Doege–Potter syndrome explained

Doege–Potter syndrome should not be confused with Potter sequence.

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 3kg (07lb), 23* 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]

Notes and References

1. Balduyck B, Lauwers P, Govaert K, Hendriks J, De Maeseneer M, Van Schil P . Solitary fibrous tumor of the pleura with associated hypoglycemia: Doege–Potter syndrome: a case report . J Thorac Oncol . 1 . 6 . 588–90 . July 2006 . 17409923 . 10.1097/01243894-200607000-00016.
2. Herrmann BL, Saller B, Kiess W . Primary malignant fibrous histiocytoma of the lung: IGF-II producing tumor induces fasting hypoglycemia . Exp. Clin. Endocrinol. Diabetes . 108 . 8 . 515–18 . 2000 . 11149628 . 10.1055/s-2000-11007. etal.
3. Doege . KW . Fibrosarcoma of the mediastinum . Ann Surg . 1930 . 92 . 955–960 . 17866430 . 5 . 1398259 .
4. Roy M, Burns MV, Overly DJ, Curd BT . Solitary fibrous tumor of the pleura with hypoglycemia: the Doege–Potter syndrome . J Ky Med Assoc . 90 . 11 . 557–60 . November 1992 . 1474302 .
5. Chamberlain MH, Taggart DP . Solitary fibrous tumor associated with hypoglycemia: an example of the Doege–Potter syndrome . J. Thorac. Cardiovasc. Surg. . 119 . 1 . 185–7 . January 2000 . 10612786 . 10.1016/S0022-5223(00)70242-X . free .
6. Book: Shields, TW . LoCicero J . Ponn RB . Rusch VW . General thoracic surgery . Lippincott Williams & Wilkins . Hagerstwon, MD . 2005 . 893. 0-7817-3889-X .
7. Ellorhaoui M, Graf B . [Intrathoracal tumor with accompanying hypoglycemia] . de . Z Gesamte Inn Med . 31 . 3 . 77–81 . February 1976 . 785836 .
8. Briselli M, Mark EJ, Dickersin GR . Solitary fibrous tumors of the pleura: eight new cases and review of 360 cases in the literature . Cancer . 47 . 11 . 2678–89 . June 1981 . 7260861 . 10.1002/1097-0142(19810601)47:11<2678::AID-CNCR2820471126>3.0.CO;2-9. 12321736 .
9. Zafar H, Takimoto CH, Weiss G . Doege–Potter syndrome: hypoglycemia associated with malignant solitary fibrous tumor . Med. Oncol. . 20 . 4 . 403–08 . 2003 . 14716039 . 10.1385/MO:20:4:403 . 40594239 .
10. Book: Light, Richard J. . Pleural diseases . Lippincott Williams & Wilkins . Hagerstwon, MD . 2007 . 172–3 . 978-0-7817-6957-0 .