Prevalence of C‐cell hyperplasia and medullary thyroid carcinoma in a consecutive series of pheochromocytoma patients

During a 27‐year period (1956–1982), 64 patients were treated surgically for pheochromocytoma. There was no kinship among any of these subjects. The true prevalence of C‐cell disease of the thyroid in this series was searched for by performing serum calcitonin determinations after provocation with p...

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Published in:World journal of surgery 1984-08, Vol.8 (4), p.493-500
Main Authors: Jansson, Svante, Hansson, Göran, Salander, Håkan, Stenström, Gunnar, Tisell, Lars ‐Erik
Format: Article
Language:English
Subjects:
Adolescent
Adrenal Gland Neoplasms - diagnosis
Adrenal Gland Neoplasms - surgery
Adrenalectomy
Adult
Aged
Calcitonin - blood
Carcinoma - diagnosis
Carcinoma - surgery
Child
Endocrine Neoplasia Type
Female
Follow-Up Studies
Humans
Male
Medullary Thyroid Carcinoma
Middle Aged
Multiple Endocrine Neoplasia - diagnosis
Multiple Endocrine Neoplasia Type
Pentagastrin
Pheochromocytoma - diagnosis
Pheochromocytoma - surgery
Thyroid Carcinoma
Thyroid Neoplasms - diagnosis
Thyroid Neoplasms - surgery
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Summary:During a 27‐year period (1956–1982), 64 patients were treated surgically for pheochromocytoma. There was no kinship among any of these subjects. The true prevalence of C‐cell disease of the thyroid in this series was searched for by performing serum calcitonin determinations after provocation with pentagastrin. Postoperative follow‐up of the pheochromocytoma disease was performed by blood pressure measurement and determination of urinary excretion of catecholamines and metabolites. Ten individuals (15.6%) were found to have C‐cell disease indicating that they had multiple endocrine neoplasia type II (MEN II) syndromes. Seven of these patients had been diagnosed before the time of follow‐up evaluation, 4 because of symptoms and 3 because of the screening procedure. At the systematic follow‐up, 3 “new” cases of C‐cell disease were diagnosed. As a result of the screening procedure, we found 4 “new” MEN II kindreds. After unilateral adrenalectomy, 7 of the 10 MEN II patients have been followed for more than 7.7 years without signs of recurrent adrenal disease. Our results suggest that the prevalence of MEN II syndromes among pheochromocytoma patients is higher than previously reported. Removal of only grossly evident pheochromocytomas and careful follow‐up is a good alternative to routine bilateral adrenalectomy in MEN II patients with pheochromocytoma. Résumé Pendant une période de 27 ans dont le terme s'est situé en 1982, 64 malades ont été opérés pour phéochromocytome. Il n'y avait pas de lien de parenté parmi ces malades. L'existence éventuelle d'une maladie à cellules C de la thyroïde fut recherchée en déterminant le taux de la calcitonine après stimulation par la pentagastrine. Les opérés furent suivis en mesurant la pression artérielle et le taux d'excrétion dans les urines des cathécholamines et des métabolites. Dix opérés (15.6%) ont présenté une affection thyroïdienne à cellules C témoignant qu'ils étaient atteints d'un syndrome type MEN II. Sept parmi ces dix opérés ont vu leur affection reconnue avant le moment du test, quatre parce qu'ils présentaient des manifestations cliniques et trois grâce à l'examen de dépistage. Lors des examens postopératoires trois nouveaux cas furent reconnus. L'examen de dépistage permit de retrouver quatre nouveaux syndromes MEN II apparentés. Sept des dix malades qui présentaient un syndrome MEN II après adrénalectomie unilatérale ont été suivis plus de 7,7 ans sans signes de récidive d'affection surrénale. Ces résult
ISSN:0364-2313
1432-2323
DOI:10.1007/BF01654922