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Doege-Potter syndrome: a systematic review of the literature and case presentation of a rare pelvic malignant solitary fibrous tumour
  1. Neill Paul Kiely1,2,
  2. Rinkita Sinha1,3,
  3. Katrina Tang4 and
  4. King Man Wan1,3
  1. 1School of Women and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia
  2. 2Obstetrics and Gynaecology, The Royal Hospital for Women, Randwick, New South Wales, Australia
  3. 3Department of Gynaecological Oncology, Royal Hospital for Women, Randwick, New South Wales, Australia
  4. 4Department of Pathology, Prince of Wales Hospital, Randwick, New South Wales, Australia
  1. Correspondence to Dr Neill Paul Kiely; neilldiver{at}gmail.com

Abstract

Solitary fibrous tumours (SFTs) are a rare mesenchymal neoplasm with an incidence of 2.8 per 100 000 of which only 1% occur in the female genital tract. Doege-Potter syndrome is a paraneoplastic phenomenon associated with approximately 5%–10% of SFTs and is characterised by non-islet cell hypoglycaemia due to tumour production of low molecular weight insulin-like growth factor-II. We present the fourth confirmed case of female pelvic SFT with Doege-Potter syndrome and a literature review.

  • gynecological cancer
  • surgery

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Footnotes

  • Contributors KMW was the Gynaecological Oncologist responsible for managing the case, he wrote the case details section and reviewed and edited the case report. KT was the Pathologist responsible for the pathological diagnosis of SFT, she contributed the Tissue Histopathology section and reviewed and edited the case report. RS was the Gynaecological Oncology Fellow involved in the case, she contributed the surgical images and reviewed and edited the case report. NPK was the Gynaecological Oncology Registrar involved in the case, he compiled the case report, completed the literature review and wrote the introduction and discussion as well as editing the case report. All team members have reviewed the final draft and given approval for it to be published.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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