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113 Doege – potter syndrome: a rare presentation of pelvic malignant solitary fibrous tumour
  1. R Sinha,
  2. N Kiely and
  3. K Wan
  1. The Royal Hospital for Women, Australia


Introduction Solitary fibrous tumours (SFTs) are a rare mesenchymal neoplasm with an incidence of 2.8 per 100,000. Only 1% are reported to occur in the female genital tract. Approximately 5–10% of SFTs are associated with Doege–Potter syndrome, a para-neoplastic phenomenon with non-islet cell hypoglycaemia due to tumour production of low molecular weight insulin growth-like factor (IGF) - II.

Methods We present the third reported case of a pelvic SFT with Doege–Potter syndrome in a 61 year-old woman who presented with an unwitnessed collapse at home and a BSL of 1.25 mg/dL. CT scan found a 23 cm right sided pelvic mass adjacent to the uterus. She underwent emergency surgery due to refractory hypoglycaemia. The mass was extremely vascular and resulted in an 8.3L blood loss requiring a massive transfusion, postoperative ICU admission and management of acute kidney injury and transfusion-related lung injury. Glycaemic control was achieved immediately upon tumour removal. Her recovery was uncomplicated and final histopathology confirmed SFT with malignant transformation. We present a literature review of the previous cases and discuss the challenges involved in diagnosis and treatment.

Conclusion Doege - Potter syndrome is an extremely rare presentation of pelvic SFT. Complete surgical excision is the gold standard for treatment of this rare condition.

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