Fiche publication
Date publication
avril 2017
Journal
Revue de pneumologie clinique
Auteurs
Membres identifiés du Cancéropôle Est :
Pr MARTINET Yves
,
Pr VIGNAUD Jean-Michel
Tous les auteurs :
Villemain A, Menard O, Mandry D, Siat J, Vignaud JM, Martinet Y, Tiotiu A
Lien Pubmed
Résumé
Doege-Potter syndrome is a paraneoplastic syndrome characterized by non-islet cell tumor hypoglycemia secondary to a solitary fibrous tumor. These tumors are rare and usually asymptomatic. The syndrome of hypoglycemia is seen in less than 5% of the cases, and the associated tumors are large with a high mitotic rate. The cause of hypoglycemia is related to insulin-like growth factors produced by these tumors called "big" IGF-2. Several biological tests can demonstrate the increase of "big" IGF-2 plasma levels confirming the diagnosis of non-islet cell tumor induced hypoglycemia. The diagnosis is suggested by imaging but diagnostic confirmation is provided by the surgery, which remains the treatment of choice. Resection in many cases is the cure leading to hypoglycemia resolution. Recurrences and malignant transformations are possible which imposes a long-term monitoring. We report a case with relapsed malignant pleural fibrous tumor for which the pathophysiological mechanism of hypoglycemia could be documented as a paraneoplastic syndrome.
Mots clés
Fibrous pleural tumor, Hypoglycémie paranéoplasique, Paraneoplastic hypoglycemia, Tumeur fibreuse pleurale
Référence
Rev Pneumol Clin. 2017 Apr;:
