Paraganglioma Not Only Secrete Catecholamines but Also Inammatory Cytokines: A Case Report

Background: Paraganglioma not only can secrete catecholamines with vasoconstriction activity resulting in hypertension and metabolic disorders,but also cytokines which can induce inammatory response syndrome. Case presentation: We report a 19-year-old girl with a paraganglioma that presented with fever and thrombocytosis, leukocytosis, and elevated high sensitivity C-reactive protein. After paraganglioma’s resection, the little girl had no fever and the platelet, white blood cell and high sensitivity C-reactive protein recover to nomal level. Conclusion: We propose in the future we should check cytokine in patients with paroxysmal hypertension complicated with inammatory response syndrome and thrombocytosis, and think of that it may be caused by pheochromocytoma or paraganglioma.


Background
Pheochromocytoma is a group of neuroendocrine tumors originating from the adrenal medulla and sympathetic nervous system. Most pheochromocytoma is located in the adrenal gland. However, a small part of pheochromocytoma is located in the autonomic nervous system outside the adrenal gland, which is called ectopic pheochromocytoma or paraganglioma in clinical. As we all know,paraganglioma can secrete catecholamines with vasoconstriction activity, resulting in hypertension and metabolic disorders.

Case Presentation
A 19-year-old girl (162 cm, 40 kg) came to our hospital due to suffering from fever, intermittent headache and unclear vision for some days. She had a two-month history of hypertension with the highest blood pressure of 200/110 mmHg. The biochemical parameters were white blood cell count 14.56 × computed tomography(CT) showed that an enhanced mass was found at the right anterior lower adrenal gland, based on which the diagnosis of paraganglioma was suggested for this patient (Fig. 1). A question came to our mind:was this girl's fever and thrombocytosis due to paraganglioma or hematologic diseases? In order to make clear whether the patient was complicated with hematologic disease, We made a bone marrow puncture, which indicated normal bone marrow .Thus, It made sure that the patient's clinical manifestation was all caused by paraganglioma.
The major difference between this patient and the other paraganglioma patient is the long-term intermittent fever and severe thrombocytosis. These characteristics of paraganglioma were rarely reported previously. This patient needs surgical treatment, and we did preoperative preparation and treatment for the young girl. To abatement the fever,antibiotic,non-steroidal anti-in ammatory drugs(NSAIDs) and dexamethasone were provided for the patient, however, These were not effective. She also orally took hydroxycarbamide and apheresis platelets to decrease platelets and prevent thrombosis, and α-adrenergic receptor blocker with calcium channel blocker to decrease blood pressure. 7 days later, the patient had the resection of right side paraganglioma with abdomen under general anesthesia. It took 40 minutes from tumor isolation to tumor vessel ligation. During this period the highest blood pressure was 185 / 118 mmHg, the heart rate was 120 times / min, and phentolamine, nitroglycerin, and magnesium sulfate were added up to 120 mg, 0.8 mg and 2.5 g, respectively. After tumor resection, the blood pressure decreased, and norepinephrine was pumped at 0.15-0.25 µg/(kg·min) to maintain blood pressure. A 5 × 4 cm tumor was resected nally.The operation was successful.After the operation,the little girl had no fever and the platelet recover to nomal level. Changes in perioperative related laboratory parameters were showed in table I.So we nally con rmed that her clinical manifestations were only caused by paraganglioma. The patient was discharged at postoperative day 11.

Discussion And Conclusions
This case rarely presents the paraganglioma not only secrete catecholamines but also in ammatory cytokines induced in ammatory response syndrome.
This patient had been diagnosed as paragangliomas before operation. Her clinical manifestation was especially prominent. Besides hypertension, dizziness, palpitation, sweating and unclear vision, the patient had recurrent fever and thrombocytosis. This case is very rare. Preoperative antibiotic,NSAIDs and corticosteroids were not effective in antipyretic, and the platelets were progressively elevated. Surgical resection is an effective way to treat this disease. [4] After surgery, the fever, platelets, high sensitive C-reactive protein (hsCRP) and coagulation parameters all decreased. Thus, it con rmed that the fever and thrombocytosis were caused by paraganglioma, and that our diagnosis was correct. The pathological report also con rmed the effect of the treatment.
Pheochromocytoma or paraganglioma associated in ammatory response syndrome is more often caused by IL-6 in the reports. [5][6][7][8][9] IL-6 is physiologically expressed in the adrenal cortex, but not in the adrenal medulla. [10] IL-6 is a multifunctional cytokine that plays a key role in the development of immune and in ammatory. IL-6 is produced not only by lymphoid cells and monocytes, but also by endothelial cells, broblasts, adipose cells, myocardial and skeletal cells. [11] It's a pity that the cytokines of this patient was not checked and it could not be determined which cytokine had increased.
Through this case and literature consulting, we know that pheochromocytoma or paraganglioma not only secrete catecholamines but also in ammatory cytokines.Therefore, in the future we should check cytokine in patients with paroxysmal hypertension complicated with in ammatory response syndrome and thrombocytosis, and think of that it may be caused by pheochromocytoma or paraganglioma.

Declarations
Ethics approval and consent to participate A consent for participation was obtained from the patient. A copy of the written informed consent is available for review by the editor of this journal.

Consent for publication
A consent for publication was obtained from the patient. A copy of the written informed consent is available for review by the editor of this journal.

Availability of data and materials
The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.

Competing interests
The authors declare that they have no competing interests. A 5 × 4 cm an enhanced mass was found at the right anterior lower adrenal gland