Nico Heß, Ali Seif Amir Hosseini, Carolin Sobotta, Giovanni Frederico Torsello, Florian Bösch, Neriona Vorpsi, Philipp Ströbel, Albrecht Neesse
Combined glucocorticoid therapy and selective embolization may provide sustained glycaemic control in elderly patients with retroperitoneal SFT and Doege-Potter syndrome when surgery is not feasible.
BACKGROUND: Solitary fibrous tumours (SFTs) are rare mesenchymal neoplasms that may cause insulin-independent hypoglycaemia (Doege-Potter-syndrome). Although surgical resection is the standard treatment, non-surgical options may be required for elderly or multimorbid patients.
CASE PRESENTATION: An 82-year-old woman presented with severe nocturnal hypoglycaemia (<40mg/dl) and progressive weakness. Imaging revealed a large hypervascular retroperitoneal mass. Laboratory findings showed low insulin and C-peptide levels with an IGF-II/IGF-I ratio of 9.3, indicating paraneoplastic hypoglycaemia. Biopsy demonstrated low-grade malignant SFT with STAT6 and CD34 positivity. Because of advanced age and comorbidities, dexamethasone (8mg/day) was initiated, followed by two selective transarterial embolizations over 6 months. Hypoglycaemia resolved rapidly, allowing dexamethasone reducing to 1.5mg/day. At 9-month follow-up, only rare hypoglycaemic episodes remained and were managed with regular meals and low-dose dexamethasone.
CONCLUSION: Combined glucocorticoid therapy and selective embolization may provide sustained glycaemic control in elderly patients with retroperitoneal SFT and Doege-Potter syndrome when surgery is not feasible.