J Yeungnam Med Sci.  2023 Oct;40(4):435-441. 10.12701/jyms.2022.00766.

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

• Lee YY
• Chung SM

• KMID: 2547370
• DOI: http://doi.org/10.12701/jyms.2022.00766

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.

Keyword

Catecholamines; Interleukin-6; Paraganglioma; Pheochromocytoma; Systemic inflammatory response syndrome

Figure

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  Fig. 1. (A) Chest X-ray performed at admission shows bilateral lung infiltrations. (B) Follow-up chest X-ray performed 24-hour after admission shows prominent resolution of the lung infiltrations.

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  Fig. 2. (A) Abdominal CT scan demonstrates the presence of a 3.8 cm-long round mass originating from the left paravertebral area. Non-enhanced attenuation of the mass reaches more than 20 Hounsfield units (arrow). (B) In the arterial phase, the tumor is heterogeneously enhanced with partial cystic changes. (C) 18F-fluorodeoxyglucose PET-CT scan demonstrates increased uptake (arrow) in the mass originating in the left paravertebral area and no metastases. CT, computed tomography; PET, positron emission tomography.

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  Fig. 3. Histologic and Immunohistochemical findings. (A) The tumor cells have abundant basophilic cytoplasm. A prominent cell-nesting pattern (zellballen) is noted (hematoxylin and eosin stain, ×100). (B) Tumor cells are diffusely positive for chromogranin A (immunohistochemical stain, ×100).

Reference

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