Neoplasia oncocítica adrenocortical de potencial maligno incierto: reporte de un caso / Adrenocortical oncocytic neoplasm with uncertain malignant potential: a case report

Resumen Objetivo Describir el caso clínico de una paciente con neoplasia oncocitica adrenocortical, tratado quirúrgicamente en una clínica de Lima, Perú. Caso clínico Paciente mujer de 26 años ingresa a emergencia por dolor abdominal inespecífico. Se evidencia tumoración de 15x14x12 cm dependiente de glándula suprarrenal izquierda por lo que se decide tratamiento quirúrgico. Al análisis patológico se evidencia neoplasia oncocítica de potencial maligno incierto. Discusión Las neoplasias oncocíticas adrenocorticales son entidades poco frecuentes, con escasos reportes de casos de esta enfermedad. Para clasificarlas, se usa la escala de Weiss modificada. Obtenemos una neoplasia oncocítica de potencial maligno incierto, cuyo tratamiento incluye la cirugía de resección de tumor y observación. Conclusión Considerar a las neoplasias oncocíticas dentro del diagnóstico diferencial de incidentalomas adrenales.

Objective To describe a case report of a oncocytic adrenocortical neoplasm, treated surgically in a clinic in Lima, Peru. Case report A 26-year-old woman is admitted to the emergency due to nonspecific abdominal pain. A tumor measuring 15x14x12 cm dependent on left adrenal gland is evidenced, so surgical treatment is decided. Pathological analysis evidences oncocytic neoplasia of uncertain malignant potential. Discussion Oncocytic adrenocortical neoplasms are rare entities, with few case reports of this disease. To classify them, the modified Weiss scale is used. We obtain an oncocytic neoplasm of uncertain malignant potential, whose treatment includes surgery for tumor resection and observation. Conclusion Consider oncocytic neoplasms within the differential diagnosis of adrenal incidentalomas.

Predictors of complication after adrenalectomy

ABSTRACT Purpose: To investigate risk factors for complications in patients undergoing adrenalectomy. Materials and Methods: A retrospective search of our institutional database was performed of patients who underwent adrenalectomy, between 2014 and 2018. Clinical parameters and adrenal disorder characteristics were assessed and correlated to intra and post-operative course. Complications were analyzed within 30-days after surgery. A logistic regression was performed in order to identify independent predictors of morbidity in patients after adrenalectomy. Results: The files of 154 patients were reviewed. Median age and Body Mass Index (BMI) were 52-years and 27.8kg/m2, respectively. Mean tumor size was 4.9±4cm. Median surgery duration and estimated blood loss were 140min and 50mL, respectively. There were six conversions to open surgery. Minor and major post-operative complications occurred in 17.5% and 8.4% of the patients. Intra-operative complications occurred in 26.6% of the patients. Four patients died. Mean hospitalization duration was 4-days (Interquartile Range: 3-8). Patients age (p=0.004), comorbidities (p=0.003) and pathological diagnosis (p=0.003) were independent predictors of post-operative complications. Tumor size (p<0.001) and BMI (p=0.009) were independent predictors of intra-operative complications. Pathological diagnosis (p<0.001) and Charlson score (p=0.013) were independent predictors of death. Conclusion: Diligent care is needed with older patients, with multiple comorbidities and harboring unfavorable adrenal disorders (adrenocortical carcinoma and pheocromocytoma), who have greater risk of post-operative complications. Patients with elevated BMI and larger tumors have higher risk of intra, but not of post-operative complications.

Carcinoma adrenocortical oncocítico virilizante / Adrenal oncocytic virilizing carcinoma

Las neoplasias oncocíticas se encuentran raramente en la glándula suprarrenal. Suelen ser benignos y no funcionales. Existen informes extremadamente limitados de neoplasias oncocíticas suprarrenales. Alrededor del 20% de las neoplasias oncocíticas adrenocorticales muestran componentes malignos y el 10-20% produce hormonas que pueden causar desbalances hormonales. Se presenta un carcinoma adrenocortical oncocítico virilizante en mujer de 35 años de edad quien acudió a consulta por presentar hipomenorrea secundaria de seis meses de duración acompañada de hirsutismo y agravamiento del acné que afectaba principalmente la cara. El examen de laboratorio revela niveles elevados de testosterona libre. La tomografía computada mostró tumor multinodular en la glándula suprarrenal derecha con necrosis central y sin evidencia de linfadenopatía o invasión de estructuras que la rodeaban. Se programó la adrenalectomía derecha lográndose resección completa del tumor. Después de la cirugía, se normalizaron los ciclos menstruales, el hirsutismo, el acné y el resto de las pruebas. El examen histopatológico del tumor fue compatible con carcinoma adrenocortical oncocítico(AU)

