Effective management of recurrent Doege-Potter syndrome with
somatostatin-analogues: A case report
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.