Imatinib use in the management of a patient with Doege–Potter syndrome
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Fecha
2023-04Autor(es)
Paz-Ibarra, Jose
Lu-Antara, Jose
Uscamayta, Brenda-Erendida
Martinez-Auris, Jhancy
Valencia-Rivera, Miriam
Sáenz-Bustamante, Sofía
Delgado-Rojas, Marialejandra
Salcedo-Vasquez, Julia
Concepción-Zavaleta, Marcio
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“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“
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