible. De estos los más frecuentes son el síndrome de Cushing y la virilización. El hiperaldosteronismo y la feminización son infrecuentes. Los pacientes con tumores no funcionales presentan un cuadro clínico producto del efecto de masa tumoral. Los estudios hormonales e imagenológicos son de vital importancia al momento de evaluar un paciente con sospecha de carcinoma suprarrenal. La adrenalectomía quirúrgica permanece como la única alternativa potencialmente curativa mientras que el Mitotano es el fármaco de elección en pacientes con tumores irresecables o enfermedad metastásica. El pronóstico del Carcinoma suprarrenal es malo, con una tasa de recurrencia de hasta el 80 por ciento y un promedio de sobrevida de 2 años.

Adrenocortical Carcinoma is an uncommon disease with an estimated incidence of 1-2 cases permillion people. Although it can occur at any age, it has a bimodal distribution, appearing more frequentlyin the first five years and during the fourth and fifth decades of life. It has a slight tendency to be more frequent in women. About 60 percent of cases are presented as a functional tumor associated with a recognizable clinical syndrome. Of these the most common are Cushing syndrome and virilization. The feminization and hyperaldosteronism are rare. Patients with a non-functional tumor present symptoms due to the tomoural mass effect. Hormonal and imaging studies are essential when assessing a patient with suspected adrenal carcinoma. Adrenalectomy remains as the only potentially curative treatment, whereas Mitotane is the drug of choice in patients with unresectable tumors or metastatic disease. The prognosis of adrenal carcinoma is poor, its rate of recurrence reaches 80 percent with an average survival of 2 years.

Adrenocortical carcinomas: a 12-year clinicopathologic study of 15 cases.

Adrenal cortical carcinomas are rare neoplasms and the definitive diagnostic criteria are distant metastasis and / or local invasion. Due to advances in imaging techniques, adrenal cortical neoplasms are discovered earlier and are smaller, increasing the need for more accurate diagnosis and pathologic indicators of prognosis. A twelve year retrospective clinicopathologic analysis of 15 histopathologically proven cases of adrenocortical carcinomas was done. Clinical details including radiologic findings, endocrine manifestations and gross finding were analysed. Hematoxylin and eosin stained slides were reviewed. Emphasis was on application of Weiss criteria. All fifteen tumors fulfilled Weiss criteria of malignancy, ie. all 15 possessed 3 or more of these criteria of malignancy. Functional tumors showed a greater representation of mixed cell type. It was concluded that Weiss criteria is easy to apply and that a combined evaluation of clinical features, size, weight and microscopic appearance seems necessary for the diagnosis of adrenocortical carcinomas.

A Case of Non-Functioning Huge Adrenocortical Carcinoma Extending Into Inferior Vena Cava and Right Atrium

Primary adrenocortical carcinoma (ACC) is a rare tumor and its usual sites of metastasis are the lung (71%), lymph node (68%), liver (42%), and bone (26%). However, intracaval invasion extending into the right atrium is very rare and spontaneous regression of tumor burden in adrenal carcinoma is also rare. We report a case of ACC with direct invasion of the inferior vena cava and right atrium. A 34-yr-old male patient presented with progressive dyspnea, weight loss, and poor oral intake over 3 months. Non-functioning ACC with direct invasion of the inferior vena cava and right atrium was confirmed by imaging, pathologic, and hormonal study. Chemo-radiotherapy was attempted. However, tumor burden was not changed, but rather toxic hepatitis and thrombocytopenia were developed. His subjective symptoms and general conditions were improved after 1 month of conservative management and the patient was discharged. During clinical follow-up, this tumor showed spontaneous regression.

Carcinoma de supra-renal com trombo em veia cava inferior: relato de caso / Supra-renal carcinoma with inferior vena cava trhombus: case report

Objetivo: descrever um caso de carcinom adrenal com trombo venoso. Método: paciente com 35 anos de idade portadora de carcinoma adrenal e trombo venoso não diagnosticado na avaliação pré-operatória com tomografia computadorizada, submetida à adrenalectomia com ressecção do trombo. Conclusão: a paciente apresentou boa evolução no pós-operatório, com quedas dos níveis pressóricos. A ressonância magnética deve portanto fazer parte obrigatória, na avaliação dos pacientes com tumores adrenais de tamanho significativo

Adrenocortical carcinoma: diagnosis by fine needle aspiration cytology.

