Imatinib use in the management of a patient with Doege–Potter syndrome

Autor: Jose Paz-Ibarra, Jose Lu-Antara, Brenda-Erendida Uscamayta, Jhancy Martinez-Auris, Miriam Valencia-Rivera, Sofía Sáenz-Bustamante, Marialejandra Delgado-Rojas, Julia Salcedo-Vasquez, Marcio Concepción-Zavaleta
Jazyk: angličtina
Rok vydání: 2023
Předmět:
Zdroj: Endocrinology, Diabetes & Metabolism Case Reports, Vol 1, Iss 1, Pp 1-5 (2023)
Druh dokumentu: article
ISSN: 2052-0573
DOI: 10.1530/EDM-22-0360
Popis: Doege–Potter syndrome (DPS) is a paraneoplastic syndrome characterized by nonislet cell tumor hypoglycemia due to a solitary fibrous tumor, which produces insulin-like growth factor II. In this report, we present the case of a 67-year-old male with recurrent and refractory hypoglycemia due to DPS successfully treated with imatinib. He initially presented with neuroglycopenic symptoms and dyspnea secondary to a giant tumor in the left hemithorax, which was totally resected. During follow-up, 7 years later, he presented with thoracoabdominal tumor recurrence associated with severe hypoglycemia and underwent subtotal tumor resection, with a subsequent improvement of symptoms. The following year, he had a recurrence of his intra-abdominal tumor, which was unresectable, associated with severe hypoglycemia refractory to dextrose infusion and corticosteroids, thus receiving imatinib with a favorable response. The clinical presentation, diagnostic approach, progression of the disease, and response to treatment with imatinib in the management of a patient with large, recurrent, and unresectable mesenchymal tumors with insulin-like growth factor-2 secretion causing hypoglycemia highlight the importance of this case report.
Databáze: Directory of Open Access Journals