Abstract
Eighty-nine patients underwent adrenal exploration for Cushing's syndrome at our institution during the period 1970–1979. On the basis of clinical and histological findings related to the initial adrenal operation, patients were divided into 6 major subgroups: (a) adrenocortical adenoma; (b) adrenocortical carcinoma; (c) proven ectopic ACTH adrenocortical hyperplasia; (d) Cushing's disease; (e) uncertain diagnostic group (probably ectopic ACTH syndrome—site unknown); and (f) “non-ACTH-dependent” hypercortisolism due to micronodular hyperplasia.
Results of pathologic examination included 25 adrenal adenomas, 15 adrenocortical carcinomas, 40 bilateral adrenocortical hyperplasias, and a single case of micronodular hyperplasia. The postoperative mortality rate was 5.6%, and significant morbidity occurred in 40% of patients. Four of the 5 patients who died within 30 days of operation were in the subgroups with adrenal carcinoma (2 patients) and Cushing's disease (2 patients). Survival in patients with adrenocortical adenomas was significantly decreased at 2 years (relative adrenocortical insufficiency?), but thereafter, it paralleled the expected survival. Patients with the ectopic ACTH syndrome and adrenocortical carcinoma had significantly decreased survival, with only one-third of carcinoma patients alive 2 years postoperatively. The survival of patients with Cushing's disease was lower than expected at both 5 and 10 years.
These results confirm that the diagnosis of patients with endogenous hypercortisolism is usually straightforward and accurate, surgical intervention in this group of patients is still accompanied by significant short-term mortality and morbidity, and long-term survival in all subgroups is decreased.
Résumé
De 1970 à 1979 les auteurs ont procédé à une exploration surrénalienne chez 89 malades qui présentaient un syndrome de Cushing. Sur la base des données cliniques et histologiques ils ont individualisé 6 groupes: (a) adénome cortico-surrénalien; (b) cancer cortico-surrénalien; (c) hyperplasie cortico-surrénalienne ACTH ectopique d'origine prouvée; (d) maladie de Cushing; (e) groupe de diagnostic incertain (probablement syndrome ACTH ectopique de site inconnu); et (f) hypercortisolisme—non ACTH dépendant due à une hyperplasie micronodulaire.
Les résultats de l'examen histologique ont permis de faire état de 25 adénomes surrénaliens, de 15 cancers cortico-surrénaliens, de 40 hyperplasies cortico-surrénaliennes bilatérales et d'un seul cas d'hyperplasie micronodulaire. Le taux de la mortalité postopératoire a été de 5.6% et celui de la morbidité s'est élevé à 40%. Quatre des 5 malades morts au cours de la période postopératoire de 30 jours étaient porteurs d'un cancer surrénalien (2 cas) ou d'une maladie de Cushing (2 cas). La survie à 2 ans chez les sujets qui présentaient un adénome cortico-surrénalien a été diminué de façon significative (insuffisance cortico-surrénale relative?) mais par la suite est restée dans les limites prévues. Les malades qui étaient atteints d'un cancer corticosurrénalien avec un syndrome ACTH ectopique ont eu une faible survie, un tiers d'entre eux seulement étant en vie 2 ans après l'intervention. La survie à 5 ans et à 10 ans chez les sujets atteints de maladie de Cushing a été moins importante que prévue.
Ces résultats confirment que le diagnostic d'hypercortisolisme endogène est habituellement exact, l'intervention chirurgicale est encore grévée d'une mortalité et surtout d'une morbidité importante, et la survie à long terme dans tous les groupes est faible.
Resumen
Ochenta y nueve pacientes fueron sometidos a exploración suprarrenal por síndrome de Cushing en nuestra institutión en el período 1970–1979. Con base en los hallazgos clínicos e histológicos de la operación suprarrenal inicial, los pacientes fueron divididos en 6 subgrupos principales: (a) adenoma adrenocortical; (b) carcinoma adrenocortical; (c) hiperplasia adrenocortical por ACTH ectópica comprobada; (d) enfermedad de Cushing; (e) grupo de diagnóstico incierto (probable síndrome de ACTH ectépica-origen desconocido); y (f) hipercortisolismo “no dependiente de ACTH” debido a hiperplasia micronodular.
Los resultados del examen histopatológico incluyeron 25 adenomas adrenales, 15 carcinomas adrenocorticales, 40 hiperplasias adrenocorticales bilaterales y un caso único de hiperplasia micronodular. La mortalidad postoperatoria fue de 5.6% y se presentó morbilidad de significación en 40% de los pacientes. Cuatro de los 5 pacientes que murieron dentro de los 30 días posteriores a la operación pertenecían a los subgrupos con carcinoma (2 pacientes) y enfermedad de Cushing (2 pacientes). La supervivencia en pacientes con adenomas adrenocorticales apareció considerablemente disminuida a los 2 años (¿insuficiencia adrenocortical relativa?) pero posteriormente resultó paralela a la supervivencia esperada. Los pacientes con el síndrome de ACTH ectópica y con carcinoma adrenocortical exhibieron supervivencia significativamente dismniuída, con sólo un tercio de los pacientes con carcinoma vivos a los 2 anos postoperatorios. La supervivencia de los pacientes con enfermedad de Cushing fue menor que la esperada a los 5 y 10 años.
Estos resultados confirman que: el diagnóstico de pacientes con hipercortisolismo es generalmente claro y preciso; la intervención quirúrgica en este grupo de pacientes todavía se acompaña de significativas morbilidad y mortalidad a corto plazo; la supervivencia en todos los grupos aparece disminuída.
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Watson, R.G.K., van Heerden, J.A., Northcutt, R.C. et al. Results of adrenal surgery for cushing's syndrome: 10 Years' experience. World J. Surg. 10, 531–538 (1986). https://doi.org/10.1007/BF01655518
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DOI: https://doi.org/10.1007/BF01655518