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Surgical management of the adrenal glands in the multiple endocrine neoplasia type II syndrome

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Abstract

During a 30-year period (1951–1981), seventeen patients underwent bilateral adrenalectomy for established adrenal medullary disease with catecholamine excess. Fourteen patients had the MEN IIa syndrome and 3 had the MEN IIb syndrome. There was no major operative morbidity and no operative mortality.

One patient died 23 months after initial operation because of metastatic pheochromocytoma. The remaining patients were followed for a mean of 129 months and all were personally interviewed. Three patients had 7 uneventful pregnancies. Twelve patients underwent 23 other surgical procedures requiring general anesthesia during the followup period without any morbidity. Two patients have metastatic pheochromocytoma; 3 have clinical metastatic medullary thyroid carcinoma; 5 patients have high calcitonin values and 6 patients are in excellent health at intervals of 15, 27, 58, 110, 134, and 373 months following resection. The need for adrenal replacement therapy has not caused significant problems in any patient.

The results of this study confirm that bilateral total adrenalectomy is a safe modality for the treatment of adrenal medullary disease in this syndrome and that it does not produce significant long-term morbidity.

Résumé

Pendant une période de 30 ans, de 1951 à 1981, 17 sujets ont subi une adrénalectomie bilatérale par une affection de la médullo-surrénale s'accompagnant d'un taux anormalement élevé de catécholamines. Quatorze d'entre eux présentaient un syndrôme MEN IIa et trois un syndrome MEN IIb. La double adrénalectomie entraîna ni complication, ni décès.

Un malade mourut 23 mois après l'intervention de métastases alors qu'il avait été opéré pour phéochromocytome. Les 16 autres sujets furent suivis pendant 129 mois en moyenne. Trois des opérées présentèrent sept grossesses normales. Douze subirent 23 interventions chirurgicales diverses conduites sous anesthésie générale sans être victimes de complications. Deux opérés présentent des phéochromocytomes métastatiques, trois des cancers thyroïdiens médullaires métastatiques, cinq malades accusent un taux élevé de calcitonine et six sont en excellente santé, respectivement 15, 17, 58, 110, 134 et 373 mois après la double adrénalectomie. L'hormonothérapie supplétive n'a entraîné aucun trouble chez les opérés.

Les résultats de cette étude montrent que l'adrénalectomie bilatérale est le traitement efficace de ce type d'affection et qu'elle n'entraîne aucune morbidité à long terme.

Resumen

En pacientes con síndrome de neoplasia endocrina multiple tipos IIa y IIb (MEN IIa y MEN IIb), la patología adrenal medular uniformemente es bilateral y multicéntrica. La hiperplasia adrenal medular (difusa o nodular) es presumiblemente la entidad patológica precursora del feocromocitoma. El espectro anatomo-patológico en estos dos tipos del síndrome varía desde la hiperplasia medular difusa y nodular hasta el feocromocitoma típico, bilateral y multicéntrico. El tratamiento quirúrgico de la enfermedad adrenal medular en este síndrome es todavía motivo de controversia. Nuestro grupo ha recomendado adrenalectomía bilateral total una vez que el diagnóstico haya sido establecido.

En el curso de un período de 30 años (1951–1981), 17 pacientes fueron sometidos a adrenalectomía bilateral por enfermedad adrenal medular con exceso de catecolamina. Catorce pacientes presentaban síndrome MEN IIa y tres MEN IIb. No hubo morbilidad operatoria mayor ni mortalidad operatoria.

Un paciente murió 23 meses después de la operación inicial debido a feocromocitoma metastásico. El resto de los pacientes fueron seguidos por un promedio de 129 meses y todos fueron entrevistados personalmente. Tres pacientes tuvieron embarazos sin complicaciones. Doce pacientes fueron sometidos a otros procedimientos quirúrgicos que requirieron anestesia general en el curso del seguimiento postoperatorio, sin morbilidad alguna. Dos pacientes presentaron feocromocitoma metastásico; tres presentaron carcinoma medular tiroideo metastásico; cinco exhibieron valores altos de calcitonina y seis pacientes se encuentran en excelente estado de salud a intervalos de 15, 27, 58, 110, 134 y 373 meses depués de la resección. La necesidad de terapia de reemplazo suprarrenal no ha causado problema de significación en paciente alguno.

Los resultados de este estudio confirman que la adrenalectomía bilateral total es una modalidad segura y eficaz para el tratamiento de la enfermedad adrenal medular en este síndrome, y que no resulta en morbilidad de significación a largo plazo.

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Authors and Affiliations

  1. Mayo Clinic and Mayo Foundation, Rochester, Minnesota, USA

    Jon A. van Heerden M.B., F.R.C.S.(C), F.A.C.S., Glen W. Sizemore M.D., F.A.C.P., J. Aidan Carney M.D., Clive S. Grant M.D., William H. ReMine M.D., F.A.C.S. & Sheldon G. Sheps M.D.

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  1. Jon A. van Heerden M.B., F.R.C.S.(C), F.A.C.S.
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  2. Glen W. Sizemore M.D., F.A.C.P.
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  3. J. Aidan Carney M.D.
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  4. Clive S. Grant M.D.
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  5. William H. ReMine M.D., F.A.C.S.
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  6. Sheldon G. Sheps M.D.
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van Heerden, J.A., Sizemore, G.W., Carney, J.A. et al. Surgical management of the adrenal glands in the multiple endocrine neoplasia type II syndrome. World J. Surg. 8, 612–619 (1984). https://doi.org/10.1007/BF01654950

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