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      The ectopic vesical pheochromocytoma a diagnostic and therapeutic challenge case report and literature review

      case-report

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          Highlights

          • Pheochromocytoma is a tumor developed at the expense of chromaffin tissues.

          • Extra-adrenal localization of pheochromocytoma is rare.

          • The main problem with these tumors is to affirm their benignity or malignancy.

          • Ectopic pheochromocytomas have a malignant development once in two.

          • Ectopic pheochromocytoma presents a diagnostic and therapeutic dilemma.

          Abstract

          Introduction

          Extra-adrenal localization of pheochromocytoma is rare. Its clinical revelation is paroxysmal but maybe in the form of permanent hypertension resistant to treatment. The main problem with these tumors is to affirm their benignity or malignancy, ectopic pheochromocytomas have a malignant development once in two.

          Case presentation

          We report the observation of a 39-year-old woman hypertensive on Amlodipine. She presents for sweating, headache, and palpation. The dosage of urinary metabolites revealed a marked increase in metanephrine and normetanephrine. Imagery had shown, at the expense of the left lateral wall of the bladder, a budding tissue formation with irregular contours which was significantly enhanced after injection of PDC. The diagnosis of an ectopic pheochromocytoma was accepted. The patient underwent a partial cystectomy, the postoperative consequences of which were unremarkable.

          Discussion

          Threatening secretory syndromes are rare medical situations that require urgent management. Pheochromocytoma can be seen at any age from childhood to old age. The treatment of pheochromocytoma is surgical, once the stages of positive and topographic diagnosis have been carried out, the patient must be immediately entrusted to a team of trained surgeons and anesthetists. The prognosis remains unpredictable, hence the interest in prolonged monitoring.

          Conclusion

          Pheochromocytoma is a tumor developed at the expense of chromaffin tissues, extra-adrenal localizations exist, but remain rare. It is a pathology that presents a diagnostic and therapeutic dilemma in which patients require regular and prolonged postoperative monitoring to detect a possible metastasis or recurrence.

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          Most cited references20

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          The SCARE 2018 statement: Updating consensus Surgical CAse REport (SCARE) guidelines

          The SCARE Guidelines were published in 2016 to provide a structure for reporting surgical case reports. Since their publication, SCARE guidelines have been widely endorsed by authors, journal editors, and reviewers, and have helped to improve reporting transparency of case reports across a range of surgical specialties. In order to encourage further progress in reporting quality, the SCARE guidelines must themselves be kept up to date. We completed a Delphi consensus exercise to update the SCARE guidelines.
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            Phaeochromocytoma.

            Phaeochromocytomas are rare neuroendocrine tumours with a highly variable clinical presentation but most commonly presenting with episodes of headaches, sweating, palpitations, and hypertension. The serious and potentially lethal cardiovascular complications of these tumours are due to the potent effects of secreted catecholamines. Biochemical testing for phaeochromocytoma is indicated not only in symptomatic patients, but also in patients with adrenal incidentalomas or identified genetic predispositions (eg, multiple endocrine neoplasia type 2, von Hippel-Lindau syndrome, neurofibromatosis type 1, and mutations of the succinate dehydrogenase genes). Imaging techniques such as CT or MRI and functional ligands such as (123)I-MIBG are used to localise biochemically proven tumours. After the use of appropriate preoperative treatment to block the effects of secreted catecholamines, laparoscopic tumour removal is the preferred procedure. If removal of phaeochromocytoma is timely, prognosis is excellent. However, prognosis is poor in patients with metastases, which especially occur in patients with large, extra-adrenal tumours.
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              Germ-line mutations in nonsyndromic pheochromocytoma.

              The group of susceptibility genes for pheochromocytoma that included the proto-oncogene RET (associated with multiple endocrine neoplasia type 2 [MEN-2]) and the tumor-suppressor gene VHL (associated with von Hippel-Lindau disease) now also encompasses the newly identified genes for succinate dehydrogenase subunit D (SDHD) and succinate dehydrogenase subunit B (SDHB), which predispose carriers to pheochromocytomas and glomus tumors. We used molecular tools to classify a large cohort of patients with pheochromocytoma with respect to the presence or absence of mutations of one of these four genes and to investigate the relevance of genetic analyses to clinical practice. Peripheral blood from unrelated, consenting registry patients with pheochromocytoma was tested for mutations of RET, VHL, SDHD, and SDHB. Clinical data at first presentation and follow-up were evaluated. Among 271 patients who presented with nonsyndromic pheochromocytoma and without a family history of the disease, 66 (24 percent) were found to have mutations (mean age, 25 years; 32 men and 34 women). Of these 66, 30 had mutations of VHL, 13 of RET, 11 of SDHD, and 12 of SDHB. Younger age, multifocal tumors, and extraadrenal tumors were significantly associated with the presence of a mutation. However, among the 66 patients who were positive for mutations, only 21 had multifocal pheochromocytoma. Twenty-three (35 percent) presented after the age of 30 years, and 17 (8 percent) after the age of 40. Sixty-one (92 percent) of the patients with mutations were identified solely by molecular testing of VHL, RET, SDHD, and SDHB; these patients had no associated signs and symptoms at presentation. Almost one fourth of patients with apparently sporadic pheochromocytoma may be carriers of mutations; routine analysis for mutations of RET, VHL, SDHD, and SDHB is indicated to identify pheochromocytoma-associated syndromes that would otherwise be missed.
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                Author and article information

                Contributors
                Journal
                Int J Surg Case Rep
                Int J Surg Case Rep
                International Journal of Surgery Case Reports
                Elsevier
                2210-2612
                02 December 2020
                2020
                02 December 2020
                : 77
                : 857-861
                Affiliations
                [a ]University Hospital Center Ibn Rochd Casablanca, Morocco
                [b ]Faculté de médicine et de pharmacie Casablanca, Morocco
                Author notes
                [* ]Corresponding author at: Hay El amal, Bloc 05, N 24, Beni Mellal, Code postal: 23040, Morocco. jissam.iatros@ 123456gmail.com
                Article
                S2210-2612(20)31161-5
                10.1016/j.ijscr.2020.11.132
                7726245
                33395912
                a6005ea3-e3c2-43a2-b89d-7c809fb256b0
                © 2020 The Author(s)

                This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

                History
                : 21 November 2020
                : 25 November 2020
                : 25 November 2020
                Categories
                Case Report

                ectopic pheochromocytoma,bladder pheochromocytoma,methoxylated derivative,partial cystectomy

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