Doege-Potter syndrome

Not to be confused with Potter sequence.

Doege-Potter syndrome
Classification and external resources
The structure of IGF-2, responsible for the hypoglycemia associated with Doege-Potter syndrome

Doege-Potter syndrome (DPS) is a paraneoplastic syndrome^[1] in which hypoglycemia is associated with the presence of one or more non-islet fibrous tumors in the pleural cavity. The hypoglycemia is the result of the tumors producing insulin-like growth factor 2.^[2] The syndrome was first described in 1930, by Doege^[3] and Potter 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 tumours 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]

References

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