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      Polyserositis: An Extremely Rare Life-Threatening Manifestation of Pheochromocytoma

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          Abstract

          The pericardium is an uncommon site for manifestation of pheochromocytoma. Herein, the case of a 57-year-old man with cardiac tamponade is presented. Pericardiocentesis was performed, and the vital signs were stabilized afterwards. An abdominal computed tomography (CT) scan illustrated a nonhomogeneous right adrenal mass suspicious of pheochromocytoma, planned for right adrenalectomy. He recovered well after surgery, and his subsequent follow-ups did not reveal any complications.

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          Initial clinical presentation and spectrum of pheochromocytoma: a study of 94 cases from a single center

          Background With the increasing access to imaging more pheochromocytomas are diagnosed in the workup of adrenal incidentalomas. This may have changed the occurrence of the classic presentation with hypertension and the classic triad (headaches, sweating and palpitation). Methods We reviewed 94 consecutive cases of pheochromocytomas. Two cases of ectopic ACTH-syndrome were subsequently excluded. Results Of the 92 cases included 64% had presented as an incidentaloma, 32% as a suspected pheochromocytoma and 4% had been screened because of previously diagnosed MEN2A. Those screened were youngest while those with incidentalomas were oldest. The females were more common in the incidentaloma and the screening groups, and males in the suspected pheochromocytoma group. Measurements of noradrenaline/normetanephrine levels were highest in the suspected pheocromocytoma group and lowest in the screening group. Hypertension was present in 63% of the incidentalomas, 79% of suspected pheochromocytomas and in none of the screening group. Paroxysmal symptoms were present in almost all with suspected pheochromocytoma while only in half of the other groups. The suspected pheocromocytoma group had most symptoms and the screening group least. The classic triad was present in 14% of the incidentalomas, in 28% of the suspected and in none of the screening group, while no symptoms at all was present in 12%, 0% and 25%, respectively. Pheochromocytoma crisis occurred in 5%. There was a positive correlation between tumor size vs hormone levels, and catecholamine levels vs blood pressure. Conclusion Clinicians need to be aware of the modern presentation of pheochromocytomas since early identification can be life-saving.
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            Interleukin-6-producing pheochromocytoma presenting with fever of unknown origin

            INTRODUCTION Pheochromocytoma usually presents with typical signs and symptoms, such as headache, sweating, and palpitations due to excessive catecholamine release. Few publications have reported that these tumors are capable of secreting a variety of bioactive neuropeptides and hormones other than catecholamines, resulting in unusual clinical manifestations.1-4 Interleukin-6 (IL-6) is a multifunctional molecule that plays an important role in hematopoiesis and immune and inflammatory responses. IL-6 stimulates the activation and differentiation of B and T lymphocytes, induces fever and regulates the acute-phase protein synthesis of proteins such as C-reactive protein (CRP) and fibrinogen.5 IL-6 is mainly produced by lymphocytes and monocytes but can also be produced by other cell types including fibroblasts, keratinocytes, mesangial cells, endothelial cells, and adrenal cortical cells.6,7 We report an 18-year-old normotensive, female patient who had an interleukin-6-producing pheochromocytoma and presented with fever of unknown origin associated with elevated levels of inflammatory markers. CASE REPORT An 18-year-old girl sought medical help for her ongoing malaise and fever following an upper respiratory tract infection. She had experienced weight loss of 3 kg, but did not have typical paroxysmal symptoms. Her physical examination showed no pathological findings except a temperature of 38.7°C. Her blood pressure was measured to be 90/60 mmHg. Laboratory data revealed both an elevated erythrocyte sedimentation rate (130 mm/h) and an elevated C-reactive protein level (98 mg/dl, normal; 0–5). Specific and nonspecific blood and urine cultures were negative for microbiological pathogens. Viral serological markers were negative as well. The total blood count revealed hypochromic and microcytic anemia (Hb: 8.6 g/dl, Hct: %25, MCV: 75 fl, RDW: 18.9%, MCHC: 33.3 g/dl) with marked thrombocytosis (PLT: 901000/µl) and slight neutrophilic leukocytosis (WBC: 12600/µl, neutrophils: 9700/µl), which was confirmed later with both peripheral blood and bone marrow smears. Her liver enzymes were within the normal range. A fever between 37.5°C and 41°C persisted. Contrast-enhanced magnetic resonance imaging of the upper abdomen indicated a smooth-contoured right adrenal mass with a diameter of 5.5 cm, which was hypointense and heterogeneous on T1-weighted images, whereas it was hyperintense and heterogeneous on T2-weighted images, with nodular contrast enhancement toward the center of the mass (Figure 1). This patient was referred to our department for further evaluation. The morning fasting serum cortisol level, the plasma aldosterone level and the renin activity of the patient in the prone and upright positions were within the normal ranges. The urinary normetanephrine level was elevated to a level of 3612 µg/24 hours (normal; 105–354). These findings supported the diagnosis of pheochromocytoma. Almost immediate cessation of the fever and a gradual normalization of the clinical inflammatory marker levels were achieved after four weeks of adrenergic blockade with doxazosin. At admission, the serum interleukin-6 level was measured to be 12.5 pg/mL (normal <3.0 pg/mL), which then became 9.9 pg/mL in the second month of the adrenergic blockade. The IL-6 level decreased to 6.6 pg/mL after complete resection of the tumor, which proved histopathologically to be a pheochromocytoma. The serum interleukin-6 level returned to normal a few weeks after the operation (Figure 2). DISCUSSION The unusual presentation of our pheochromocytoma case, with normal blood pressure, fever, and a marked inflammatory response, indicates the complexity of the clinical manifestations of this rare tumor. There have been other reports of adrenal tumors presenting with fever, hypertension, anemia, thrombocytosis, megakaryocytosis, and hyperfibrinogenemia associated with elevated levels of IL-6.1,4, Cytokine production by the tumor could occasionally play a crucial role in the emergence of unusual symptoms such as fever and weight loss. IL-6 is clearly associated with the inflammatory response mediated by the differentiation of B-lymphocytes into immunoglobulin-producing plasmacytes and potently stimulates the production of acute inflammatory proteins while decreasing serum albumin levels.8,9 IL-6 is also the cause of megakaryocytosis.12,13 IL-6 over-production can be either ascribed directly to the tumor or indirectly accounted for by tumoral production as a consequence of the high levels of circulating norepinephrine. The relief of symptoms and the normalization of the inflammatory marker levels following the decrease in the level of IL-6 after resection of the tumor strongly support the role of IL- 6 in this unusual presentation of our case. Although there are some data demonstrating the effectiveness of non-steroidal anti-inflammatory drugs in reducing IL-6 levels and inflammatory symptoms in patients with pheochromocytomas,1,4,12 our patient achieved fever remission through the use of alpha-adrenergic receptor blockers, corroborating the results of a previous study.14 Our patient had no hypertension despite high catecholamine levels. This situation could be explained by the increased nitric oxide synthesis due to IL–6 activity, which might have led to vasodilation.15,16 CONCLUSION Pheochromocytoma may be a cause of paraneoplastic syndrome with pyrexia in addition to inducing a marked increase in the levels of inflammatory markers. IL-6 appears to be the primary mediator. This rare tumor has to be considered in the vast differential diagnosis of fever of unknown origin.
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              Elevated Inflammation Markers in Pheochromocytoma Compared to Other Forms of Hypertension

