Doege-Potter Syndrome: A Presumptive Case of Metastatic Hemangiopericytoma with Persistent Hypoglycemia in a 27-Year-Old Male

  • Von Lovel Zarra Southern Philippines Medical Center, Davao City, Philippines https://orcid.org/0000-0002-4551-9434
  • Kenny Jun Demegillo Southern Philippines Medical Center, Davao City, Philippines
  • May Uyking-Naranjo Southern Philippines Medical Center, Davao City, Philippines
  • Ahmad Domado Southern Philippines Medical Center, Davao City, Philippines
Keywords: Doege-Potter syndrome, hemangioperictyoma, hypoglycemia

Abstract

Doege-Potter syndrome (DPS) is a rare paraneoplastic condition characterized by hypoinsulinemic hypoglycemia from a solitary fibrous tumor. The underlying mechanism is the secretion of a prohormone form of insulin-like growth factor II (IGF-II) by the tumor, which causes decreased release of glucose into the circulation. We report the case of a 27-year-old Filipino male with presumptive DPS from a recurrent right temporo-zygomatic hemangiopericytoma (HPC). The complexity of DPS requires a multidisciplinary approach. Early screening for metastases from HPC may prevent the undesirable sequelae of the disease process.

 

Downloads

Download data is not yet available.

Author Biographies

Von Lovel Zarra, Southern Philippines Medical Center, Davao City, Philippines

Medical Officer III, Department of Internal Medicine

Kenny Jun Demegillo, Southern Philippines Medical Center, Davao City, Philippines

Medical Specialist, Department of Endocrinology

May Uyking-Naranjo, Southern Philippines Medical Center, Davao City, Philippines

Medical Specialist, Section of Endocrinology, Department of Internal Medicine

Ahmad Domado, Southern Philippines Medical Center, Davao City, Philippines

Department of Internal Medicine

References

Kim DW, Na KJ, Yun JS, Song SY. Doege-Potter syndrome: A report of a histologically benign but clinically malignant case. J Cardiothorac Surg. 2017;12(1):64. https://www.ncbi.nlm.nih.gov/pubmed/28784156. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5547527. https://doi.org/10.1186/s13019-017-0630-4.

Schutt RC, Gordon TA, Bhabhra R, et al. Doege-Potter Syndrome presenting with hypoinsulinemic hypoglycemia in a patient with malignant extrapleural solitary fibrous tumor: A case report. J Med Case Rep. 2013;7:11. https://www.ncbi.nlm.nih.gov/pubmed/23302323. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3546898. https://doi.org/10.1186/1752-1947-7-11.

Bi X, Zhai J, Chun CD. Solitary fibrous tumor of the abdominal wall resurfacing as unilateral pleural effusion and mass: A case report and review of the literature. Respir Med Case Rep. 2018;23:4-7. https://www.ncbi.nlm.nih.gov/pubmed/29159031. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5683803. https://doi.org/10.1016/j.rmcr.2017.10.013.

Jameson JL, Kasper DL, Longo DL, Fauci AS, Hauser SL, Loscalzo J, eds. Harrison's Principles of Internal Medicine. 20th ed. Columbus, Ohio: McGraw-Hill Education, 2018.

Faulhaber GAM, Borges FK, Ascoli AM, Seligman R, Furlanetto TW. Adrenal failure due to adrenal metastasis of lung cancer: A case report. Case Rep Oncol Med. 2011;2011:326815.

https://www.ncbi.nlm.nih.gov/pubmed/22606443. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3350132. https://doi.org/10.1155/2011/326815.

Cryer PE, Axelrod L, Grossman AB, et al. Evaluation and management of adult hypoglycemic disorders: An Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2009;94(3):709-28. https://www.ncbi.nlm.nih.gov/pubmed/19088155. https://doi.org/10.1210/jc.2008-1410.

Dutta P, Aggarwal A, Gogate Y, et al. 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. https://www.ncbi.nlm.nih.gov/pubmed/24616774. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3922193. https://doi.org/10.1530/EDM-13-0046.

Daughaday WH, Kapadia M. Significance of abnormal serum binding of insulin-like growth factor II in the development of hypoglycemia in patients with non-islet cell tumors. Proc Natl Acad Sci USA. 1989;86(17):6778-82. https://www.ncbi.nlm.nih.gov/pubmed/2771956. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC297929. https://doi.org/10.1073/pnas.86.17.6778.

