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      Relevance of concurrent hypercalcemia in ureteric sarcoidosis complicated with bladder urothelial carcinoma: a case report

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          Abstract

          Background

          Sarcoidosis is a multisystem inflammatory disorder and can affect any organ; however, ureteric involvement is extremely rare with only four cases reported in the literature to date, all of which were diagnosed with surgical ureteral resection including a nephroureterectomy. This study reports the first case of ureteric sarcoidosis controlled with medical therapy where a differential diagnosis was performed based on the diagnostic clue of hypercalcemia. A definitive diagnosis was established without surgical resection of the ureter.

          Case presentation

          A 60-year-old man presented with anorexia and weight loss. Blood tests showed renal dysfunction and hypercalcemia. Computed tomography revealed left hydronephrosis associated with left lower ureteral wall thickening, which showed high signal intensity on diffusion-weighted magnetic resonance imaging. Similarly, we detected a bladder tumor on cystoscopy, and a 2-cm-long stenosis was revealed by retrograde ureterography; therefore, ureteral cancer was suspected. Meanwhile, considering the clinical implication of hypercalcemia, a differential diagnosis of sarcoidosis was established based on elevated levels of sarcoidosis markers. Fluorodeoxyglucose positron emission tomography showed fluorodeoxyglucose accumulation in the left lower ureter, skin, and muscles, suggestive of ureteric sarcoidosis with systemic sarcoid nodules. For a definitive diagnosis, transurethral resection of the bladder tumor and ureteroscopic biopsy were performed. Histopathological examination revealed ureteric sarcoidosis with bladder urothelial carcinoma. Following an oral administration of prednisolone, hypercalcemia instantly resolved, the renal function immediately improved, and the left ureteral lesion showed complete resolution with no recurrence.

          Conclusions

          In this case, the co-occurrence of ureteral lesion with bladder tumor evoked a diagnosis of ureteral cancer. However, considering a case of ureteral lesion complicated with hypercalcemia, assessment for differential diagnosis was performed based on the calcium metabolism and sarcoidosis markers. In cases of suspected ureteric sarcoidosis from the assessment, pathological evaluation with ureteroscopic biopsy should be performed to avoid nephroureterectomy.

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          Most cited references13

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          Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999.

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            Corticosteroid treatment in sarcoidosis.

            At present there is no curative treatment for sarcoidosis. Immunosuppressive and/or immunomodulatory drugs can, however, be used for controlling the disease. Corticosteroids remain the mainstay of therapy. They function by suppressing the pro-inflammatory cytokines and chemokines that are involved in cell-mediated immune responses and granuloma formation. Only in a select group of patients is it justifiable to use these drugs, after careful evaluation of the pros and cons. Importantly, disease severity, e.g. threatened organ functions, and not disease activity itself should be the deciding factor in this process. In the case of parenchymal involvement, there is substantial evidence that corticosteroids can improve respiratory symptoms and chest radiography and lung function parameters over 6-24 months. Other generally acknowledged (empirical) criteria for systemic treatment include neurological, cardiac and sight-threatening ocular involvement and hypercalcaemia. Remarkably, despite >50 yrs of use, there is no proof of long-term (survival) benefit from corticosteroid treatment. In addition, there are still no data regarding the optimal dose and duration of corticosteroid or other immunosuppressive therapy. One of the weightiest questions remaining is whether or not these drugs can prevent scarring in patients with a fibrogenic phenotype. As new agents, including infliximab and thalidomide, enter the stage and new diagnostic tools are now available, there is clearly a momentum to design multicentric randomised controlled trials with long enough follow-up (>5 yrs) to answer this pivotal question.
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              Typical and Atypical Manifestations of Intrathoracic Sarcoidosis

              Sarcoidosis is a systemic disorder of unknown cause that is characterized by the presence of noncaseating granulomas. The radiological findings associated with sarcoidosis have been well described. The findings include symmetric, bilateral hilar and paratracheal lymphadenopathy, with or without concomitant parenchymal abnormalities (multiple small nodules in a peribronchovascular distribution along with irregular thickening of the interstitium). However, in 25% to 30% of cases, the radiological findings are atypical and unfamiliar to most radiologists, which cause difficulty for making a correct diagnosis. Many atypical forms of intrathoracic sarcoidosis have been described sporadically. We have collected cases with unusual radiological findings associated with pulmonary sarcoidosis (unilateral or asymmetric lymphadenopathy, necrosis or cavitation, large opacity, ground glass opacity, an airway abnormality and pleural involvement) and describe the typical forms of the disorder as well. The understanding of a wide range of the radiological manifestations of sarcoidosis will be very helpful for making a proper diagnosis.
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                Author and article information

                Contributors
                yanoaki.uro@hotmail.com
                Journal
                BMC Nephrol
                BMC Nephrol
                BMC Nephrology
                BioMed Central (London )
                1471-2369
                22 June 2020
                22 June 2020
                2020
                : 21
                : 235
                Affiliations
                [1 ]GRID grid.410813.f, ISNI 0000 0004 1764 6940, Department of Urology, , Toranomon Hospital, ; 2–2–2 Toranomon, Minato-ku, Tokyo, 105–0001 Japan
                [2 ]GRID grid.410813.f, ISNI 0000 0004 1764 6940, Department of Nephrology, , Toranomon Hospital, ; Tokyo, Japan
                Author information
                http://orcid.org/0000-0001-8453-2474
                Article
                1893
                10.1186/s12882-020-01893-8
                7310021
                dc7659bd-6eb3-4fd2-b9be-601fe15aad42
                © The Author(s) 2020

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                History
                : 19 November 2019
                : 15 June 2020
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2020

                Nephrology
                ureteral cancer,ureteric sarcoidosis,bladder urothelial carcinoma,hypercalcemia
                Nephrology
                ureteral cancer, ureteric sarcoidosis, bladder urothelial carcinoma, hypercalcemia

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