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Case report
Doege-Potter syndrome presenting as ‘end-stage renal disease-associated hypoglycaemia’: a primary presentation of retroperitoneal sarcoma
  1. Skand Shekhar1,
  2. Julie Chen2 and
  3. Kaniksha Desai2
  1. 1Section on Genetics and Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Bethesda, Maryland, USA
  2. 2Division of Endocrinology, Gerontology, and Metabolism, Stanford University School of Medicine, Stanford, California, USA
  1. Correspondence to Dr Skand Shekhar; skand.shekhar{at}nih.gov

Abstract

A middle-aged woman with end-stage renal disease (ESRD) due to obstructive nephropathy presented to the hospital for an episode of unresponsiveness and hypoglycaemia. Initially, she was diagnosed with hypoglycaemia associated with ESRD and was discharged. However, she returned to the hospital after experiencing tonic–clonic seizures and recurrent hypoglycaemia. Her hypoglycaemia workup revealed an elevated insulin-like growth factor 2 (IGF2) to IGF1 ratio consistent with paraneoplastic IGF2 secretion. Subsequently, a CT abdomen revealed a retroperitoneal mass, found to be a retroperitoneal sarcoma. Her hypoglycaemia was treated with glucocorticoids and growth hormone. Surgical debulking of her tumour was attempted, but she expired due to postoperative haemorrhagic shock. Doege-Potter syndrome is a rare cause of hypoglycaemia which should be suspected in any new-onset, worsening, inexplicable or refractory hypoglycaemia, particularly in non-diabetic ESRD. Here we present a report of retroperitoneal sarcoma presenting with hypoglycaemia in a patient with ESRD without diabetes.

  • endocrinology
  • metabolic disorders
  • chronic renal failure
  • endocrine cancer
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Footnotes

  • Twitter @skandshekharMD

  • Contributors SS drafted portions of the manuscript, was involved in patient care, obtained images, made editorial changes and formatted the text. JC assisted in drafting the manuscript, was involved in patient care as a fellow physician, reviewing the literature and assembling tables. KD conceptualised the manuscript, was involved in patient care as attending physician, drafted portions of the manuscript and made revisions. All the authors approved the final version of the manuscript.

  • Funding SS receives funding from the Intramural Research Program (IRP) of the National Institutes of Health (NIH).

  • Competing interests None declared.

  • Patient consent for publication Not required.

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

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