Stout AP, Murray MR. Hemangiopericytoma: A vascular tumor featuring Zimmermann's pericytes. Ann Surg. 1942;116(1):26-33. https://www.ncbi.nlm.nih.gov/pubmed/17858068. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1543753. https://doi.org/10.1097/00000658-194207000-00004.

Koch M, Nielsen GP, Yoon SS. Malignant tumors of blood vessels: Angiosarcomas, hemangioendotheliomas, and hemangioperictyomas. J Surg Oncol. 2008;97(4):321-9.

https://www.ncbi.nlm.nih.gov/pubmed/18286475. https://doi.org/10.1002/jso.20973.

Hendrick L, Angel W, Besh S, O’Brien T, Goins N, Dickson P. Multidisciplinary management of metastatic hemangiopericytoma presenting with hypoglycemia. Ann Hematol Oncol. 2017; 4(3):1140.

Penel N, Amela EY, Decanter G, Robin YM, Marec-Berard P. Solitary fibrous tumors and so-called hemangiopericytoma. Sarcoma. 2012;2012:690251. https://www.ncbi.nlm.nih.gov/pubmed/22566753. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3337510. https://doi.org/10.1155/2012/690251.

Robinson LA. Solitary fibrous tumor of the pleura. Cancer Control. 2006;13(4):264-9.

https://www.ncbi.nlm.nih.gov/pubmed/17075563. https://doi.org/10.1177/107327480601300403.

Vallat-Decouvelaere AV, Dry SM, Fletcher CD. Atypical and malignant solitary fibrous tumors in extrathoracic locations: Evidence of their comparability to intra-thoracic tumors. Am J Surg Pathol. 1998;22(12):1501-11. https://www.ncbi.nlm.nih.gov/pubmed/9850176. https://doi.org/10.1097/00000478-199812000-00007.

Espat NJ, Lewis JJ, Leung D, et al. Conventional hemangiopericytoma: Modern analysis of outcome. Cancer. 2002;95(8):1746-51. https://www.ncbi.nlm.nih.gov/pubmed/12365023. https://doi.org/10.1002/cncr.10867.

Ng VY, Scharschmidt TJ, Mayerson JL. Solitary fibrous tumor. 2019. https://emedicine.medscape.com/article/1255879-overview#a4.

Chen LF, Yang Y, Yu XG, Gui QP, Xu BN, Zhou DB. Multimodal treatment and management strategies for intracranial hemangiopericytoma. J Clin Neurosci. 2015;22(4):718-25. https://www.ncbi.nlm.nih.gov/pubmed/25744076. https://doi.org/10.1016/j.jocn.2014.11.011.

Wu R. Solitary fibrous tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lungtumorsolitaryfibroustumor.html. Accessed 27 February 2021.

Geramizadeh B, Marzban M, Churg A. Role of immunohistochemistry in the diagnosis of solitary fibrous tumor, a review. Iran J Pathol. 2016;11(3):195-203.

https://www.ncbi.nlm.nih.gov/pubmed/27799967. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5079451.

England DM, Hochholzer L, McCarthy MJ. Localized benign and malignant fibrous tumors of the pleura. A clinicopathologic review of 223 cases. Am J Surg Pathol. 1989;13(8):640-58. https://www.ncbi.nlm.nih.gov/pubmed/2665534. https://doi.org/10.1097/00000478-198908000-00003.

Kalebi AY, Hale MJ, Wong ML, Hoffman T, Murray J. Surgically cured hypoglycemia secondary to pleural solitary fibrous tumour: Case report and update review on the Doege-Potter syndrome. J Cardiothorac Surg. 2009;4:45.

https://www.ncbi.nlm.nih.gov/pubmed/19689813. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2739517. https://doi.org/10.1186/1749-8090-4-45.

Roy TM, Burns MV, Overly DJ, Curd BT. Solitary fibrous tumor of the pleura with hypoglycemia: The Doege-Potter syndrome. J Ky Med Assoc. 1992;90(11): 557-60.

https://www.ncbi.nlm.nih.gov/pubmed/1474302.

Chamberlain MH, Taggart DP. Solitary fibrous tumor associated with hypoglycemia: An example of the Doege-Potter syndrome. J Thorac Cardiovasc Surg. 2000;119(1):185-7.

https://www.ncbi.nlm.nih.gov/pubmed/10612786. https://doi.org/10.1016/s0022-5223(00)70242-x.

Davda R, Seddon BM. Mechanisms and management of non-islet cell tumour hypoglycaemia in gastrointestinal stromal tumour: Case report and a review of published studies. Clin Oncol (R Coll Radiol). 2007;19(4):265-8.

https://www.ncbi.nlm.nih.gov/pubmed/17433971. https://doi.org/10.1016/j.clon.2006.12.008.

