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      A retroperitoneal pseudomyxoma of the appendix: A case report

      case-report

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          Abstract

          Introduction

          Retroperitoneal pseudomyxoma (RP) is a rare condition characterized by mucinous tumor growth within the retroperitoneal space, commonly originating from the appendix or other abdominal organs. RP presents with non-specific symptoms such as abdominal pain, bloating, and weight loss, making accurate diagnosis challenging. Due to its rarity and symptom overlap with other conditions, RP requires careful management by a multidisciplinary healthcare team.

          Case presentation

          An 82-year-old female with a three-month history of abdominal pain and an abdominal mass underwent diagnostic imaging, revealing a retroperitoneal cystic mass. Exploratory laparotomy identified gelatinous masses originating from the retrocaecal appendix, which were resected. Histopathological examination confirmed a diagnosis of retroperitoneal pseudomyxoma with mucinous appendicular low-grade cystadenoma as the primary source. The patient had an uneventful postoperative course and remained disease-free at a 12-month follow-up.

          Discussion

          RP's exact cause is unknown but often originates from the appendix, leading to mucinous fluid accumulation in the retroperitoneal cavity. This condition presents with varied symptoms, including abdominal pain and distension, mimicking other abdominal diseases. Diagnosis involves clinical, radiological, and pathological assessments, with imaging showing characteristic features. Treatment comprises surgical resection, potentially combined with HIPEC, aiming for complete cytoreduction. Prognosis depends on factors like tumor grade, extent, and cytoreduction completeness.

          Conclusion

          Retroperitoneal pseudomyxoma is a rare condition with challenging diagnosis due to its non-specific symptoms. Early recognition, surgical resection, and appropriate adjuvant therapies like HIPEC or chemotherapy can improve outcomes in patients with this uncommon disease.

          Highlights

          • Retroperitoneal pseudomyxoma (RP) often originates from the appendix but can also arise from the ovaries or colon, characterized by excessive mucinous fluid accumulation.

          • RP's clinical presentation is diverse and can mimic other abdominal diseases, with symptoms like abdominal pain, distension, and bowel changes.

          • Diagnosis involves clinical, radiological (CT and MRI), and pathological evaluations, with characteristic findings in imaging.

          • Multidisciplinary management of RP includes surgical resection, often combined with heated intraperitoneal chemotherapy, to improve outcomes.

          • RP prognosis varies based on factors like tumor extent, cytoreduction completeness, and histological subtype, with high recurrence rates and better outcomes for low-grade tumors with complete cytoreduction. Regular follow-up is essential.

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          Most cited references9

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          The SCARE 2023 guideline: updating consensus Surgical CAse REport (SCARE) guidelines

          The Surgical CAse REport (SCARE) guidelines were first published in 2016 as a tool for surgeons to document and report their surgical cases in a standardised and comprehensive manner. However, with advances in technology and changes in the healthcare landscape, it is important to revise and update these guidelines to ensure they remain relevant and valuable for surgeons. Materials and methods: The updated guidelines were produced through a Delphi consensus exercise. Members of the SCARE 2020 guidelines Delphi group, editorial board members, and peer reviewers were invited to participate. Potential contributors were contacted by e-mail. An online survey was completed to indicate their agreement with the proposed changes to the guideline items. Results: A total of 54 participants were invited to participate and 44 (81.5%) completed the survey. There was a high degree of agreement among reviewers, with 36 items (83.7%) meeting the threshold for inclusion. Conclusion: Through a completed Delphi consensus exercise we present the SCARE 2023 guidelines. This will provide surgeons with a comprehensive and up-to-date tool for documenting and reporting their surgical cases while highlighting the importance of patient-centred care.
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            Retroperitoneal cystic masses: CT, clinical, and pathologic findings and literature review.

            Cystic lesions of the retroperitoneum can be classified as either neoplastic or nonneoplastic. Neoplastic lesions include cystic lymphangioma, mucinous cystadenoma, cystic teratoma, cystic mesothelioma, müllerian cyst, epidermoid cyst, tailgut cyst, bronchogenic cyst, cystic change in solid neoplasms, pseudomyxoma retroperitonei, and perianal mucinous carcinoma. Nonneoplastic lesions include pancreatic pseudocyst, nonpancreatic pseudocyst, lymphocele, urinoma, and hematoma. Because the clinical implications of and therapeutic strategies for retroperitoneal cystic masses vary depending on the cause, the ability to noninvasively differentiate between masses is important. Although there is substantial overlap of computed tomographic (CT) findings in various retroperitoneal cysts, some CT features, along with clinical characteristics, may suggest a specific diagnosis. CT may provide important information regarding lesion location, size, and shape; the presence and thickness of a wall; the presence of septa, calcifications, or fat; and involvement of adjacent structures. The most important clinical parameters include patient gender, age, symptoms, and clinical history. Familiarity with the CT and clinical features of various retroperitoneal cystic masses facilitates accurate diagnosis and treatment. Copyright RSNA, 2004
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              Review: Pathology and its clinical relevance of mucinous appendiceal neoplasms and pseudomyxoma peritonei

              Until recently, many classifications existed for the terminology and histopathologic classification of appendiceal mucinous neoplasms, mucinous appendiceal adenocarcinomas, and pseudomyxoma peritonei (PMP). A major accomplishment was achieved by consensus-based histopathologic classifications on behalf of the Peritoneal Surface Oncology Group International regarding mucinous appendiceal tumours and PMP. As different classifications were used over the years and also owing to the rare nature of these tumors, many clinicians are not familiar with the terminology and the impact on patient management. Hence, an overview concerning mucinous appendiceal neoplasms, mucinous appendiceal adenocarcinomas, and PMP is provided to serve as an introduction into the basic morphology of these tumors with tentative recommendations for management.
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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
                08 March 2024
                April 2024
                08 March 2024
                : 117
                : 109492
                Affiliations
                [a ]Department of General Surgery, Hopital la Rabta, Tunis, Tunisia
                [b ]Department of Gastroenterology, Hopital la Rabta, Tunis, Tunisia
                Author notes
                Article
                S2210-2612(24)00273-6 109492
                10.1016/j.ijscr.2024.109492
                10940759
                38461584
                02700af9-bd69-4002-b800-53c25d766d8c
                © 2024 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd.

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

                History
                : 2 February 2024
                : 1 March 2024
                : 6 March 2024
                Categories
                Case Report

                retroperitoneum,pseudomyxoma,appendix,case report
                retroperitoneum, pseudomyxoma, appendix, case report

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