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Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.

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Doege-potter syndrome

Introduction

Not to be confused with Potter sequence.

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 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  (Expression error: Missing operand for *. ), 23  (Expression error: Unexpected round operator. ) 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. ↑ 1.0 1.1 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.
  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. ↑ 5.0 5.1 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.
  6. ↑ 6.0 6.1 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]”. Z Gesamte Inn Med (in German). 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. ↑ 9.0 9.1 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. ↑ 10.0 10.1 Light, Richard J. (2007). Pleural diseases. Hagerstwon, MD: Lippincott Williams & Wilkins. pp. 172–3. ISBN 0-7817-6957-4.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Carlos A Lopez, M.D. [2]