Journal of Yeungnam Medical Science

Yeungnam University College of Medicine, Yeungnam University Institute Medical Science (영남대 의대 학술지 편집위원회)
권호 표지
DOI QR코드

DOI QR Code

Interleukin-6-producing paraganglioma as a rare cause of systemic inflammatory response syndrome: a case report

  • Yin Young Lee (Division of Endocrinology and Metabolism, Department of Internal Medicine, Daegu Veterans Hospital) ;
  • Seung Min Chung (Division of Endocrinology and Metabolism, Department of Internal Medicine, Yeungnam University College of Medicine)
  • Received : 2022.10.17
  • Accepted : 2023.01.15
  • Published : 2023.10.31
Download PDF
⟨ Previous Next ⟩

Abstract

Pheochromocytomas and paragangliomas (PPGLs) may secrete hormones or bioactive neuropeptides such as interleukin-6 (IL-6), which can mask the clinical manifestations of catecholamine hypersecretion. We report the case of a patient with delayed diagnosis of paraganglioma due to the development of IL-6-mediated systemic inflammatory response syndrome (SIRS). A 58-year-old woman presented with dyspnea and flank pain accompanied by SIRS and acute cardiac, kidney, and liver injuries. A left paravertebral mass was incidentally observed on abdominal computed tomography (CT). Biochemical tests revealed increased 24-hour urinary metanephrine (2.12 mg/day), plasma norepinephrine (1,588 pg/mL), plasma normetanephrine (2.27 nmol/L), and IL-6 (16.5 pg/mL) levels. 18F-fluorodeoxyglucose (FDG) positron emission tomography/CT showed increased uptake of FDG in the left paravertebral mass without metastases. The patient was finally diagnosed with functional paraganglioma crisis. The precipitating factor was unclear, but phendimetrazine tartrate, a norepinephrine-dopamine release drug that the patient regularly took, might have stimulated the paraganglioma. The patient's body temperature and blood pressure were well controlled after alpha-blocker administration, and the retroperitoneal mass was surgically resected successfully. After surgery, the patient's inflammatory, cardiac, renal, and hepatic biomarkers and catecholamine levels improved. In conclusion, our report emphasizes the importance of IL-6-producing PPGLs in the differential diagnosis of SIRS.

