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Effective management of recurrent Doege-Potter syndrome with somatostatin-analogues: A case report
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  • Felix Schöler,
  • Maximilian Andreas Storz,
  • Ashkan Khavaran,
  • Nicolas Hümmler,
  • Maximilian Russe,
  • Christoph Wielenberg,
  • Katharina Laubner,
  • Jochen Seufert
Felix Schöler
Universitätsklinikum Freiburg Medizinische Universitätsklinik
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Maximilian Andreas Storz
University of Freiburg Faculty of Medicine

Corresponding Author:[email protected]

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Ashkan Khavaran
Universitätsklinikum Freiburg Medizinische Universitätsklinik
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Nicolas Hümmler
Universitätsklinikum Freiburg Medizinische Universitätsklinik
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Maximilian Russe
Universitätsklinikum Freiburg Medizinische Universitätsklinik
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Christoph Wielenberg
Universitätsklinikum Freiburg Medizinische Universitätsklinik
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Katharina Laubner
Universitätsklinikum Freiburg Medizinische Universitätsklinik
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Jochen Seufert
Universitätsklinikum Freiburg Medizinische Universitätsklinik
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Abstract

Background Doege-Potter syndrome is defined as paraneoplastic hypoinsulinemic hypoglycemia associated with a benign or malignant solitary fibrous tumor frequently located in pleural, but also extrapleural sites. Hypoglycemia can be attributed to paraneoplastic secretion of ‘Big-IGF-II’, a precursor of Insulin-like growth factor-II. This prohormone aberrantly binds to and activates insulin receptors, with consecutive initiation of common insulin actions such as inhibition of gluconeogenesis, activation of glycolysis and stimulation of cellular glucose uptake culminating in recurrent tumor-induced hypoglycemic episodes. Complete tumor resection or debulking surgery is considered the most promising treatment for DPS. Case Here we report a rare case of a recurrent Doege-Poter Syndrome with atypical gelatinous tumor lesions of the lung, pleura and pericardial fat tissue in an 87-year-old woman. Although previously described as ineffective, we demonstrate that adjuvant treatment with Octreotide in conjunction with intravenous glucose helps to maintain tolerable blood glucose levels before tumor resection. The somatostatin-analogue Lanreotide was successfully used after tumor debulking surgery to maintain adequate blood glucose control. Conclusion We conclude that somatostatin-analogues bear the potential of being effective in conjunction with limited surgical approaches for the treatment of hypoglycemia in recurrent or non-totally resectable SFT entities underlying DPS.
23 Jul 2023Submitted to Cancer Reports
25 Jul 2023Assigned to Editor
25 Jul 2023Submission Checks Completed
10 Aug 2023Review(s) Completed, Editorial Evaluation Pending
16 Aug 2023Reviewer(s) Assigned
12 Nov 2023Editorial Decision: Revise Major