Generic placeholder image

Endocrine, Metabolic & Immune Disorders - Drug Targets

Editor-in-Chief

ISSN (Print): 1871-5303
ISSN (Online): 2212-3873

Case Report

Doege-Potter Syndrome; A Case of Solitary Fibrous Pleura Tumor Associated with Severe Hypoglycemia: A Case Report in Internal Medicine

Author(s): Viviana Castaldo, Daniela Domenici*, Mauro Valentino Biscosi, Paolo Ubiali, Cesare Miranda, Giorgio Zanette, Cinzia Mazzon and Maurizio Tonizzo

Volume 23, Issue 12, 2023

Published on: 24 July, 2023

Page: [1562 - 1569] Pages: 8

DOI: 10.2174/1871530323666230623112047

open access plus

Open Access Journals Promotions 2
conference banner
Abstract

Background: Doege-Potter syndrome is a rare paraneoplastic entity that is often diagnosed incidentally during the work-up of hypoglycemia of unclear etiology. It is characterized by a non-islet cell tumor hypoglycemia mostly associated with solitary fibrous tumors. These uncommon tumors have been reported in <5% of solitary fibrous tumors. Although not unique in its kind, this case is extremely important as this syndrome often conceals unrecognized tumors that can be surgically resolved.

Case Presentation: We present the case of a 59-year-old non-diabetic man with a 2-month history of severe and recurrent fasting hypoglycaemia presenting with severe dyspnea and sweating. Further workup revealed low insulin, C-peptide, and IGF-1 levels and a large right in-trathoracic solitary fibrous tumor. Unfortunately, bioassays for IGF-2 were unavailable at our hos-pital. Nevertheless, as hypoglycemia completely resolved after resection of the mass, Doege-Potter syndrome was highly suspected.

Conclusion: Doege-Potter syndrome is a complication of rare tumors. If hy-poglycemia is unexplained, this syndrome should always be suspected, and the presence of un-known masses should be investigated.

Keywords: Hypoglycemia, doege-potter syndrome, non-islet cell tumor hypoglycemia, solitary fibrous tumor, IGF-2, STAT6.