Oncocytic neoplasms are found more rarely in the adrenal gland. They are usually benign and nonfunctional. There are limited reports of adrenal oncocytic neoplasms. About 20% of adrenocortical oncocytic neoplasms show malignant components and 10-20% produce hormones that can cause hormonal imbalances. We present a virilizing oncocytic adrenocortical carcinoma in a 35-year-old woman who consulted for hypomenorrhea accompanied by hirsutism and face acne worsening. Laboratory test revealed high levels of free testosterone. Computed tomography showed multinodular tumor of the right adrenal gland with central necrosis and without evidence of lymphadenopathy or invasion of surrounding structures. A right adrenalectomy was performed. After surgery, menstrual cycle, hirsutism and acne, as well as hormonal tests were normal. Histopathological examination of tumor showed an oncocytic adrenocortical carcinoma(AU)

Adenoma corticoadrenal, presentándose como un incidentaloma: presentación de un caso y revisión de la literatura / Adrenocortical adenoma, presenting as an incidentaloma: case report and literature review

Resumen Objetivo: Presentamos un caso clínico con diagnóstico de incidentaloma adrenal no funcionante asintomático y analizamos las implicaciones clínicas y el abordaje realizado. Caso clínico: Se reporta el caso de un masculino de 53 años, asintomático, con hallazgo ecográfico accidental de imagen hipoecoica de contornos bien definidos en la glándula suprarrenal derecha que presentó incremento en su tamaño. Su estudio hormonal fue negativo para hiperfunción adrenal. Resultados: Se realizó suprarrenalectomía laparoscópica con técnica de 4 trocares con resección completa de la lesión. El paciente presentó buena evolución posquirúrgica. El estudio anatomopatológico concluyó el diagnóstico de adenoma corticoadrenal no funcionante. Conclusión: Ante el hallazgo de una masa adrenal mayor de 1 cm corresponde realizar una identificación hormonal y una evaluación del riesgo de malignidad en los pacientes, los cuales, junto con parámetros imagenológicos y los síntomas presentados, permitirán definir las complicaciones en el manejo y el pronóstico del paciente. El diagnóstico diferencial de los adenomas adrenales está basado en la identificación hormonal, el conocimiento radiológico y el grado de compromiso de la lesión. El abordaje laparoscópico es de elección en las lesiones pequeñas y sin sospecha de malignidad.

Objective: We present a clinical case with diagnosis of an asymptomatic nonfunctional adrenal incidentaloma, in which we discuss the clinical implications and the approach. Clinical case: Male patient, 53 years old with an accidental sonographic finding, characterized by a hypoechoic image of well-defined contours in the right adrenal gland of less than 2 cm. The hormonal test showed no adrenal hyperfunctioning. Laparoscopic adrenalectomy technique is performed with 4 trocars with complete excision of the lesion. The patient presented good postoperative evolution. Results: The pathology study showed a well-defined and benign tumor lesion of the adrenal gland, being similar to the fascicular zone and cortical hyperplasia next to it. The diagnosis is a non-functioning adenoma of the adrenal gland derived from the fascicular zone. Conclusion: Given the finding of an adrenal mass greater than 1 cm mass corresponds perform a hormonal identification and risk assessment of malignancy in patients, which with imaging parameters (echogenicity, bilateralism and the adjacent commitment) and symptoms presented allow to identify the complications in the management and prognosis of the patient. The differential diagnosis of adrenal adenomas is based on the hormonal evaluation, radiological knowledge and the commitment of the injury.

Adjuvant radiotherapy for the primary treatment of adrenocortical carcinoma: are we offering the best?