Keywords

References

  1. Whitelaw BC, Prague JK, Mustafa OG, Schulte KM, Hopkins PA, Gilbert JA, et al. Phaeochromocytoma [corrected] crisis. Clin Endocrinol (Oxf) 2014;80:13-22. https://doi.org/10.1111/cen.12324
  2. Tanaka T, Narazaki M, Kishimoto T. IL-6 in inflammation, immunity, and disease. Cold Spring Harb Perspect Biol 2014;6: a016295.
  3. Akira S, Hirano T, Taga T, Kishimoto T. Biology of multifunctional cytokines: IL 6 and related molecules (IL 1 and TNF). FASEB J 1990;4:2860-7. https://doi.org/10.1096/fasebj.4.11.2199284
  4. Carvalho Cunha N, Gomes L, Saraiva J, Paiva I. Interleukin-6 producing pheochromocytoma: a rare cause of systemic inflammatory response syndrome. Case Rep Endocrinol 2019;2019: 7906272.
  5. Meijs AC, Schroijen MA, Snel M, Corssmit EP. Interleukin-6 producing pheochromocytoma/paraganglioma: case series from a tertiary referral centre for pheochromocytomas and paragangliomas. J Endocrinol Invest 2021;44:2253-9. https://doi.org/10.1007/s40618-021-01532-5
  6. Angelousi A, Peppa M, Chrisoulidou A, Alexandraki K, Berthon A, Faucz FR, et al. Malignant pheochromocytomas/paragangliomas and ectopic hormonal secretion: a case series and review of the literature. Cancers (Basel) 2019;11:724.
  7. Cheng X, Zhang M, Xiao Y, Li H, Zhang Y, Ji Z. Interleukin-6-producing pheochromocytoma as a new reason for fever of unknown origin: a retrospective study. Endocr Pract 2018; 24:507-11. https://doi.org/10.4158/EP-2018-0048
  8. Minetto M, Dovio A, Ventura M, Cappia S, Daffara F, Terzolo M, et al. Interleukin-6 producing pheochromocytoma presenting with acute inflammatory syndrome. J Endocrinol Invest 2003;26:453-7.
  9. Kang JM, Lee WJ, Kim WB, Kim TY, Koh JM, Hong SJ, et al. Systemic inflammatory syndrome and hepatic inflammatory cell infiltration caused by an interleukin-6 producing pheochromocytoma. Endocr J 2005;52:193-8. https://doi.org/10.1507/endocrj.52.193
  10. Yarman S, Soyluk O, Altunoglu E, Tanakol R. Interleukin-6-producing pheochromocytoma presenting with fever of unknown origin. Clinics (Sao Paulo) 2011;66:1843-5. https://doi.org/10.1590/S1807-59322011001000028
  11. Martucci VL, Pacak K. Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment. Curr Probl Cancer 2014;38:7-41.
  12. Ku EJ, Kim KJ, Kim JH, Kim MK, Ahn CH, Lee KA, et al. Diagnosis for pheochromocytoma and paraganglioma: a joint position statement of the Korean Pheochromocytoma and Paraganglioma Task Force. Endocrinol Metab (Seoul) 2021;36:322-38. https://doi.org/10.3803/EnM.2020.908
  13. Nolting S, Bechmann N, Taieb D, Beuschlein F, Fassnacht M, Kroiss M, et al. Personalized management of pheochromocytoma and paraganglioma. Endocr Rev 2022;43:199-239. https://doi.org/10.1210/endrev/bnab019
  14. Gimenez-Roqueplo AP, Dahia PL, Robledo M. An update on the genetics of paraganglioma, pheochromocytoma, and associated hereditary syndromes. Horm Metab Res 2012;44:328-33. https://doi.org/10.1055/s-0031-1301302
  15. van Hulsteijn LT, Dekkers OM, Hes FJ, Smit JW, Corssmit EP. Risk of malignant paraganglioma in SDHB-mutation and SDHD-mutation carriers: a systematic review and meta-analysis. J Med Genet 2012;49:768-76. https://doi.org/10.1136/jmedgenet-2012-101192
  16. Benn DE, Gimenez-Roqueplo AP, Reilly JR, Bertherat J, Burgess J, Byth K, et al. Clinical presentation and penetrance of pheochromocytoma/paraganglioma syndromes. J Clin Endocrinol Metab 2006;91:827-36. https://doi.org/10.1210/jc.2005-1862
  17. Boyd J, Leung AA, Sadrzadeh HS, Pamporaki C, Pacak K, Deutschbein T, et al. A high rate of modestly elevated plasma normetanephrine in a population referred for suspected PPGL when measured in a seated position. Eur J Endocrinol 2019; 181:301-9. https://doi.org/10.1530/EJE-19-0176
  18. Shimizu C, Kubo M, Takano K, Takano A, Kijima H, Saji H, et al. Interleukin-6 (IL-6) producing phaeochromocytoma: direct IL-6 suppression by non-steroidal anti-inflammatory drugs. Clin Endocrinol (Oxf) 2001;54:405-10. https://doi.org/10.1046/j.1365-2265.2001.01082.x
  19. Fiebich BL, Lieb K, Hull M, Berger M, Bauer J. Effects of NSAIDs on IL-1 beta-induced IL-6 mRNA and protein synthesis in human astrocytoma cells. Neuroreport 1996;7:1209-13. https://doi.org/10.1097/00001756-199604260-00023
  20. Tung D, Ciallella J, Cheung PH, Saha S. Novel anti-inflammatory effects of doxazosin in rodent models of inflammation. Pharmacology 2013;91:29-34. https://doi.org/10.1159/000343762