Two cases of adrenal cortical carcinoma are described, wherein the diagnosis was established by fine needle aspiration cytology (FNAC). FNAC is increasingly being accepted as a means of diagnosis of adrenal cortical carcinomas. In yester years the diagnosis was established largely on post-operative histopathology. Adrenal cortical carcinomas are rare and account for less than 0.05% of all malignant neoplasms. Adrenal gland has become frequent target of needle biopsies with the availability of sensitive imaging techniques and better localization. Currently, needle biopsy is the only non-surgical means of obtaining a diagnosis in patient with adrenal mass. We present two cases of adrenal cortical carcinoma diagnosed on fine needle aspiration cytology (FNAC).

Avances en el diagnóstico y tratamiento de los tumores de la glándula suprarrenal / Diagnosis and treatment of adrenal gland tumors

Antecedentes: La cirugía de la glándula suprarrenal ha sido tradicionalmente acompañada de una elevada mortalidad, pero nuevas técnicas permiten realizarla en forma más segura. Objetivos: Analizar nuestra experiencia en el diagnóstico y tratamiento de los tumores suprarrenales. Lugar de Aplicación: Servicio de Cirugía General, Hospital Privado de Comunidad. Diseño: Estudio observacional retrospectivo. Población y Métodos: Hemos revisado en forma retrospectiva una serie de 53 pacientes (56 procedimientos quirúrgicos) sometidos a resecciones de tumores de glándula seprarrenal. En total se realizaron 57 seprarrenalectomías (5 bilaterales). Resultados y conclusiones: El tumor más frecuente fue el feocromocitoma. En 13 pacientes hallamos adenomas corticoles, de los cuales 8 fueron Sindromes de Conn. La edad promedio fue de 42 años y 31 fueron mujeres. El 60 por ciento de los enfermos pudieron ser seguidos entre 2 y 180 meses. La suma de RMN, TC y ecografía permitió localizar las lesiones en el 98 por ciento de los casos. La mortalidad de esta serie fue 0 por ciento y la morbilidad fue 9.4 por ciento. En 33 oportunidades se utilizó la vía anterior, en 16 cirugía mininvasiva (laparoscópica) y en 8 la vía lumbar posterior. La vía laparoscópica resulta más costo eficiente en días de internación comparada con las otras (P < 0.01)

Tumores da córtex da supra-renal: o uso do p53 na diferenciaçäo entre carcinomas e adenomas / Adrenocortical tumors: use of p53 in differentiation between carcinomas and adenomas

A marcação imunohistoquímica da proteina p53 foi estudada em tumores da adrenal conservados em formol ou em blocos de parafina, pelo método da avidina-biotina-peroxidase com recuperação antigênica. Foram estudados 24 carcinomas e 26 adenomas com o objetivo de verificar se o marcador mostrava capacidade de distinção entre eles. Em 62,5 por cento dos carcinomas a marcação foi positiva enquanto que nos adenomas foi de 15,4 por cento, diferença essa estatisticamente significante (p=0,0003). A sensibilidade, especificidade e valor preditivo positivo desse marcador para o diagnóstico do câncer foram, respectivamente: 83,3 por cento, 71,8 por cento e 62,5 por cento. Não houve relação entre o índice de marcação e outros parâmetros clínicos, como peso do tumor, estádio local, recidiva e metástases. Os autores concluem que o marcador é útil no diagnóstico diferencial de massas da adrenal, mas não tem relação com a agressividade biológica da neoplasia maligna.

Papel do MIB-1 no prognóstico dos carcinomas da córtex de supra-renal / Role of MIB-1 on prognosis of adrenocortical carcinomas

Estudou-se a marcação imunohistoquímica para o MIB-1 (Ki-67) em 24 carcinomas da córtex adrenal. A idade dos pacientes variou de 1 a 53 anos, com mediana de 7,5. Excetuando 1 paciente com doença avançada, todos foram submetidos à adrenalectomia. O tempo de seguimento variou de 1 a 13 anos. No momento do diagnóstico 7/24 pacientes já apresentavam metástases e no decorrer do seguimento 3 pacientes adicionais as desenvolveram. A média do peso tumoral dos sobreviventes livres da doença foi de 400 +/- 687 g enquanto que nos falecidos ou com neoplasia após a cirurgia foi de 508 +/- 335 g, diferença essa sem significado estatístico. Tumores maiores que 200 g mostraram pior prognóstico do que aqueles com peso menor (p = 0,01). Os valores médios do índice mitótico (IM) para tumores em estádio I, II, III e IV foram respectivamente: 17,4 +/- 10,0, 12,4 +/- 11,2, 18,3 +/- 14,0 e 28,3+/- 21,0. A comparação das médias revelou p>0,2. Os valores médios do índice de proliferação(IP) determinado pelo MIB-1 nos estádios I, II, III e IV foram respectivamente: 201,4 +/- 131,0, 179,6 98,7, 129,1 +/- 170,2 e 124,0 +/- 99,4. A comparação estatística dessas médias mostrou p>0,3. Valores do IP maiores que 20 estão associados a pior prognóstico. O IP não mostrou relação com o prognóstico.