ABSTRACT Purpose: To evaluate the role of ARDT after surgical resection of ACC. Materials and Methods: Records of patients from our institutional ACC database were retrospectively assessed. A paired comparison analysis was used to evaluate the oncological outcomes between patients treated with surgery followed by ARDT or surgery only (control). The endpoints were LRFS, RFS, and OS. A systematic review of the literature and meta-analysis was also performed to evaluate local recurrence of ACC when ARDT was used. Results: Ten patients were included in each Group. The median follow-up times were 32 months and 35 months for the ARDT and control Groups, respectively. The results for LRFS (p=0.11), RFS (p=0.92), and OS (p=0.47) were similar among subsets. The mean time to present with local recurrence was significantly longer in the ARDT group compared with the control Group (419±206 days vs. 181±86 days, respectively; p=0.03). ARDT was well tolerated by the patients; there were no reports of late toxicity. The meta-analysis, which included four retrospective series, revealed that ARDT had a protective effect on LRFS (HR=0.4; CI=0.17-0.94). Conclusions: ARDT may reduce the chance and prolong the time to ACC local recurrence. However, there were no benefits for disease recurrence control or overall survival for patients who underwent this complementary therapy.

Long-term Results after CT-Guided Percutaneous Ethanol Ablation for the Treatment of Hyperfunctioning Adrenal Disorders

OBJECTIVES: To evaluate the safety and long-term efficacy of computed tomography-guided percutaneous ethanol ablation for benign primary and secondary hyperfunctioning adrenal disorders. METHOD: We retrospectively evaluated the long-term results of nine patients treated with computed tomography-guided percutaneous ethanol ablation: eight subjects who presented with primary adrenal disorders, such as pheochromocytoma, primary macronodular adrenal hyperplasia and aldosterone-producing adenoma, and one subject with Cushing disease refractory to conventional treatment. Eleven sessions were performed for the nine patients. The patient data were reviewed for the clinical outcome and procedure-related complications over ten years. RESULTS: Patients with aldosterone-producing adenoma had clinical improvement: symptoms recurred in one case 96 months after ethanol ablation, and the other patient was still in remission 110 months later. All patients with pheochromocytoma had clinical improvement but were eventually submitted to surgery for complete remission. No significant clinical improvement was seen in patients with hypercortisolism due to primary macronodular adrenal hyperplasia or Cushing disease. Major complications were seen in five of the eleven procedures and included cardiovascular instability and myocardial infarction. Minor complications attributed to sedation were seen in two patients. CONCLUSION: Computed tomography-guided ethanol ablation does not appear to be suitable for the long-term treatment of hyperfunctioning adrenal disorders and is not without risks.

Therapeutic approach to Class II, Division 1 malocclusion with maxillary functional orthopedics

INTRODUCTION: Interceptive treatment of Class II, Division 1 malocclusion is a challenge orthodontists commonly face due to the different growth patterns they come across and the different treatment strategies they have available.OBJECTIVE: To report five cases of interceptive orthodontics performed with the aid of Klammt's elastic open activator (KEOA) to treat Class II, Division 1 malocclusion.METHODS: Treatment comprehends one or two phases; and the use of functional orthopedic appliances, whenever properly recommended, is able to minimize dentoskeletal discrepancies with consequent improvement in facial esthetics during the first stage of mixed dentition. The triad of diagnosis, correct appliance manufacture and patient's compliance is imperative to allow KEOA to contribute to Class II malocclusion treatment.RESULTS: Cases reported herein showed significant improvement in skeletal, dental and profile aspects, as evinced by cephalometric analysis and clinical photographs taken before, during and after interceptive orthodontics.

INTRODUÇÃO: o tratamento interceptor da má oclusão de Classe II primeira divisão é um desafio comum para os ortodontistas, em função dos diferentes padrões de crescimento que podem ser encontrados e das estratégias de tratamento disponíveis.OBJETIVO:apresentar cinco casos de interceptação da má oclusão de Classe II primeira divisão usando o ativador aberto elástico de Klammt (AAEK).MÉTODOS: o tratamento dessa má oclusão pode ser realizado em uma ou duas fases; e a utilização dos aparelhos ortopédicos funcionais, de acordo com suas indicações, pode minimizar as discrepâncias dentoesqueléticas, melhorando, consequentemente, a estética facial na primeira fase da dentição mista. O diagnóstico, a correta confecção do aparelho e a cooperação do paciente são a tríade indispensável para que o AAEK seja um coadjuvante no tratamento dessa má oclusão.RESULTADOS: os casos clínicos apresentaram melhora significativa nos aspectos esquelético, dentário e de perfil, evidenciada pelas análises cefalométricas e, no aspecto clínico, pelas fotografias pré- e trans-tratamento e após a interceptação.

