Solitary mediastinal fibrous tumor complicated by hypoglycemic crises (Doege—Potter syndrome)

Parshin V.D.; Fadeev V.V.; Ursov M.A.; Parshin A.V.; Runova G.E.

doi:https://doi.org/10.17116/hirurgia2023061114

Parshin V.D.

Sechenov First Moscow State Medical University;
National Medical Research Center of Phthisiopulmonology and Infectious Diseases;
Federal Medical Biological Agency

ORCID: 0000-0002-0104-481X

Fadeev V.V.

Sechenov First Moscow State Medical University (Sechenov University)

SPIN RSCI: 6825-8417
Scopus AuthorID: 7005742629
ORCID: 0000-0002-3026-6315

Ursov M.A.

Sechenov First Moscow State Medical University

ORCID: 0000-0003-1501-6374

Parshin A.V.

Sechenov First Moscow State Medical University

ORCID: 0000-0003-1804-3386

Runova G.E.

Sechenov First Moscow State Medical University

ORCID: 0000-0003-2144-8595

Solitary mediastinal fibrous tumor complicated by hypoglycemic crises (Doege—Potter syndrome)

Authors:

Parshin V.D., Fadeev V.V., Ursov M.A., Parshin A.V., Runova G.E.

More about the authors

Journal: Pirogov Russian Journal of Surgery. 2023;(6): 114‑121

DOI: 10.17116/hirurgia2023061114

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To cite this article:

Parshin VD, Fadeev VV, Ursov MA, Parshin AV, Runova GE. Solitary mediastinal fibrous tumor complicated by hypoglycemic crises (Doege—Potter syndrome). Pirogov Russian Journal of Surgery. 2023;(6):114‑121. (In Russ.)
https://doi.org/10.17116/hirurgia2023061114

Подписка 2026 Хирургия. Журнал им. Н.И. Пирогова (Pirogov Russian Journal of Surgery)

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Abstract:

Diagnostic and treatment algorithms for large mediastinal tumors are clear. However, long-term results are not always good. They largely depend on early diagnosis and morphological structure of tumor. Neoplasms may be asymptomatic for a long time, especially in case of slow growth. These tumors are usually diagnosed as soon as complications occur (for example, compression syndrome). Routine X-ray screening is rarer situation. Paraneoplastic syndromes are rare, and some ones are casuistic and unknown to surgical community. We describe the diagnosis and treatment of a patient with giant solitary mediastinal tumor complicated by hypoglycemic crises (Doege-Potter syndrome). This complication was life-threatening and required a multidisciplinary approach. Aggressive surgical approach cured the patient and returned her to normal lifestyle. The proposed algorithm for perioperative drug therapy was effective and deserves attention. This report will be useful for surgeons, oncologists, anesthesiologists, intensive care specialists and endocrinologists.

Keywords:

mediastinal surgery

rare mediastinal tumors

mediastinal solitary fibrotic tumor

Doege—Potter syndrome

hypoglycemic crisis

Authors:

Parshin V.D.

Sechenov First Moscow State Medical University;
National Medical Research Center of Phthisiopulmonology and Infectious Diseases;
Federal Medical Biological Agency

ORCID: 0000-0002-0104-481X

Fadeev V.V.

Sechenov First Moscow State Medical University (Sechenov University)

SPIN RSCI: 6825-8417
Scopus AuthorID: 7005742629
ORCID: 0000-0002-3026-6315

Ursov M.A.

Sechenov First Moscow State Medical University

ORCID: 0000-0003-1501-6374

Parshin A.V.

Sechenov First Moscow State Medical University

ORCID: 0000-0003-1804-3386

Runova G.E.

Sechenov First Moscow State Medical University

ORCID: 0000-0003-2144-8595

Received:

20.11.2022

Accepted:

15.12.2022

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References:

Qian X, Zhou D, Gao B, Wang W. Metastatic solitary fibrous tumor of the pancreas in a patient with Doege-Potter syndrome. Hepatobiliary surgery and nutrition. 2020;9(1):112-115. https://doi.org/10.21037/hbsn.2019.12.01
Delvecchio A, Duda L, Conticchio M, Fiore F, Lafranceschina S, Riccelli U, Cristofano A, Pascazio B, Colagrande A, Resta L, Memeo R. Doege-Potter syndrome by malignant solitary fibrous tumor of the liver: A case report and review of literature. World journal of gastrointestinal surgery. 2019;11(8):348-357. https://doi.org/10.4240/wjgs.v11.i8.348
Kiely NP, Sinha R, Tang K, Wan KM. Doege-Potter syndrome: a systematic review of the literature and case presentation of a rare pelvic malignant solitary fibrous tumour. BMJ case reports. 2021;14(8):e242447. https://doi.org/10.1136/bcr-2021-242447
Fort-Culillas R, Barahona San Millán R, Garcia-Fructuoso I, Quera González A, Grau Martín A, Puigdemont Guinart M, Rubió-Casadevall J, Recasens Sala M, Porta Balanyà R. Doege-Potter syndrome in a facial solitary fibrous tumor: Diagnose and clinical management discussion. Clinical case reports. 2021;9(6):e04291. https://doi.org/10.1002/ccr3.4291
Schöffski P, Timmermans I, Hompes D, et al. Clinical presentation, natural history, and therapeutic approach in patients with solitary fibrous tumor: a retrospective analysis. Sarcoma. 2020;2020:1385978. https://doi.org/10.1155/2020/1385978
Pikin OV, et al. Opuholi sredosteniya: sbornik pod red. Kaprina A.D. M.: Molodaya gvardiya; 2019. (In Russ.).
Doege KW. Fibro-sarcoma of the mediastinum. Annals of surgery. 1930;92(5):955-960.
Zafar H, Takimoto CH, Weiss G. Doege-Potter syndrome: hypogly- cemia associated with malignant solitary fibrous tumor. Med Oncol. 2003;20(4):403-408. https://doi.org/10.1385/MO:20:4:403
Zafar H, Takimoto CH, Weiss G. Doege-Potter syndrome: hypoglycemia associated with malignant solitary fibrous tumor. Med Oncol. 2003;20(4): 403-408. https://doi.org/10.1385/MO:20:4:403
Algoritmy specializirovannoj medicinskoj pomoshchi bol’nym saharnym diabetom. Pod red. Dedova I.I., Shestakovoj M.V., Majorova A.Yu. 9-j vypusk (dopolnennyj). M. 2019. (In Russ.).
NAB2-STAT6 fusions are a hallmark of solitary fibrous tumors. Cancer discovery. 2013;3(3):OF18. https://doi.org/10.1158/2159-8290.CD-RW2013-016
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: A review of the literature including a new case report. Medicine. 2017;96(27):e7417. https://doi.org/10.1097/MD.0000000000007417
Daughaday WH, Trivedi B, Baxter RC. Serum «big insulin-like growth factor II» from patients with tumor hypoglycemia lacks normal E-domain O-linked glycosylation, a possible determinant of normal propeptide processing. Proceedings of the National Academy of Sciences of the United States of America. 1993;90(12):5823-5827. https://doi.org/10.1073/pnas.90.12.5823
Pavlíková P, Sochorová L, Snížková O, Malý V, Staněk I. Solitary fibrous tumor of the pleura as a rare cause of severe hypoglycemia: Doege-Potter syndrome. Solitární fibrózní tumor pleury jako nezvyklá příčina recidivující hypoglykémie: Doege-Potterův syndrom. Rozhledy v chirurgii: mesicnik Ceskoslovenske chirurgicke spolecnosti. 2020;99(2):95-98. https://doi.org/10.33699/PIS.2020.99.2.95-98
de Perrot M, Fischer S, Bründler MA, Sekine Y, Keshavjee S. Solitary fibrous tumors of the pleura. The Annals of thoracic surgery. 2002;74(1):285-293. https://doi.org/10.1016/s0003-4975(01)03374-4
Yarwood GD, Ross RJ, Medbak S, Coakley J, Hinds CJ. Administration of human recombinant insulin-like growth factor-I in critically ill patients. Critical care medicine. 1997;25(8):1352-1361. https://doi.org/10.1097/00003246-199708000-00023
Weiss B, Horton DA. Preoperative embolization of a massive solitary fibrous tumor of the pleura. The Annals of thoracic surgery. 2002;73(3):983-985. https://doi.org/10.1016/s0003-4975(01)03117-4
Santos RS, Haddad R, Lima CE, Liu YL, Misztal M, Ferreira T, Boasquevisque CH, Luketich JD, Landreneau RJ. Patterns of recurrence and long-term survival after curative resection of localized fibrous tumors of the pleura. Clinical lung cancer. 2005;7(3):197-201. https://doi.org/10.3816/clc.2005.n.036
Tsuro K, Kojima H, Okamoto S, Yoshiji H, Fujimoto M, Uemura M, Yoshikawa M, Nakamura T, Kou S, Nakajima Y, Fukui H. Glucocorticoid therapy ameliorated hypoglycemia in insulin-like growth factor-II-producing solitary fibrous tumor. Internal medicine (Tokyo, Japan). 2006;45(8):525-529. https://doi.org/10.2169/internalmedicine.45.1611
Bourcigaux N, Arnault-Ouary G, Christol R, Périn L, Charbonnel B, Le Bouc Y. Treatment of hypoglycemia using combined glucocorticoid and recombinant human growth hormone in a patient with a metastatic non-islet cell tumor hypoglycemia. Clinical therapeutics. 2005;27(2):246-251. https://doi.org/10.1016/j.clinthera.2005.02.004
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. Journal of thoracic oncology: official publication of the International Association for the Study of Lung Cancer. 2006;1(6):588-590.
Holt RI, Simpson HL, Sönksen PH. The role of the growth hormone-insulin-like growth factor axis in glucose homeostasis. Diabetic medicine: a journal of the British Diabetic Association. 2003;20(1):3-15. https://doi.org/10.1046/j.1464-5491.2003.00827.x
Baxter R. C. (1996). The role of insulin-like growth factors and their binding proteins in tumor hypoglycemia. Hormone research. 46(4-5), 195–201. https://doi.org/10.1159/000185023
Kim DW, Na KJ, Yun JS, Song SY. Doege-potter syndrome: a report of a histologically benign but clinically malignant case. Journal of cardiothoracic surgery. 2017;12(1):64. https://doi.org/10.1186/s13019-017-0630-4
Mohammed T, Ozcan G, Siddique AS, Araneta Iii RN, Slater DE, Khan A. Doege-Potter Syndrome with a Benign Solitary Fibrous Tumor: A Case Report and Literature Review. Case reports in oncology. 2021;14(1):470-476. https://doi.org/10.1159/000512823