Imatinib use in the management of a patient with Doege–Potter syndrome
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 su...
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| Format: | Article |
| Language: | English |
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Bioscientifica
2023-04-01
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| Series: | Endocrinology, Diabetes & Metabolism Case Reports |
| Online Access: | https://edm.bioscientifica.com/view/journals/edm/2023/2/EDM22-0360.xml |
| _version_ | 1797840431611904000 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Jose Paz-Ibarra |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-09T16:15:00Z |
| format | Article |
| id | doaj.art-bd2e4dbbf6ff40308e34afd05fdf5bd8 |
| institution | Directory Open Access Journal |
| issn | 2052-0573 |
| language | English |
| last_indexed | 2024-04-09T16:15:00Z |
| publishDate | 2023-04-01 |
| publisher | Bioscientifica |
| record_format | Article |
| series | Endocrinology, Diabetes & Metabolism Case Reports |
| spelling | doaj.art-bd2e4dbbf6ff40308e34afd05fdf5bd82023-04-24T06:42:23ZengBioscientificaEndocrinology, Diabetes & Metabolism Case Reports2052-05732023-04-01111510.1530/EDM-22-0360Imatinib use in the management of a patient with Doege–Potter syndromeJose Paz-Ibarra0Jose Lu-Antara1Brenda-Erendida Uscamayta2Jhancy Martinez-Auris3Miriam Valencia-Rivera4Sofía Sáenz-Bustamante5Marialejandra Delgado-Rojas6Julia Salcedo-Vasquez7Marcio Concepción-Zavaleta8Faculty of Medicine, National University of San Marcos, Lima, Peru; National Hospital Edgardo Rebagliati Martins, Lima, PeruFaculty of Medicine, National University of San Marcos, Lima, Peru; Scientific Society of San Fernando, Lima, PeruFaculty of Medicine, National University of San Marcos, Lima, Peru; Scientific Society of San Fernando, Lima, PeruFaculty of Medicine, National University of San Marcos, Lima, Peru; Scientific Society of San Fernando, Lima, PeruFaculty of Medicine, National University of San Marcos, Lima, Peru; Scientific Society of San Fernando, Lima, PeruFaculty of Medicine, National University of San Marcos, Lima, Peru; National Hospital Edgardo Rebagliati Martins, Lima, PeruFaculty of Medicine, National University of San Marcos, Lima, Peru; National Hospital Edgardo Rebagliati Martins, Lima, PeruFaculty of Medicine, National University of San Marcos, Lima, Peru; National Hospital Edgardo Rebagliati Martins, Lima, PeruDivision of Endocrinology. School of Medicine. Norbert Wiener University. Lima, PeruDoege–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.https://edm.bioscientifica.com/view/journals/edm/2023/2/EDM22-0360.xml |
| spellingShingle | 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 Imatinib use in the management of a patient with Doege–Potter syndrome Endocrinology, Diabetes & Metabolism Case Reports |
| title | Imatinib use in the management of a patient with Doege–Potter syndrome |
| title_full | Imatinib use in the management of a patient with Doege–Potter syndrome |
| title_fullStr | Imatinib use in the management of a patient with Doege–Potter syndrome |
| title_full_unstemmed | Imatinib use in the management of a patient with Doege–Potter syndrome |
| title_short | Imatinib use in the management of a patient with Doege–Potter syndrome |
| title_sort | imatinib use in the management of a patient with doege potter syndrome |
| url | https://edm.bioscientifica.com/view/journals/edm/2023/2/EDM22-0360.xml |
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