Adrenocortical oncocytoma

Adrenocortical oncocytoma is an exceedingly rare pathological variant of an adrenal neoplasm. The pathogenesis of oncocytic neoplasms is poorly understood. Females have been reported to be affected 2.5 times more frequently than males, and left-sided lesions are more common than those on right side. This case describes a 20 years old female with right lumbar pain. She found to have a right adrenal gland mass measuring about 6 x 5 cm. A computed tomogram showed hypodense mass lesion 6 x 4.2 cm involving right adrenal gland. Differential diagnosis of non-functional adrenal adenoma was made. A laparoscopic right adrenalectomy was performed using the 3-ports lateral transperitoneal approach. Histopathology showed adrenocortical oncocytoma of adrenal gland

Hiperaldosteronismo primário: relato de dois casos / Primary hyperaldosteronism: report of two cases

The authors present two cases of Primary Hiperaldosteronism, caused by functioning adenoma of the adrenal's cortex. The two females patients presented classic symptoms of the Conn's disease, especially hypocalemia and hypertension. Both were operated and unilateral adrenalectomy was done, with excelent outcome in one and satisfactory in the other one. It is discussed many aspects related to the incidence, the problems with the diagnosis, indication and surgery treatment.

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 tumor in 20 months old female]

Adrecortical virilizing tumors are rare in the pediatric age group. There is 1% incidence for adneral cancer. In comparison with adult patients, there is function adrenalectomy tumor in pediatric group. The patient in this report was a 20 months old female presenting with clinical signs of virilizition that were characterized by increased bone mass, pubic hair growth external genitalia. The laboratory test showed: High level of testosterone [400 ng/dl], andrestandion [3.6ng/ml] and progestron [19.9ng/ml] and very high level of [8000ng/ml] dehydroepiandrosterone. In CT scan there was right adrenal mass with size>5 cm. The diagnosis of right an adrenocortical functional tumor led to the choice of open surgical adrenal with flank apreach between 10 and 11 ribs. Pathologic examination showed carcinoma of the adrenal. Patient discharged 5 days after operation. Surgery was done via lumbar incision and follow up was carreid out for 10 years, and there was not any pathological lesion

Subclinical Cushing's syndrome: [review]

Subclinical Cushing's syndrome (CS) is attracting increasing interest since the serendipitous discovery of an adrenal mass has become a rather frequent event owing to the routine use of sophisticated radiologic techniques. Cortical adenoma is the most frequent type of adrenal incidentaloma accounting for approximately 50 percent of cases in surgical series and even greater shares in medical series. Incidentally discovered adrenal adenomas may secrete cortisol in an autonomous manner that is not fully restrained by pituitary feedback, in 5 to 20 percent of cases depending on study protocols and diagnostic criteria. The criteria for qualifying subclinical cortisol excess are controversial and presently there is no consensus on a gold standard for the diagnosis of this condition. An increased frequency of hypertension, central obesity, impaired glucose tolerance, diabetes and hyperlipemia has been described in patients with subclinical CS; however, there is still no clear demonstration of the long-term complications of this condition whose management remains largely empirical. Either adrenalectomy or careful observation associated with treatment of the metabolic syndrome have been suggested as treatment options.

A síndrome de Cushing subclínica (SCS) tem atraído interesse cada vez maior desde que a descoberta casual de uma massa adrenal se tornou um evento freqüente devido ao emprego rotineiro de técnicas sofisticadas de imagem. O adenoma cortical é o tipo mais freqüente de incidentaloma adrenal, correspondendo a cerca de 50 por cento dos casos em séries cirúrgicas e até mais do que isso em séries médicas. Adenomas adrenais descobertos incidentalmente podem secretar cortisol de maneira autônoma ou não controlada totalmente pelo feedback hipofisário, em 5 a 20 por cento dos casos, dependendo do protocolo de estudo e dos critérios diagnósticos. Os critérios para qualificar um excesso subclínico de cortisol são controversos e atualmente não existe consenso a respeito de "padrão ouro" para o diagnóstico dessa condição. Em pacientes com SCS, tem sido descrita uma freqüência elevada de hipertensão, obesidade central, intolerância à glicose, diabetes e hiperlipemia; entretanto, ainda não existe uma evidente demonstração de complicações a longo prazo dessa condição, cujo manejo permanece amplamente empírico. Tanto a adrenalectomia como a observação cuidadosa, associada com o tratamento da síndrome metabólica, têm sido sugeridos como opções terapêuticas.