Sohda T, Yun K. Insulin-like growth factor II expression in primary meningeal hemangiopericytoma and its metastasis to the liver accompanied by hypoglycemia. Hum Pathol. 1996;27(8):858-61. https://www.ncbi.nlm.nih.gov/pubmed/8760024. https://doi.org/10.1016/s0046-8177(96)90463-3.

Strâmbu IR, Leonte DG, Bolca CN. Large pleural tumor revealed by severe hypoglycemia: Doege-Potter syndrome. Rom J Morphol Embryol. 2017;58(1):287-91.

https://www.ncbi.nlm.nih.gov/pubmed/28523334.

Holt RI, Teale JD, Jones JS, Quin JD, McGregor AM, Miell JP. Gene expression and serum levels of insulin-like growth factors (IGFs) and IGF-binding proteins in a case of non-islet cell tumour hypoglycaemia. Growth Horm IGF Res. 1998;8(6):447-54.

https://www.ncbi.nlm.nih.gov/pubmed/10985756. https://doi.org/10.1016/s1096-6374(98)80297-9.

Trivedi N, Mithal A, Sharma AK, et al. Non-islet cell tumour induced hypoglycaemia with acromegaloid facial and acral swelling. Clin Endocrinol (Oxf). 1995;42(4):433-5.

https://www.ncbi.nlm.nih.gov/pubmed/7750199. https://doi.org/10.1111/j.1365-2265.1995.tb02654.x.

Teale JD, Wark G. The effectiveness of different treatment options for non-islet cell tumour hypoglycaemia. Clin Endocrinol (Oxf). 2004;60(4):457-60.

https://www.ncbi.nlm.nih.gov/pubmed/15049960. https://doi.org/10.1111/j.1365-2265.2004.01989.x.

Bodnar TW, Acevedo MJ, Pietropaolo M. Management of non-islet-cell tumor hypoglycemia: A clinical review. J Clin Endocrinol Metab. 2014;99(3):713-22.

https://www.ncbi.nlm.nih.gov/pubmed/24423303. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5393479. https://doi.org/10.1210/jc.2013-3382.

Han G, Zhang Z, Shen X, et al. Doege-Potter syndrome: A review of the literature including a new case report. Medicine (Baltimore). 2017;96(27):e7417.

https://www.ncbi.nlm.nih.gov/pubmed/28682900. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5502173. https://doi.org/10.1097/MD.0000000000007417.

Park MS, Patel SR, Ludwig JA, et al. Activity of temozolomide and bevacizumab in the treatment of locally advanced, recurrent, and metastatic hemangiopericytoma and malignant solitary fibrous tumor. 2011;117(21):4939-47.

https://www.ncbi.nlm.nih.gov/pubmed/21480200. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3135685. NIHMSID: NIHMS277775. https://doi.org/10.1002/cncr.26098.

Lee ATJ, Benson C. Pazopanib as a choice for advanced solitary fibrous tumour. Lancet Oncol. 2020;21(3):332-3. https://www.ncbi.nlm.nih.gov/pubmed/32066545. https://doi.org/10.1016/S1470-2045(20)30018-8.

Martin-Broto J, Cruz J, Penel N, et al. Pazopanib for treatment of typical solitary fibrous tumours: A multicentre, single-arm, phase 2 trial. Lancet Oncol. 2020;21(3):456-66.

https://www.ncbi.nlm.nih.gov/pubmed/32066540. https://doi.org/10.1016/S1470-2045(19)30826-5.

Martin-Broto J, Stacchiotti S, Lopez-Pousa A, et.al. Pazopanib for treatment of advanced malignant and dedifferentiated solitary fibrous tumour: A multicentre, single-arm, phase 2 trial. Lancet Oncol. 2019;20(1):134-44. https://www.ncbi.nlm.nih.gov/pubmed/30578023. https://doi.org/10.1016/S1470-2045(18)30676-4.

Published
2021-05-12
How to Cite
Zarra, V. L., Demegillo, K. J., Uyking-Naranjo, M., & Domado, A. (2021). Doege-Potter Syndrome: A Presumptive Case of Metastatic Hemangiopericytoma with Persistent Hypoglycemia in a 27-Year-Old Male. Journal of the ASEAN Federation of Endocrine Societies, 36(1), 90-94. https://doi.org/10.15605/jafes.036.01.16
Section
Case Reports