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Doege-Potter Syndrome: A Solitary Fibrous Tumor Causing Non-Islet Cell Tumor Hypoglycemia.
Sheikh, Khalid; Mody, Avni; Haynes, Alex B; Kumar, Pratima.
Afiliación
  • Sheikh K; Department of Internal Medicine, Dell Medical School at the University of Texas at Austin, Austin, TX 78712, USA.
  • Mody A; Department of Internal Medicine, Division of Endocrinology, Dell Medical School at the University of Texas at Austin, Austin, TX 78712, USA.
  • Haynes AB; Department of Surgery and Perioperative Care and Department of Oncology, Dell Medical School at the University of Texas at Austin, Austin, TX 78712, USA.
  • Kumar P; Department of Internal Medicine, Division of Endocrinology, Dell Medical School at the University of Texas at Austin, Austin, TX 78712, USA.
JCEM Case Rep ; 2(2): luae017, 2024 Feb.
Article en En | MEDLINE | ID: mdl-38405103
ABSTRACT
Doege-Potter syndrome occurs when incompletely processed insulin-like growth factor 2 (IGF-2), also known as big IGF-2, is produced by a solitary fibrous tumor (SFT) and results in non-islet cell tumor hypoglycemia (NICTH). We discuss here the case of a 66-year-old male who presented with a 2-week history of increasing confusion and a serum glucose of 34 mg/dL. The patient's symptoms immediately improved with dextrose. The patient did not use insulin, serum sulfonylurea screen was negative, and testing for adrenal insufficiency was unremarkable. Outpatient laboratory evaluation revealed a serum glucose of 48 mg/dL along with low insulin, C-peptide, and proinsulin levels. Further work-up showed an IGF-2 to IGF-1 ratio of 381. A ratio greater than 101 is diagnostic of NICTH. Imaging demonstrated a 21-cm mass in the lower abdomen and pelvis. The patient underwent surgical resection. The hypoglycemia resolved immediately postoperatively. Surgical pathology revealed a malignant SFT. In NICTH, big IGF-2 forms a complex that is biologically active and saturates the insulin and IGF receptors, resulting in refractory hypoglycemia. Although glucocorticoids can mitigate hypoglycemia, complete surgical resection is the only definitive treatment of NICTH. This case highlights the importance of maintaining a broad differential for seemingly simple hypoglycemia.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: JCEM Case Rep Año: 2024 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Reino Unido
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: JCEM Case Rep Año: 2024 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Reino Unido