Abstract
Seventy-nine patients underwent subtotal or total adrenalectomy for pituitary-dependent Cushing's syndrome (1953–1980); 76 survived the operation and 75 were followed for 1 to 27 (mean 11) years. Pigmentation, plasma ACTH, and sellar X-rays were assessed at intervals.
Pigmentation developed in 37 (5 before and 32 after operation), most frequently after total adrenalectomy. The sella was definitely enlarged in 6 of 65 patients before the operation and in 14 of 65 after it. Enlargement was more common in pigmented than in non-pigmented patients. The plasma ACTH concentration rose after adrenalectomy. It was significantly higher in pigmented than in non-pigmented patients. It was also higher after total than after subtotal adrenalectomy.
Pituitary tumors were confirmed histologically in 15 patients. The tumors presented at the same time as the Cushing's syndrome in 8 and after adrenalectomy in 7. They were malignant and fatal in 6. Pigmentation, present in 11 of these patients, developed only after adrenalectomy. In 9 other patients with benign lesions and 21 with clinically probable but histologically unconfirmed tumors, 7 were treated by hypophysectomy with or without irradiation (4 cured, 3 improved), and 4 by irradiation alone: external 3, internal 1 (all improved).
Résumé
De 1953 à 1980, 79 malades atteints de maladie de Cushing hypophyso-dépendantes ont subi une surrénalectomie subtotale ou totale. Soixante-seize ont survécu à l'opération et 75 ont été suivis de 1 à 27 ans après l'intervention. On a évalué à intervalles réguliers la pigmentation, le taux plasmatique d'ACTH et l'état radiologique de la selle turcique.
La pigmentation s'est développée chez 37 malades (5 avant et 32 après l'intervention) plus particulièrement après surrénalectomie totale.
L'élargissement de la selle turcique a été constaté chez 65 sujets étudiés, 6 fois avant l'intervention et 14 fois après celle-ci, l'élargissement étant plus fréquent chez les sujets accusant une nette pigmentation.
La concentration plasmatique en ACTH s'est élevée après surrénalectomie, le taux étant plus élevé chez les malades pigmentés et après surrénalectomie totale.
Le diagnostic de tumeur hypophysaire a été confirmé histologiquement chez 15 sujets: 8 fois au moment de la découverte du Cushing et 7 fois après la surrénalectomie. Chez 6 de ces 7 opérés la tumeur de nature maligne entraîna la mort. Onze des 15 sujets développèrent une pigmentation après la surrénalectomie.
Chez 9 autres malades qui présentaient des lésions bénignes et chez 21 qui présentaient une tumeur cliniquement probable mais qui ne fut pas confirmée histologiquement, 7 furent traités par hypophysectomie avec ou sans irradiation (4 guéris, 3 améliorés) et 4 par irradiation externe (3 cas) et interne (1 cas), tous les 4 améliorés.
Resumen
Setenta y nueve pacientes fueron sometidos a adrenalectomía subtotal o total por síndrome de Cushing pituitario-dependiente (1953–1980); 76 sobrevivieron la operación y 75 fueron seguidos por 1 a 27 (media de 11) años. La pigmentación, los niveles plasmáticos de ACTH y radiografías de la silla turca fueron evaluados periódicamente.
La pigmentación se desarrolló en 37 (5 antes y 32 después de la operación), más frecuentemente después de adrenalectomía total.
La silla turca apareció claramente aumentada de tamano en 6 de 65 pacientes antes de la operación y en 14 de 65 después. El aumento de tamano fue más común en los pacientes pigmentados que en los no pigmentados.
La concentración plasmática de ACTH ascendió después de la adrenalectomía. Se presentó en forma significativamente más alta en los pacientes pigmentados que en los no pigmentados. Fue más alta después de adrenalectomía total que de adrenalectomía subtotal.
Tumores pituitarios fueron confirmados histológicamente en 15 pacientes. Se presentaron al tiempo con el síndrome de Cushing en 8 y después de adrenalectomía en 7. Los tumores fueron malignos y fatales en 6. La pigmentación, présente en la totalidad de los 11 pacientes, se desarrolló solo después de la adrenalectomía. En otros 9 pacientes con lesiones benignas y en 21 con tumores clínicamente probables pero histológicamente no confirmados, 7 fueron tratados con hipofisectomía con o sin irradiación (4 curados 3 mejorados) y 4 con irradiación solamente (externa 3, interna 1), todos mejorados.
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Supported by the State Scholarship Foundation of Greece (IKY) and the Yvonne Gregory Fund.
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Manolas, K.J., Farmer, H.M., Wilson, H.K. et al. The pituitary before and after adrenalectomy for Cushing's syndrome. World J. Surg. 8, 374–386 (1984). https://doi.org/10.1007/BF01655082
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DOI: https://doi.org/10.1007/BF01655082