« Previous
Graphical Abstract
[1]
Kim, D.W.; Na, K.J.; Yun, J.S.; Song, S.Y. Doege-potter syndrome: A report of a histologically benign but clinically malignant case. J. Cardiothorac. Surg., 2017, 12(1), 64.
[http://dx.doi.org/10.1186/s13019-017-0630-4] [PMID: 28784156]
[2]
Rötgens, J.; Lapauw, B. T’Sjoen, G. Doege-Potter syndrome in a patient with a giant abdominal solitary fibrous tumor: a case report and review of the literature. Acta Clin. Belg., 2023, 78(4), 358-364.
[http://dx.doi.org/10.1080/17843286.2023.2165652] [PMID: 36641633]
[3]
Dutta, P.; Aggarwal, A.; Gogate, Y.; Nahar, U.; Shah, V.N.; Singla, M.; Khandelwal, N.; Bhansali, A. Non-islet cell tumor-induced hypoglycemia: A report of five cases and brief review of the literature. Endocrinol. Diabetes Metab. Case Rep., 2013, 2013, 130046.
[http://dx.doi.org/10.1530/EDM-13-0046 ] [PMID: 24616774]
[4]
Pincelli, A.I.; Perotti, M.; Massariello, F.; Gatti, A.; Calella, D.; Cimino, V.; Haas, J.; Bellelli, G.; Mazzola, P.; Annoni, G. A Rare diagnosis after the fall of a 96-year-old woman: Doege-potter syndrome. Curr. Aging Sci., 2019, 11(3), 195-200.
[http://dx.doi.org/10.2174/1874609812666181205142247] [PMID: 30520387]
[5]
Meng, W.; Zhu, H.H.; Li, H.; Wang, G.; Wei, D.; Feng, X. Solitary fibrous tumors of the pleura with Doege-Potter syndrome: a case report and three-decade review of the literature. BMC Res. Notes, 2014, 7(1), 515.
[http://dx.doi.org/10.1186/1756-0500-7-515] [PMID: 25113505]
[6]
Ershadi, R.; Rahim, M.; Abbasi, M.; Erfanian, R. Giant solitary fibrous tumor of the pleura. J. Surg. Case Rep., 2018, 2018(11), rjy270.
[http://dx.doi.org/10.1093/jscr/rjy270] [PMID: 30397437]
[7]
Han, G.; Zhang, Z.; Shen, X.; Wang, K.; Zhao, Y.; He, J.; Gao, Y.; Shan, X.; Xin, G.; Li, C.; Liu, X. Doege–Potter syndrome. Medicine, 2017, 96(27), e7417.
[http://dx.doi.org/10.1097/MD.0000000000007417] [PMID: 28682900]
[8]
Smrke, A.; Thway, K.; Huang, H. P.; Jones, R.L.; Hayes, A.J. Solitary fibrous tumor: Molecular hallmarks and treatment for a rare sarcoma. Future Oncol., 2021, 17(27), 3627-3636.
[http://dx.doi.org/10.2217/fon-2021-0030] [PMID: 34409859]
[9]
Davda, R.; Seddon, B.M. Mechanisms and management of non-islet cell tumour hypoglycaemia in gastrointestinal stromal tumour: case report and a review of published studies. Clin. Oncol., 2007, 19(4), 265-268.
[http://dx.doi.org/10.1016/j.clon.2006.12.008] [PMID: 17433971]
[10]
Doorn, J. Insulin‐like growth factor‐II and bioactive proteins containing a part of the E‐domain of pro‐insulin‐like growth factor‐II. Biofactors, 2020, 46(4), 563-578.
[http://dx.doi.org/10.1002/biof.1623] [PMID: 32026557]
[11]
Olson, N.J.; Linos, K. Dedifferentiated solitary fibrous tumor: A concise review. Arch. Pathol. Lab. Med., 2018, 142(6), 761-766.
[http://dx.doi.org/10.5858/arpa.2016-0570-RS] [PMID: 29848035]
[12]
Ajouz, H.; Sohail, A.H.; Hashmi, H.; Martinez Aguilar, M.; Daoui, S.; Tembelis, M.; Aziz, M.; Zohourian, T.; Brathwaite, C.E.M.; Cerfolio, R.J. Surgical considerations in the resection of solitary fibrous tumors of the pleura. J. Cardiothorac. Surg., 2023, 18(1), 79.
[http://dx.doi.org/10.1186/s13019-023-02168-7] [PMID: 36823638]
[13]
van Doorn, J.; van de Hoef, W.; Dullaart, R.P.F. Quantitative analysis of the concentrations of IGFs and several IGF-binding proteins in a large fibrous abdominal tumor and the circulation of a patient with hypoglycemia. Biofactors, 2015, 41(3), 183-189.
[http://dx.doi.org/10.1002/biof.1212] [PMID: 26073062]
[14]
Tariq, M.U.; Din, N.U.; Abdul-Ghafar, J.; Park, Y.K. The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior. Diagn. Pathol., 2021, 16(1), 32.
[http://dx.doi.org/10.1186/s13000-021-01095-2] [PMID: 33879215]
[15]
Solsi, A.; Pho, K.; Shojaie, S.; Findakly, D.; Noori, T. Doege-potter syndrome and pierre-marie-bamberger syndrome in a patient with pleural solitary fibrous tumor: A rare case with literature review. Cureus, 2020, 12(5), e7919.
[http://dx.doi.org/10.7759/cureus.7919] [PMID: 32494533]
[16]
Mohammed, T.; Ozcan, G.; Siddique, A.S.; Araneta, R.N., III; Slater, D.E.; Khan, A. Doege-potter syndrome with a benign solitary fibrous tumor: A case report and literature review. Case Rep. Oncol., 2021, 14(1), 470-476.
[http://dx.doi.org/10.1159/000512823] [PMID: 33976622]
[17]
De Los Santos-Aguilar, R.G.; Chávez-Villa, M.; Contreras, A.G.; García-Herrera, J.S.; Gamboa-Domínguez, A.; Vargas-Sánchez, J.; Almeda-Valdes, P.; Reza-Albarrán, A.A.; Iñiguez-Ariza, N.M. Successful multimodal treatment of an igf2-producing solitary fibrous tumor with acromegaloid changes and hypoglycemia. J. Endocr. Soc., 2019, 3(3), 537-543.
[http://dx.doi.org/10.1210/js.2018-00281] [PMID: 30788455]
[18]
Bodnar, T.W.; Acevedo, M.J.; Pietropaolo, M. Management of non-islet-cell tumor hypoglycemia: A clinical review. J. Clin. Endocrinol. Metab., 2014, 99(3), 713-722.
[http://dx.doi.org/10.1210/jc.2013-3382] [PMID: 24423303]
[19]
Schutt, R.C.; Gordon, T.A.; Bhabhra, R.; Cathro, H.P.; Cook, S.L.; McCartney, C.R.; Weiss, G.R. Doege-Potter syndrome presenting with hypoinsulinemic hypoglycemia in a patient with a malignant extrapleural solitary fibrous tumor: A case report. J. Med. Case Reports, 2013, 7(1), 11.
[http://dx.doi.org/10.1186/1752-1947-7-11] [PMID: 23302323]

© 2024 Bentham Science Publishers | Privacy Policy