Carcinoma adrenocortical virilizante: relato de caso / Virilizing adrenocortical carcinoma: report of a case

A incidência do Carcinoma adrenocortical é aproximadamente de 1 : 1.7000.000. Isso perfaz somente 0.025 dos tumores malignos. A manifestação clínica depende dos hormônios predominantemente produzidos, estando a sindrome de cushing presente em 30-40 (por cento) dos pacientes com carcinoma adrenocortical e a virilização ocorrendo em 20-30 (por cento) dos adultos. Relatamos o caso de uma paciente que se apresentou com quadro clínico de virilização às custas de carcinoma adrenocortical produtor de andrógenos (principlamente testoterona), associado à produção de cortisol, não tendo sido exuberante o quadro clínico de Síndrome de Cushing, devido ao curto tempo de duração da doença

Carcinoma corticosuprarrenal en niños. Experiencia de 16 años en el Hospital Infantil de México Federico Gómez / Corticosuprarenal carcinoma in childre. Sixteen years of experience in the \"Hospital Infantil de Mexico Federico Gomez\"

Introducción. Los tumores de la corteza suprarrenal son raros, se estima una incidencia de 0.5-2 casos/millón de habitantes /año, con muy escasos reportes pediátricos en la literatura. Material y métodos. Se presenta una revisión de los casos de carcinoma corticosuprarrenal vistos en un período de 16 años en el Hospital Infantil de México Federico Gómez. Resultados. En total fueron 8 pacientes, con edades de 5 meses a 7 años 11 meses; predominó el sexo femenino. Siete con manifestaciones clínicas de exceso hormonal, uno se consideró hormonalmente no funcionante. El síndrome más común fue virilización, con pubarca como dato más frecuente seguido por clitoromegalia. Un paciente presentó síndrome de Cushing con sobrepeso como dato inicial, acompañado de detención del crecimiento. Todos los casos mostraron alteración en la determinación de metabolitos urinarios y los estudios de gabinete revelaron la presencia de masa tumoral. Se realizó resección quirúrgica total a todos los pacientes. Hubo 2 pacientes con metástasis desde el momento del diagnóstico y uno más con metástasis tardías; los 3 fallecieron a pesar de quimioterapia múltiple. Conclusión. El diagnóstico precoz, resección quirúrgica total y peso bajo del tumor favorecieron el pronóstico

A clinicopathologic profile of adrenocortical tumors.

OBJECTIVE: To study the clinical, biochemical, hormonal, radiological and histopathological profile of adrenocortical tumors in children; to assess the clinicopathological correlations and note the future outcome. DESIGN: Retrospective and prospective study. SETTING: Hospital based; Endocrine Service of our institution and other institution based services. SUBJECTS: 14 children (Females = 11, Males = 3) with adrenocortical tumor, aged 8 months to 13 years (mean age 5.1 +/- 3.42 years), seen over a period of 9 years. RESULTS: Females predominated (F:M = 3.7:1). Majority (64%) had a mixed picture with cushingoid features and virilization, whereas 36% presented only for virilization. Elevated serum cortisol levels with loss of diurnal variation was noted only in those with mixed clinical presentation. Adrenal androgen elevation was noted in majority of cases as virilization was common to all. CT confirmed the diagnosis of tumor, 7 on either side. Thirteen cases were operated. Histopathologic diagnosis was carcinoma in 7 and adenoma in 6 cases. Three of the seven with carcinoma died within 3 months to 2 years but two of these with small tumours (weight 60-65 g and diameter < 6 cm) were well at 2 and 5 years, while as one of the six with a large adenoma had recurrence and metastasis after three years. CONCLUSION: Female preponderance was marked (4 times), 43% of tumors had occurred by 3 years of age and 64% by 6 years. Neither the hormonal parameters nor the histopathology correlated well with the biological behavior and outcome. Prolonged and vigilant follow up is essential.