Doege–Potter syndrome | |
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The structure of IGF-2, responsible for the hypoglycemia associated with Doege–Potter syndrome | |
Specialty | Oncology |
Doege–Potter syndrome (DPS) is a paraneoplastic syndrome [1] in which hypoglycemia is associated with solitary fibrous tumors. The hypoglycemia is the result of the tumors producing insulin-like growth factor 2. [2] The syndrome was first described in 1930, by Karl Walter Doege (1867–1932), a German-American physician [3] and by Roy Pilling Potter (1879–1968), an American radiologist, working independently; [4] the full term Doege–Potter syndrome was infrequently used until the publication of a 2000 article [5] using the eponym. [6]
DPS is rare (as of 1976, less than one hundred cases were described [7]), 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 [8]), and are linked to large tumors with high rates of mitosis. [9] Removal of the tumor will normally resolve the symptoms. [1] [9]
Tumors causing DPS tend to be quite large; [10] in one case a 3 kg (6.6 lb), 23×21×12 cm (9.1×8.3×4.7 in) mass was removed, sufficiently large to cause a collapsed lung. [5] 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. [10] Similar hypoglycemic effects have been related to mesenchymal tumors. [6]
Doege–Potter syndrome | |
---|---|
The structure of IGF-2, responsible for the hypoglycemia associated with Doege–Potter syndrome | |
Specialty | Oncology |
Doege–Potter syndrome (DPS) is a paraneoplastic syndrome [1] in which hypoglycemia is associated with solitary fibrous tumors. The hypoglycemia is the result of the tumors producing insulin-like growth factor 2. [2] The syndrome was first described in 1930, by Karl Walter Doege (1867–1932), a German-American physician [3] and by Roy Pilling Potter (1879–1968), an American radiologist, working independently; [4] the full term Doege–Potter syndrome was infrequently used until the publication of a 2000 article [5] using the eponym. [6]
DPS is rare (as of 1976, less than one hundred cases were described [7]), 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 [8]), and are linked to large tumors with high rates of mitosis. [9] Removal of the tumor will normally resolve the symptoms. [1] [9]
Tumors causing DPS tend to be quite large; [10] in one case a 3 kg (6.6 lb), 23×21×12 cm (9.1×8.3×4.7 in) mass was removed, sufficiently large to cause a collapsed lung. [5] 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. [10] Similar hypoglycemic effects have been related to mesenchymal tumors. [6]