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Doege–Potter syndrome

Hypoglycemia (low blood sugar) due to the presence of fibrous tumors


Hypoglycemia (low blood sugar) due to the presence of fibrous tumors

FieldValue
nameDoege–Potter syndrome
imagePBB Protein IGF2 image.jpg
captionThe structure of IGF-2, responsible for the hypoglycemia associated with Doege–Potter syndrome
specialtyOncology

Doege–Potter syndrome (DPS) is a paraneoplastic syndrome in which hypoglycemia is associated with solitary fibrous tumors. The hypoglycemia is the result of the tumors producing insulin-like growth factor 2. The syndrome was first described in 1930, by Karl Walter Doege (1867–1932), a German-American physician and by Roy Pilling Potter (1879–1968), an American radiologist, working independently; the full term Doege–Potter syndrome was infrequently used until the publication of a 2000 article using the eponym.

DPS is rare (as of 1976, less than one hundred cases were described), with a malignancy rate of 12–15%. Actual rates of hypoglycemia associated with a fibrous tumor are quite rare (a 1981 study of 360 solitary fibrous tumors of the lungs found that only 4% caused hypoglycemia), and are linked to large tumors with high rates of mitosis. Removal of the tumor will normally resolve the symptoms.

Tumors causing DPS tend to be quite large; in one case a 3 kg, 23 * mass was removed, sufficiently large to cause a collapsed lung. In X-rays, they appear as a single mass with visible, defined borders, appearing at the edges of the lungs or a fissure dividing the lobes of the lungs. Similar hypoglycemic effects have been related to mesenchymal tumors.

References

References

  1. (July 2006). "Solitary fibrous tumor of the pleura with associated hypoglycemia: Doege–Potter syndrome: a case report". J Thorac Oncol.
  2. (2000). "Primary malignant fibrous histiocytoma of the lung: IGF-II producing tumor induces fasting hypoglycemia". Exp. Clin. Endocrinol. Diabetes.
  3. Doege, KW. (1930). "Fibrosarcoma of the mediastinum". Ann Surg.
  4. (November 1992). "Solitary fibrous tumor of the pleura with hypoglycemia: the Doege–Potter syndrome". J Ky Med Assoc.
  5. (January 2000). "Solitary fibrous tumor associated with hypoglycemia: an example of the Doege–Potter syndrome". J. Thorac. Cardiovasc. Surg..
  6. Shields, TW. (2005). "General thoracic surgery". Lippincott Williams & Wilkins.
  7. (February 1976). "[Intrathoracal tumor with accompanying hypoglycemia]". Z Gesamte Inn Med.
  8. (June 1981). "Solitary fibrous tumors of the pleura: eight new cases and review of 360 cases in the literature". Cancer.
  9. (2003). "Doege–Potter syndrome: hypoglycemia associated with malignant solitary fibrous tumor". Med. Oncol..
  10. Light, Richard J.. (2007). "Pleural diseases". Lippincott Williams & Wilkins.
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