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      Sclerosing mesenteritis: a benign cause of mesenteric mass lesions

      case-report

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          Abstract

          Sclerosing mesenteritis is a rare disease of the mesentery. Associations with surgery, trauma, autoimmunity and paraneoplastic syndrome have been suggested, but most of the cases remain idiopathic. Diagnosis is often incidental, based upon the finding of a single or multiple mesenteric lesions on abdominal CT and histopathological confirmation. Optimal treatment is still controversial, but most of the cases reported have a favourable prognosis. We present a case of a 54-year-old male with long-standing abdominal pain and nausea, whose CT revealed the presence of a large mesenteric mass. A biopsy was performed, revealing benign chronic inflammation, fibrosis and IgG4-positive plasmocytes consistent with sclerosing mesenteritis. Clinical remission was achieved with corticosteroids and follow-up CTs at six and twelve months documented stability of the lesion. Furthermore, we review the current literature on the diagnosis and treatment options for this rare disease.

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          Most cited references10

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          Sclerosing mesenteritis, mesenteric panniculitis and mesenteric lipodystrophy: a single entity?

          We reviewed 84 cases coded as mesenteric lipodystrophy (ML), mesenteric panniculitis (MP), or retractile mesenteritis and sclerosing mesenteritis (SM), grading fibrosis, inflammation, and fat necrosis, and evaluating clinical subgroups. There was no gender or racial predominance. Patient age range was 23-87 years (average 60). Patients most often presented with abdominal pain or a palpable mass. A history of trauma or surgery was present in four of 84 patients. The most common site of involvement was the small bowel mesentery as a single mass (58 of 84) with an average size of 10 cm, multiple masses (15 of 84), or diffuse mesenteric thickening (11 of 84). All patients had some degree of fibrosis, chronic inflammation, and fat necrosis. Although a few patients showed a sufficient prominence of fibrosis, inflammation, or fat necrosis to permit a separation into SM, MP, or ML, respectively, in most patients these three components were too mixed for a clear separation. The clinical, demographic, and gross features did not help in defining these three entities. Contributors diagnosed 12 as sarcoma. Of 39 patients followed beyond the postoperative period, none died of these lesions. We conclude that SM, MP, and ML appear to represent histologic variants of one clinical entity, and in most cases "sclerosing mesenteritis" is the most appropriate diagnostic term.
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            Sclerosing mesenteritis: clinical features, treatment, and outcome in ninety-two patients.

            Sclerosing mesenteritis is a rare non-neoplastic disease that affects the small bowel mesentery with chronic fibrosing inflammation. There are few data on the natural history and therapeutic options for this condition. We performed a retrospective and prospective study to describe the clinical characteristics, therapy, and outcome of all cases of sclerosing mesenteritis diagnosed at the Mayo Clinic, Rochester, from 1982-2005. Ninety-two cases were identified; 70% were male, with a median age of 65 years (interquartile range, 55-72). Common presenting symptoms included abdominal pain in 70%, diarrhea in 25%, and weight loss in 23%. Treatment included medical therapy alone in 26%, surgery alone in 13%, surgery followed by medical therapy in 9%, and 52% received no treatment. Ten percent responded to surgery alone, 20% responded to additional medical treatment after surgery, and 38% responded to medical therapy alone. Tamoxifen in combination with prednisone was used in 20 patients, and 60% improved. Non-tamoxifen-based regimens were used in 12 patients, and 8% improved. Eighteen deaths were noted during the study period, and 17% were attributed to complications of sclerosing mesenteritis or its treatment. Although a relatively benign condition, sclerosing mesenteritis can have a prolonged debilitating course with a fatal outcome. Our results suggest that symptomatic patients might benefit from medical therapy, particularly tamoxifen and prednisone combination treatment. Long-term follow-up is needed to substantiate these results.
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              CT evaluation of mesenteric panniculitis: prevalence and associated diseases.

              This study was undertaken to assess the prevalence of mesenteric panniculitis on CT and to describe its appearance and associated diseases. A total of 7620 consecutive abdominal CT examinations were prospectively evaluated for features common to mesenteric panniculitis such as a well-delineated inhomogeneous hyperattenuated fatty mass at the mesenteric root, envelopment of mesenteric vessels, and no evidence of invasion of the adjacent small-bowel loops that may be displaced. CT findings of mesenteric panniculitis were seen in 49 patients (0.6%). We found a female predominance. Mesenteric panniculitis coexisted with malignancy in 34 patients and with benign disorders in 11 patients. In the remaining four patients, mesenteric panniculitis, verified on histology, was considered to be responsible for the patients' clinical manifestations; no other abnormality was identified. Soft-tissue nodules (n = 39) and a fatty halo surrounding vessels and nodules (n = 42) were observed in most patients. Follow-up abdominal CT examinations in 29 of the 49 patients showed changes in only one patient. CT findings of mesenteric panniculitis may be seen in patients undergoing abdominal CT for various symptoms.
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                Author and article information

                Journal
                Pan Afr Med J
                Pan Afr Med J
                PAMJ
                The Pan African Medical Journal
                The African Field Epidemiology Network
                1937-8688
                28 July 2017
                2017
                : 27
                : 228
                Affiliations
                [1 ]Surgery Service, Hospital Center Lisboa Central, Lisboa, Portugal
                Author notes
                [& ]Corresponding author: Diogo Carrola Gomes, Surgery Service, Hospital Center Lisboa Central, Lisboa, Portugal
                Article
                PAMJ-27-228
                10.11604/pamj.2017.27.228.11542
                5622839
                1a00d82a-c612-4207-9c62-93c232713ad5
                © Diogo Carrola Gomes et al.

                The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 31 December 2016
                : 01 May 2017
                Categories
                Case Report

                Medicine
                sclerosing mesenteritis,mesenteric panniculitis,mesenteric mass
                Medicine
                sclerosing mesenteritis, mesenteric panniculitis, mesenteric mass

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