Tratamento do tumor do córtex adrenal na infância / Treatment of childhood adrenocortical tumor

O tumor do córtex adrenal (TCA) na infância é raro. Entretanto, no Paraná sua incidência é 15 vezes maior do que a observada em outros países. A mutação germinativa R337H TP53, presente em mais de 95 por cento dos nossos pacientes, provavelmente está relacionada à maior incidência. Cento e vinte e cinco pacientes foram tratados no período de 1966 a 2003. A cirurgia é o único tratamento curativo. Em nossa experiência, tumores no estádio I, ausência de spillage durante a cirurgia e ausência de trombo são parâmetros relacionados à maior sobrevida. Dados preliminares mostram que a associação de etoposídeo, doxorrubicina, cisplatina e mitotano produziu remissão completa do tumor e/ou das metástases em alguns pacientes. Os efeitos colaterais destas drogas são comuns e pode ocorrer insuficiência adrenocortical. As doses de reposição de glicocorticóides e mineralocorticóides devem ser 2 a 3 vezes maiores que as doses fisiológicas.

Conn's Syndrome Associated with Behcet's Disease

A 39-yr-old woman, who had been treated for Behcet's disease for 4 yr, was admitted for further investigation of recently identified hypokalemia and hypertension. Suppressed plasma renin activity with elevated plasma aldosterone concentration and an anomalous postural decrease in plasma aldosterone were observed. An abdominal CT scan revealed a right adrenal mass. The patient was diagnosed with Conn's syndrome. The association of Conn's syndrome with Behcet's disease was thought to be coincidental. To our knowledge, this is the first case of Conn's syndrome associated with Behcet's disease.

Adenocarcinoma virilizante de adrenal / Adrenocortical carcinoma and virilism

Os autores relataram o caso de uma paciente com dois anos e dois meses de idade com adenocarcinoma virilizante de adrenal (AVA), que apresentou trombose em veia cava inferior, invadindo o átrio direito. Neste caso, revisaram a literatura e discutiram a importância do quadro clínico no diagnóstico precoce da patologia. Mostraram, ainda, o tratamento realizado e enfatizaram a raridade da doença.

Aldosteronismo primário: é necessário sempre atençäo em busca do diagnóstico etiológico da hipertensäo arterial sistêmica / Primary aldosteronism: continuous attention is require for ethiologic diagnosis of essential hypertension

Paciente adulta jovem, com hipertensäo arterial sistêmica (HAS) crônica e miomatose uterina, internada no Hospital das Clínicas/UFMG para ser submetida à histerectomia. Evidenciou-se hipopotassemia e pressäo arterial elevada no pré-operatório, ambas refratárias à terapêutica medicamentosa. Surgiu diarréia líquida sem repercussöes sistêmicas ou evidências de etiologia infecciosa, que persistiu durante quase toda a internaçäo. A ultrassonografia revelou massa em topografia de glândula supra-renal esquerda. Foi realizada adrenalectomia através de videolaparoscopia, evidenciando-se melhora parcial dos níveis pressóricos no período pós-operatório. O potássio sérico normalizou-se e a diarréia desapareceu progressivamente. O exame anátomo-patológico da glândula revelou adenoma de supra-renal. O presente caso evidencia a necessidade de se manter postura crítica constante sobre a busca de explicaçäo para a hipertensäo arterial sistêmica.

