Abstract
Organic hypoglycemia may be caused by an insulinoma. This is a rare tumor, multiple in about 10% and malignant in about 10% of cases. Multiplicity is particularly likely when insulinoma occurs as part of the MEN 1 syndrome. When a patient exhibits Whipple's triad, synchronous blood sugar and insulin estimations must be made, and repeated during fasting. Diagnosis depends on the finding of inappropriately high insulin levels in the presence of low blood sugars. Many stimulatory tests are available but none are as reliable as prolonged starvation, and probably should be abandoned.
Measurement of proinsulin and C-peptide are of particular value in the diagnosis and in elimination of factitious hypoglycemia.
Preoperative localization is most useful and can be achieved in up to 90% of patients by skillful selective angiography. Percutaneous portal venous sampling has been used and is particularly indicated in patients after a fruitless first exploration.
Tumors of the body and tail are usually treated by distal pancreatectomy, those of the head by enucleation. There is a risk of pancreatic fistula, and duct damage can be unmasked by secretin injection. Occult tumors and hyperplasia may require “blind” progressive distal resection with blood sugar monitoring until the hypoglycemia is controlled, but this is something of a desperation measure. In malignant tumors, hypoglycemia may be controlled by diazoxide or somatostatin. Streptozotocin may control the syndrome and lead to prolongation of life. Like other malignant apudomas, these tumors are usually slow growing and relatively benign.
Résumé
L'hyperglycémie organique est le fait de l'insulinome secrétant. C'est une tumeur rare. Elle est multiple dans 10% des cas et maligne également dans 10% des cas. La multiplicité tumorale est particulièrement fréquente lorsque le malade présente une polyendocrinopathie du type MEN 1.
Dès qu'un malade présente une triade de Whipple il convient de doser simultanément la glycémie et l'insulinémie en période de jeune. Le diagnostic repose sur l'association de l'hypoglycémie et de l'hyperinsulinémie lors d'examens répétés. De multiples tests ont été décrits pour affirmer le diagnostic, aucun ne vaut l'épreuve du jeune prolongé. Le dosage de la pro-insuline et celui du C peptide revêtent une valeur particulière pour éliminer les hypoglycémies fonctionnelles.
La localisation pré-opératoire de la lésion peut être obtenue dans 90% des cas par l'angiographie pancréatique. Elle est des plus utiles. En cas d'échec de cette exploration il convient d'avoir rerecours au dosage de l'insuline dans le sang prélevé aux différents niveaux du système porte.
Les tumeurs du corps et de la queue relèvent de la pancréatectomie distale alors que celles de la tête sont justiciables de la simple énucléation. Cette intervention est susceptible de donner lieu à une fistule pancréatique secondaire à la blessure de la voie excrétrice du pancréas. Il convient de la rechercher systématiquement en cours d'intervention en injectant de las secrétine pour provoquer un flux de suc pancréatique.
Les tumeurs occultes et l'hyperplasie insulaire à l'origine d'une hypoglycémie organique incitent à pratiquer une exérèse pancréatique à la demande, exérèse qui est interrompue dès lors que les dosages per opératoires montrent que l'hypoglycémie est contrôlée encore que ce type d'intervention reste aveugle.
En cas de tumeurs malignes l'hypoglycémie peut être contrôlée par la diazoxide et la somatostatine. La streptozocotine permet également de contrôler le syndrome et de prolonger la vie. En effet, comme tous les autres apudomes malins ces tumeurs sont de croissance lente et donc relativement bénignes.
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Kennedy, T. The management of the hypoglycemic patient. World J. Surg. 6, 718–724 (1982). https://doi.org/10.1007/BF01655364
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DOI: https://doi.org/10.1007/BF01655364