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      Pheochromocytoma crisis presenting with hypotension, hemoptysis, and abnormal liver function : A case report

      case-report

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          Abstract

          Rationale:

          Pheochromocytomas are rare catecholamine-secreting tumors arising from adrenomedullary chromaffin cells, usually causing hypertension, palpitation and headache. However, pheochromocytoma crisis, on the contrary, might present with hypotension, multiple organ dysfunction or even mimicking other diseases, leaving physician with diagnostic difficulties. In this study, we present a case featured hypotension, shock and multiple organ dysfunction syndrome on admission, which nearly lead us to miss the diagnosis of pheochromocytoma.

          Patient concerns:

          A 14-year-old female student presented with cough, hemoptysis and dyspnea for one week was reported.

          Diagnoses:

          The laboratory test showed significantly increase in plasma norepinephrine and 24-hour urine norepinephrine, the enhanced CT of bilateral adrenal gland showed two round-like masses (left: 4 × 5 × 3 cm; right: 6 × 4 × 3 cm) with soft tissue density in each adrenal gland. The post-surgical pathology confirmed the diagnosis of pheochromocytoma.

          Interventions:

          The resection of bilateral adrenal tumors was conducted after the preoperative medical treatment of phenoxybenzamine for two weeks.

          Outcomes:

          The patient underwent follow-up for a year and a half and showed no signs of recurrence.

          Lessons:

          The diagnosis and treatment process of the patient in this study indicates us that when we meet a patient with hypotension and multiple organ dysfunctions in a relatively short time, the suspicion of pheochromocytoma should not be missed.

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          Most cited references12

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          Management of high blood pressure in children and adolescents: recommendations of the European Society of Hypertension.

          Hypertension in children and adolescents has gained ground in cardiovascular medicine, thanks to the progress made in several areas of pathophysiological and clinical research. These guidelines represent a consensus among specialists involved in the detection and control of high blood pressure in children and adolescents. The guidelines synthesize a considerable amount of scientific data and clinical experience and represent best clinical wisdom upon which physicians, nurses and families should base their decisions. They call attention to the burden of hypertension in children and adolescents, and its contribution to the current epidemic of cardiovascular disease, these guidelines should encourage public policy makers, to develop a global effort to improve identification and treatment of high blood pressure among children and adolescents.
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            Cardiovascular manifestations of phaeochromocytoma.

            Clinical expression of phaeochromocytoma may involve numerous cardiovascular manifestations, but usually presents as sustained or paroxysmal hypertension associated with other signs and symptoms of catecholamine excess. Most of the life-threatening cardiovascular manifestations of phaeochromocytoma, such as hypertensive emergencies, result from a rapid and massive release of catecholamines from the tumour. More rarely, patients with phaeochromocytoma present with low blood pressure or even shock that may then precede multisystem crisis. Sinus tachycardia, with palpitations as the presenting symptom, is the most prevalent abnormality of cardiac rhythm in phaeochromocytoma, but tumours can also be associated with more serious ventricular arrhythmias or conduction disturbances. Reversible dilated or hypertrophic cardiomyopathy are well established cardiac manifestations of phaeochromocytoma, with more recent attention to an increasing number of cases with Takotsubo cardiomyopathy. This review provides an update on the cause, clinical presentation and treatment of the cardiovascular manifestations of phaeochromocytoma. As the cardiovascular complications of phaeochromocytoma can be life-threatening, all patients who present with manifestations that even remotely suggest excessive catecholamine secretion should be screened for the disease.
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              Pheochromocytoma crisis is not a surgical emergency.

              Pheochromocytoma crisis is a feared and potentially lethal complication of pheochromocytoma. We sought to determine the best treatment strategy for pheochromocytoma crisis patients and hypothesized that emergency resection is not indicated. Retrospective cohort study (1993-2011); literature review (1944-2011). Tertiary referral center. There were 137 pheochromocytoma patients from our center and 97 pheochromocytoma crisis patients who underwent adrenalectomy from the literature. Medical management of pheochromocytoma crisis; adrenalectomy. Perioperative complications, conversion, and mortality. In our database, 25 patients (18%) presented with crisis. After medical stabilization and α-blockade, 15 patients were discharged and readmitted for elective surgery and 10 patients were operated on urgently during the same hospitalization. None underwent emergency surgery. Postoperatively, patients who underwent elective surgery had shorter hospital stays (1.7 vs 5.7 d, P = 0.001) and fewer postoperative complications (1 of 15 [7%] vs 5 of 10 [50%], P = 0.045) and were less often admitted to the intensive care unit (1 of 15 [7%] vs 5 of 10 [50%], P = 0.045) in comparison with urgently operated patients. There was no mortality. Review of the literature (n = 97) showed that crisis patients who underwent elective or urgent surgery vs emergency surgery had less intraoperative (13 of 31 [42%] vs 20 of 25 [80%], P < 0.001) and postoperative complications (15 of 45 [33%] vs 15 of 21 [71%], P = 0.047) and a lower mortality (0 of 64 vs 6 of 33 [18%], P = 0.002). Management of patients presenting with pheochromocytoma crisis should include initial stabilization of the acute crisis followed by sufficient α-blockade before surgery. Emergency resection of pheochromocytoma is associated with high surgical morbidity and mortality.
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                Author and article information

                Journal
                Medicine (Baltimore)
                Medicine (Baltimore)
                MEDI
                Medicine
                Wolters Kluwer Health
                0025-7974
                1536-5964
                June 2018
                22 June 2018
                : 97
                : 25
                : e11054
                Affiliations
                [a ]Division of Endocrinology and Metabolism
                [b ]Sichuan University, West China Hospital, Chengdu, Sichuan, People's Republic of China.
                Author notes
                []Correspondence: Yuwei Zhang, Division of Endocrinology and Metabolism, Sichuan University, West China Hospital, No. 37 Guo Xue Xiang, Chengdu, Sichuan, 610041, People's Republic of China ( e-mail: doczhangyuwei@ 123456sina.com ).
                Article
                MD-D-18-01400 11054
                10.1097/MD.0000000000011054
                6023654
                29923996
                fddc91c4-a34b-4749-a3d8-d1dee263513f
                Copyright © 2018 the Author(s). Published by Wolters Kluwer Health, Inc.

                This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (CCBY-NC), where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc/4.0

                History
                : 4 March 2018
                : 15 May 2018
                Categories
                4300
                Research Article
                Clinical Case Report
                Custom metadata
                TRUE

                case report,emergency,hypotension,liver dysfunction,pheochromocytoma crisis

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