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A Rare Diagnosis After the Fall of a 96-Year-Old Woman: Doege-Potter Syndrome

INTRODUCTION: Doege-Potter Syndrome (DPS) is a rare but life-threatening paraneoplastic syndrome, characterized by Non-Islet Cell Tumor-Induced Hypoglycemia (NICTH) secondary to a Solitary Fibrous Tumor (SFT), which secretes an incompletely processed form of Insulin-like Growth Factor 2 (IGF-2). RES...

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Detalles Bibliográficos
Autores principales: Pincelli, Angela Ida, Perotti, Mario, Massariello, Francesca, Gatti, Antonella, Calella, Damiano, Cimino, Vincenzo, Haas, Justin, Bellelli, Giuseppe, Mazzola, Paolo, Annoni, Giorgio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Bentham Science Publishers 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6388424/
https://www.ncbi.nlm.nih.gov/pubmed/30520387
http://dx.doi.org/10.2174/1874609812666181205142247
Descripción
Sumario:INTRODUCTION: Doege-Potter Syndrome (DPS) is a rare but life-threatening paraneoplastic syndrome, characterized by Non-Islet Cell Tumor-Induced Hypoglycemia (NICTH) secondary to a Solitary Fibrous Tumor (SFT), which secretes an incompletely processed form of Insulin-like Growth Factor 2 (IGF-2). RESULTS: A 96-year-old woman was admitted with head trauma due to an accidental fall. During her hospital stay she experienced frequent hypoglycemic episodes. Multiple injections of 33% dextrose and continuous infusion with 10% dextrose were required to maintain normal blood glucose levels. Biochemical analyses revealed hypoinsulinemic hypoglycemia, low C-peptide levels, suppressed in-sulin-like growth factor-1, normal insulin-like growth factor-2, and an elevated IGF-2:IGF-1 ratio, all consistent with IGF-2 secretion by a non-islet cell tumor. A contrast-enhanced chest and abdominal CT scans showed a single large pleural mass in the left lower hemithorax measuring 15x14 cm without secondary lesions. Histological analysis of biopsied specimens suggested a solitary fibrous pleural tumor; accordingly, a diagnosis of Doege-Potter syn-drome was considered. Due to extensive tumor burden and the advanced age of the patient, supportive and non-invasive management was chosen. Dexamethasone therapy was started, and while receiving this therapy she was able to discontinue glucose infusion and successfully maintain euglycemia. DISCUSSION: In the elderly, a sudden and unexplained fall can be the expression of severe hypogly-cemia, usually as a complication of insulin therapy or of oral hypoglycemic agents administered to pa-tients with diabetes. However, in patients without diabetes, other causes should be investigated, and the hypothesis of neoplastic diseases should be considered. CONCLUSION: In this case report we describe an uncommon cause of paraneoplastic hypoglycemia oc-curring in the oldest patient with a non-islet cell tumor reported thus far.