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      A rare presentation of unilateral periureteral renal lymphangiomatosis

      case-report

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          Abstract

          Renal lymphangiomatosis is a rare developmental malformation of the perirenal lymphatic system. We report a unique case with unilateral massive periureteral involvement in addition to intrarenal and peripelvic lymphangiomatosis. Although this is a rare entity, it should be considered in patients with peripelvic or periureteric cystic lesions as it may affect appropriate management and follow-up. This case report reviews the imaging features of this entity and a comprehensive literature review and discussion about the entity will be provided.

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          Most cited references12

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          Lymphangioma of the retroperitoneum: CT and sonographic characteristic.

          Lance A. Davidson, D Hartman (1990)
          The authors retrospectively evaluated radiologic, clinical, and pathologic findings in 19 cases of lymphangioma of the retroperitoneum. The tumors were judged confined to one compartment of the retroperitoneum in 68% of the cases, whereas in 32% of cases the tumor involved more than one compartment. Abdominal radiography depicted the mass in all cases. Excretory urography demonstrated organ displacement without tumor invasion in all cases. Sonography showed multiloculated fluid in 61% of cases and a unicameral mass in 39% of cases. All but one of the multiloculated lymphangiomas had thick septa. Sonography also depicted the fluid as uncomplicated in 56% of cases. The remainder had debris that sometimes layered in the dependent portion of the cyst. Computed tomography (CT) showed a unicameral mass in 57% and a septated mass in 43% of cases. CT also showed thin, smooth walls in 79% and thick, irregular walls in 21% of cases. At CT the fluid contents were found to be homogeneous and of fluid attenuation in 64% and were complex in 36% of cases. The attenuation of fluid in one case was the same as that of retroperitoneal fat. In two cases the mass contained mural calcification. The most characteristic radiologic finding of lymphangioma of the retroperitoneum is an elongated tumor containing uncomplicated fluid with or without septa. Chyle and mural calcification are very uncommon in this location.
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            Cystic retroperitoneal lymphangiomas in adults.

            A Beham, Edward Berger, J Razmara … (1997)
            Lymphangioma (LA) is a rare benign tumour of the lymphatic tissue, most common in the neck and head, and clinically manifests itself mostly in childhood. Within this group, intra-abdominal and retroperitonal LA are the rarest tumours, especially when occurring in adults. We report four LAs localized in the retroperitoneum of patients aged between 28 and 72 years. One of these tumours infiltrated the transverse mesocolon and greater omentum, others were situated in the left retroperitoneum and retroperitoneally at the duodeno-jejunal flexure, and in the retrosplenal and retropancreatic area. Diagnosis was made by light microscopy supported by immunohistochemistry. In three cases the tumour could be removed by radical surgery and none of these patients had a recurrence (median follow-up time: 4 years). The tumour could not be removed completely from one patient with pre-operative chylascos. Six months after diagnosis of LA this patient died of cardiopulmonary failure due to progressive tumour chylascos. Isolation and ligation of the cystic LA's peduncle as well as ligation of lymph channels can prevent recurrences and chylascos.
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              Exacerbation of familial renal lymphangiomatosis during pregnancy.

              C. G. Ahlström, Alaina J. Grantham, Keith E Levine … (1988)
              Article 0 comments Cited 18 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): J Clin Imaging Sci
                Journal ID (iso-abbrev): J Clin Imaging Sci
                Journal ID (publisher-id): JCIS
                Title: Journal of Clinical Imaging Science
                Publisher: Scientific Scholar
                ISSN (Print): 2156-7514
                ISSN (Electronic): 2156-5597
                Publication date (Electronic): 08 December 2022
                Publication date Collection: 2022
                Volume: 12
                Electronic Location Identifier: 65
                Affiliations
                [1 ]Department of Urology, University of Virginia , Charlottesville, Virginia, United States
                [2 ]Department of Radiology , Mayo Clinic, Rochester, Minnesota, United States
                [3 ]Department of Radiology, University of Virginia , Charlottesville, Virginia, United States.
                Author notes
                [* ] Corresponding author: Sumit Isharwal, Department of Urology, University of Virginia, Charlottesville, Virginia, United States. si2uf@ 123456uvahealth.org
                Author information
                https://orcid.org/0000-0001-5660-2359
                Article
                Publisher ID: 10.25259/JCIS_125_2022
                DOI: 10.25259/JCIS_125_2022
                PMC ID: 9805599
                PubMed ID: 36601601
                SO-VID: e4696085-7b99-4994-9633-a2d06f858aef
                Copyright © © 2022 Published by Scientific Scholar on behalf of Journal of Clinical Imaging Science
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

                History
                Date received : 17 October 2022
                Date accepted : 21 November 2022
                Categories
                Subject: Genitourinary and Gynecologic Imaging
                Subject: Case Report

                ScienceOpen disciplines: Radiology & Imaging
                Keywords: renal,lymphangiomatosis,peripelvic cyst
                Data availability:
                ScienceOpen disciplines: Radiology & Imaging
                Keywords: renal, lymphangiomatosis, peripelvic cyst

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