RESUMO
PURPOSE: The subclassification of adrenal cancers according to the WHO classification in ordinary, myxoid, oncocytic, and sarcomatoid as well as pediatric types is well established, but the criteria for each subtype are not sufficiently determined and the relative frequency of the different types of adrenal cancers has not been studied in large cohorts. Therefore, our large collection of surgically removed adrenal cancers should be reviewed o establish the criteria for the subtypes and to find out the frequency of the various types. METHODS: In our series of 521 adrenal cancers the scoring systems of Weiss et al., Hough et al., van Slooten et al. and the new Helsinki score system were used for the ordinary type of cancer (97% of our series) and the myxoid type (0.8%). For oncocytic carcinomas (2%), the scoring system of Bisceglia et al. was applied. RESULTS: Discrepancies between benign and malignant diagnoses from the first thee classical scoring systems are not rare (22% in our series) and could be resolved by the Helsinki score especially by Ki-67 index (more than 8% unequivocally malignant). Since all our cancer cases are positive in the Helsinki score, this system can replace the three elder systems. For identification of sarcomatoid cancer as rarest type in our series (0.2%), the scoring systems are not practical but additional immunostainings used for soft tissue tumors and in special cases molecular pathology are necessary to differentiate these cancers from adrenal sarcomas. According to the relative frequencies of the different subtypes of adrenal cancers the main type is the far most frequent (97%) followed by the oncocytic type (2%), the myxoid type (0.8%) and the very rare sarcomatoid type (0.2%). CONCLUSIONS: The Helsinki score is the best for differentiating adrenal carcinomas of the main, the oncocytic, and the myxoid type in routine work. Additional scoring systems for these carcinomas are generally not any longer necessary. Signs of proliferation (mitoses and Ki-67 index) and necroses are the most important criteria for diagnosis of malignancy.
Assuntos
Neoplasias das Glândulas Suprarrenais/classificação , Neoplasias das Glândulas Suprarrenais/patologia , Humanos , Gradação de TumoresAssuntos
Humanos , Neoplasias das Glândulas Suprarrenais/cirurgia , Neoplasias das Glândulas Suprarrenais/classificação , Neoplasias das Glândulas Suprarrenais/diagnóstico , Diagnóstico por Imagem/métodos , Feocromocitoma/cirurgia , Cuidados Pré-Operatórios , Prognóstico , Síndrome de Cushing/etiologiaRESUMO
During the past 20 years (1970-90), we had 24 patients with pheochromocytoma: 19 diagnosed clinically and 5 post-mortem. Their ages ranged from 17 to 74 (mean, 43.2 years). Males (n = 14) outnumbered females (n = 10), a 1.41:1 M:F ratio. A majority were symptomatic (95%), with a typical triad of headaches, palpitations and diaphoresis. Most frequent finding was hypertension (95%). It was sustained in 60% and paroxysmal in 35%. In 6 patients (25%) pheochromocytomas were bilateral, all familial. Fifteen were solitary adrenal tumors (63%); 3 (12.5%) were extra-adrenal: 2 intra-abdominal, and 1 cardiac paraganglioma of right atrium. Of 6 familial cases, 4 were associated to Von Hippel-Lindau (VHL) disease, while 2 were multiple endocrine neoplasia (MEN-II) patients. All familial cases were bilateral and in the adrenals. There were no malignancies. Among the 19 clinical cases pre-operative Dx was made by positive urine VMA or catecholamines urine levels: (95 and 100% sensitivity respectively). Preoperative visualization by CT or MRI was done in 62% of the most recent patients. In 5 earlier cases the diagnosis was made post mortem: 3 died of cerebral hemorrhage, 1 with a pons infarct and 1 with congestive heart failure (CHF). There were 2 post-operative deaths and another died 13 years later from thyroid medullary carcinoma. Of the 19 operated, 13 (68%) were cured. Thus pheochromocytomas retain considerable morbidity and some mortality. These rare tumors constitute a clinical diagnostic challenge yet a rewarding therapeutic experience for the alert physician.
Assuntos
Neoplasias das Glândulas Suprarrenais/diagnóstico , Feocromocitoma/diagnóstico , Adolescente , Neoplasias das Glândulas Suprarrenais/classificação , Neoplasias das Glândulas Suprarrenais/cirurgia , Adulto , Idoso , Catecolaminas/urina , Criança , Diagnóstico por Imagem , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Feocromocitoma/classificação , Feocromocitoma/cirurgia , Ácido Vanilmandélico/urinaRESUMO
Durante los últimos 20 años (1970-90), hemos tenido 24 pacientes con feocromocitomas: 19 diagnosticados clínicamente y 5 en autopsia. Sus edades variaron de 17 a 74 años (promedio de 43.2) y el número de varones (14), fueron mayor que el de las hembras (n=10). La mayoría de los sujetos tuvieron síntomas (95%) consistentes en la tríada típica de dolor de cabeza, palpitaciones y sudoración. El hallazgo más frecuente fue la hipertensión (95%), la cual fue sostenida en 60% y paroxística en 35% de los casos. Un 25% (n=6) de casos tenía tumores bilaterales, todos familiares. Quince eran tumores adrenales solitarios (63%) y 3 (12.5%) eran extra-adrenales: 2 en el abdomen (órgano de Zuckerbandl) y uno intratorácico (paraganglioma del atrio derecho del corazón). De los 6 casos familiares, 4 estaban asociados a la enfermedad de Von Hipper-Lindau (VHL), mientras que 2 tenían el síndrome de neoplasia múltiple tipo 2 (MEN-II. Todos los casos familiares eran bilaterales y se hallaban en las adrenales. No hubo tumores malignos. Entre los 19 casos diagnosticados pre-operatoriamente, fueron positivas las pruebas en orina del ácido vanililmandélico (VMA) en 95% y la de catecolaminas totales en 100%. Se logró visualizar los tumores pre-operatoriamente por medio de tomografía computadorizada (CT) o por imagen de resonancia magnética (MRI) en 62% de los pacientes más recientes. En 5 casos más antiguos sólo se hizo el diagnóstico post-mortem: 3 murieron de hemorragias cerebrales, 1 de infarto del pons y otro en fallo cardíaco congestivo. Hubo dos muertes post-operatorias y otra, 13 años post-operación, de carcinoma medular del tiroides. De los 19 operados 13 (68% fueron curados. Se concluye que los feocromocitomas aún retienen un considerable potencial de morbilidad y alguna mortalidad. Estos raros tumores constituyen un reto diagnóstico clínico, pero conllevan una gran satisfacción para el médico que los atiende