Síndrome de Cushing por secreción ectópica de ACTH: análisis del mecanismo fisiopatológico de la hipokalemia: comunicación de dos casos / Cushing syndrome with severe hipokalemia caused by ectopic ACTH secretion: report of two cases

We report a 42 years old male and a 66 years old female with a Cushing syndrome caused by ectopic ACTH secretion secondary to a carcinoid tumor. These patients had both severe hypokalemia, resistant to medical treatment and that subsided with bilateral adrenalectomy and supplementation with dexametasone. Cushing syndrome caused by ectopic ACTH secretion is characterized by a severe and rapidly evolving hypercortisolism. Hypokalemia is present in 90 percent of cases and is probably caused by a defect in 11 ß hydroxysteroid dehydrogenase, that limits the binding of cortisol to aldosterone receptor, metabolizing it to cortisone. Therefore, this alteration will increase the mineralocorticoid action of cortisol

Feocromocitoma y neurofibromatosis de von Recklinghausen: crisis postparto y trombosis de arteria renal / Pheochromocytoma and von Recklinghausen neurofibromatosis presenting with a crisis during the puerperal period: report of 1 case

Pheochromocytoma is observed with higher frequency in patients with von Recklinghausen neurofibromatosis. We report a 36 years old female with von Recklinghausen neurofibromatosis who developed mild hypertension during the fourth month of pregnancy. A cesarean section was performed at 37 weeks of pregnancy. Thereafter, the patient presented severe hypertensive and hypotensive crises, sinus tachycardia and fever. No evidences of an infectious disease were found. Abdominal ultrasound examination showed a right adrenal mass of 7 x 5 cm. High levels of urinary cathecolamines confirmed the diagnosis of pheochromocytoma. After three weeks of prazosin therapy, the patient was operated. During the surgical procedure, an encapsulated pheochromocytoma was found and excised. A right renal atrophy and renal artery thrombosis were also found and a nephrectomy was done. Postoperative evolution was uneventful and the patient remains with normal blood pressure levels six months after the operation

Tumores do córtex da supra-renal na infância: Avaliaçäo clínica, cirúrgica, histopatológica e imunohistoquímica (p53,Ki-67,c-erb-B2 E bcl-2), E suas correlaçöes com prognóstico e a sobrevida de 33 pacientes

Resumo: Os tumores do córtex da supra-renal (TCSR) na infância säo muito raros e de comportamento imprevisível. Os dados de 33 crianças com TCSR, diagnosticadas entre 1980 e 1996 no Hospital das Clínicas da UNiversidade Estadual de Campinas e no Centro Infantil "Dr. Domingos Boldrini" em Campinas, foram analisados com o objetivo de se verificar a relaçäo de fatores clinicos, cirúrgicos, anatomopatológicos e imunohistoquímicos com prognósticos e a sobrevida. A coleta de dados clínicos, laboratoriais, cirúrgicos e evolutivos foi feita por análise retrospectiva por prontuários médicos. A avaliçäo histológica foi feita pelo autor, e cada caso foi classificado de acordo com os critérios de HOUGH et al. (1979), (WEISS)et al. (1984),VAN SLOOTEN et al. (1985), CAGLE et al. (1986), WEISS et al. (1989) modificado e RIBEIRO et al.(1990)., e estadiado pelos critérios de SULLIVAN et al. (1978). Os estudos imunohistoquímicos co p53, Ki67, c-erb B2 e bcl-2 foram realizados de acordo de acordo com a técnica de HSU et al.(1981) modificada.Os testes estatísticos empregados foram o exato de Fischer, o Cochran, o Log, o Wilcoson, além das curvas de sobrevida de Kaplan-Meier aplicados no total dos pacientes e nos pacientes com sobrevida livre de doença (EFS). Os resultados mostraram que 64 por cento dos pacientes eram do sexo feminino, 85 por cento caucasóides, com idade ao diagóstico entre dois e 96 meses (média de 31 e mediana de 27 meses), 85 por cento da zona urbana, 27 por cento com antecedentes pessoal ou familial de contato com agrotóxico e 30 por cento das famílias com história de outros casos de neoplasias. A apresentaçäo clínica predominante foi vilirizaçäo isolada (70 por cento),seguida da associaçäo de virilizaçäo e síndrome de Cushing (21 por cento), sendo apenas dois pacientes assintomáticas e um com síndrome de Cushing isolada. Os exames laboratórios endocrinológicos (17-OH e 17-KS e urinários e o SDHEA sérico)mostraram-se alterados nos 31 pacientes que foran submetidos à coleta.