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      Malignant pheochromocytoma with multiple vertebral metastases causing acute incomplete paralysis during pregnancy : Literature review with one case report

      case-report

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          Abstract

          Rationale:

          We present a rare case of malignant pheochromocytoma with thoracic metastases during pregnancy that presented with symptoms of myelopathy and was treated with circumferential decompression, stabilization, and radiation. The management of this unique case is not well documented. The clinical manifestations, imaging results, pathological characteristics, treatment and prognosis of the case were analyzed.

          Patient concerns:

          A 26-year-old pregnant woman with a history of paroxysmal hypertension during the second trimester presented with lower extremity weakness, numbness, urinary incontinence, and back pain. Imaging studies revealed a right adrenal pheochromocytoma, multiple metastases at T8, T11, T12, and the pelvis girdle causing significant multilevel cord compression and significant osteolytic lesions at T11 and T12.

          Diagnoses:

          We believe this is the first reported case of metastatic pheochromocytoma of the thoracic spine presenting with symptoms of myelopathy during pregnancy.

          Interventions:

          A healthy neonate was delivered by emergency caesarean section at 34 weeks. Subsequently, the patient underwent a circumferential spinal cord decompression and a stabilization procedure.

          Outcomes:

          The patient's neurological deficits improved significantly after the surgery, and the postoperative period was uneventful at the 6-month follow-up visit.

          Lessons:

          This article emphasizes that metastatic pheochromocytoma of the spine, although rare, should be part of the differential when a patient presents with elevated blood pressure, weakness, and urinary incontinence.

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          Most cited references37

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          Recent advances in genetics, diagnosis, localization, and treatment of pheochromocytoma.

          Pheochromocytoma is a rare but important tumor of chromaffin cells that is frequently considered in the evaluation of hypertension, arrhythmias, or panic disorder and in the follow-up of patients with particular genetic diseases. This report provides an update about the genetics, neurochemical diagnosis, localization by imaging, and surgical management of pheochromocytoma. Specific mutations of the RET proto-oncogene cause familial predisposition to pheochromocytoma in multiple endocrine neoplasia type II, and mutations in the von Hippel-Lindau tumor suppressor gene cause familial disposition to pheochromocytoma in von Hippel-Lindau disease. Recent findings demonstrating extraordinarily high sensitivity of plasma levels of metanephrines for detecting pheochromocytoma have led to an algorithm for clinical diagnostic steps. Nuclear imaging approaches, such as(123) I-metaiodobenzylguanidine scintigraphy and 6-[(18) F]fluorodopamine positron emission tomography, enhance both diagnosis and localization of the tumor, as described in an algorithm for patients with positive biochemical test results. Since pheochromocytoma is often benign, surgical resection by laparoscopic adrenalectomy can be curative. Areas requiring further work include determining appropriate follow-up of patients with familial pheochromocytoma, elucidating the bases for phenotypic differences, improving both specificity and sensitivity of biochemical tests, optimizing cost-effectiveness of diagnostic imaging, and testing the risk for tumor recurrence after partial adrenalectomy.
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            Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979.

            For the 30 years from 1950 through 1979, the population of Rochester, Minnesota, averaged 45,800, resulting in almost 1.4 million person-years of observation. During that time, 11 cases of pheochromocytoma were diagnosed in this population; thus, the average annual incidence rate was 0.8 per 100,000 person-years. With the inclusion of two additional diagnosed familial cases, the incidence rate would be 0.95 per 100,000 person-years. Five of the 11 cases were diagnosed initially at autopsy.
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              Risk Factors for Hemodynamic Instability during Surgery for Pheochromocytoma.

              Surgery on pheochromocytoma carries a risk for hemodynamic (HD) instability. The aim of this study was to identify preoperative risk factors for intraoperative HD instability. In addition, efficacy of pretreatment with the alpha-adrenergic receptor (alpha) antagonists phenoxybenzamine (PXB) and doxazosin (DOX) was compared with respect to reduction of intraoperative HD instability. Seventy-three patients operated in Erasmus Medical Center between 1995 and 2007 were included. Parameters studied were catecholamine type and concentration, tumor diameter, mean arterial pressure (MAP) before and after (MAP(alpha)) pretreatment with alpha-antagonist, postural fall in blood pressure (BP) after pretreatment, type of alpha-blockade, type of operation, and presence of a familial polytumor syndrome. HD instability was assessed by measuring the number and time period MAP was below 60 mm Hg and systolic BP (SBP) was above 160 mm Hg. A correlation was found between the intraoperative time periods of SBP above 160 mm Hg and plasma norepinephrine levels (r = 0.23; P < 0.05), tumor diameter (r = 0.36; P < 0.01), and postural BP fall (r = 0.30; P < 0.05). MAP at presentation and after alpha-blockade above 100 mm Hg (BP, 130/85 mm Hg) was related to more and longer episodes with a SBP above 160 mm Hg (P < 0.01). Type of operation or alpha-blockade and presence of a familial polytumor syndrome were not related to intraoperative HD instability. Postoperative MAP was lower in the DOX group than in the PXB group (P < 0.05). Risk factors for HD instability during surgery for pheochromocytoma include a high plasma NE concentration, larger tumor size, more profound postural BP fall after alpha-blockade, and a MAP above 100 mm Hg (130/85 mm Hg). Efficacy for preventing HD instability was identical for PXB and DOX.
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                Author and article information

                Journal
                Medicine (Baltimore)
                Medicine (Baltimore)
                MEDI
                Medicine
                Wolters Kluwer Health
                0025-7974
                1536-5964
                November 2017
                03 November 2017
                : 96
                : 44
                : e8535
                Affiliations
                [a ]Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences
                [b ]Department of Endocrinology, Key Laboratory of Endocrinology, National Health and Family Planning Commission, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College
                [c ]Department of Neurosurgery, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China
                [d ]Department of Neurosurgery, Wayne State University School of Medicine, Detroit, MI.
                Author notes
                []Correspondence: Yong Liu, Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, No. 1 Shuaifuyuan Wangfujing, Beijing 100730, China (e-mail: liuyong_pumch@ 123456126.com ).
                Article
                MD-D-17-02832 08535
                10.1097/MD.0000000000008535
                5682838
                29095319
                33551e06-0836-4b23-8b5b-038d87fb0462
                Copyright © 2017 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
                : 5 May 2017
                : 10 October 2017
                : 12 October 2017
                Categories
                5700
                Research Article
                Clinical Case Report
                Custom metadata
                TRUE

                acute incomplete paralysis,metastatic spinal pheochromocytoma,pregnancy,radiation therapy,surgical treatment

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