              Objective: To investigate the effect of long-term catecholamine excess in pheochromocytoma on leukocyte and platelet count and on proteins of acute-phase response. Methods: Fifteen subjects with pheochromocytoma, 16 with primary aldosteronism, 18 with essential hypertension and 17 healthy controls were studied. Sixteen subjects with pheochromocytoma were investigated after tumor removal. Leukocyte, neutrophil and platelet count, as well as C-reactive protein were measured in all subjects, while fibrinogen, α 1 -antitrypsin, α 2 -macroglobulin, orosomucoid, transferrin and prealbumin were only measured in subjects with pheochromocytoma, primary aldosteronism and essential hypertension. Results: Subjects with pheochromocytoma showed significantly higher leukocyte [7.5 ± 0.9 10 9 /l, p 9 /l) and healthy controls (5 ± 0.9 10 9 /l), p = 0.04 vs. essential hypertension (6.3 ± 1.6 10 9 /l)], neutrophil (p 2 -macroglobulin: p = 0.009 vs. primary aldosteronism]. No significant differences were found in plasma levels of α 1 -antitrypsin, transferrin and prealbumin. Tumor removal led to a significant decrease in leukocyte (p = 0.004), neutrophil (p = 0.007) and platelet count (p = 0.003) and also to a significant decrease in acute-phase proteins (C-reactive protein: p = 0.03, fibrinogen: p = 0.008, α 1 -antitrypsin: p = 0.003, orosomucoid: p = 0.04). Conclusions: Chronic catecholamine excess in pheochromocytoma is accompanied by an increase in inflammation markers which was reversed by the tumor removal.
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                Author and article information

                Journal
                Case Reports in Endocrinology
                Case Reports in Endocrinology
                Hindawi Limited
                2090-6501
                2090-651X
                July 06 2020
                July 06 2020
                : 2020
                : 1-3
                Affiliations
                [1 ]Isfahan Endocrine and Metabolism Research Center, Isfahan University of Medical Sciences, Isfahan, Iran
                [2 ]Isfahan University of Medical Sciences, Isfahan, Iran
                [3 ]University Hospitals of Leicester NHS Foundation Trust, LE1 5WW Leicester, UK
                [4 ]Medical Library and Information Sciences Department, Health Information Technology Research Center, School of Management and Medical Information Sciences, Isfahan University of Medical Sciences, Isfahan, Iran
                [5 ]Heart Failure Research Center, Cardiovascular Research Institute, Isfahan University of Medical Sciences, Isfahan, Iran
                Article
                10.1155/2020/8814699
                eb786b6e-3818-48cf-bc9c-dc5a14b21273
                © 2020

                http://creativecommons.org/licenses/by/4.0/

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