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[Paraneoplastic hypoglycemia: The hopes of pathophysiological documentation]

A Villemain1, O Menard2, D Mandry3

  • 1Service de pneumologie, CHR de Metz-Thionville, hôpital de Mercy, 1, allée du Château, 57530 Ars-Laquenexy, France.

Revue De Pneumologie Clinique
|April 16, 2017
PubMed

Insights

Doege-Potter syndrome, a rare paraneoplastic syndrome, involves non-islet cell tumor hypoglycemia caused by solitary fibrous tumors. Surgical resection of these tumors often cures the hypoglycemia, but long-term monitoring is crucial due to recurrence risks.

Area of Science:

  • Endocrinology
  • Oncology
  • Pathology

Background:

  • Doege-Potter syndrome is a rare paraneoplastic phenomenon.
  • It is characterized by non-islet cell tumor hypoglycemia.
  • Hypoglycemia is secondary to solitary fibrous tumors.

Observation:

  • Solitary fibrous tumors are typically asymptomatic and rare.
  • Hypoglycemia occurs in less than 5% of cases.
  • Associated tumors are often large with high mitotic rates.

Findings:

  • The hypoglycemia is caused by increased "big" insulin-like growth factor 2 (IGF-2) produced by the tumor.
  • Biological tests confirming elevated "big" IGF-2 plasma levels aid diagnosis.
  • Surgical resection is the primary diagnostic and therapeutic intervention.

Implications:

  • Surgical resection is often curative, resolving hypoglycemia.
  • Malignant transformation and recurrence are possible, necessitating long-term patient monitoring.
  • Understanding the pathophysiological mechanism is key for managing this rare paraneoplastic syndrome.
Keywords:
Fibrous pleural tumorHypoglycémie paranéoplasiqueParaneoplastic hypoglycemiaTumeur fibreuse pleurale

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