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      Contrast-enhanced ultrasonography combined with superb microvascular imaging for preoperative diagnosis of sporadic intra-abdominal desmoid-type fibromatosis: A case report

      case-report
      , MD a , b , , MD, PhD c , * , , MD, PhD c , , MD, PhD d , , MD, PhD e , , MD, PhD f , , MD c , d , , MD c , , MD c , , MD, PhD c , , MD, PhD c , g , , MD, PhD h , , MD, PhD i , , MD, PhD f , , MD, PhD i , , MD, PhD h , , MD, PhD d , , MD, PhD d , , MD, PhD d , , MD, PhD b , , MD, PhD i , , MD, PhD j
      Radiology Case Reports
      Elsevier
      Intra-abdominal desmoid-type fibromatosis, Diagnosis, Transabdominal ultrasonography, Contrast-enhanced ultrasonography, Superb microvascular imaging

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          Abstract

          We herein report a case of sporadic intra-abdominal desmoid-type fibromatosis in which contrast-enhanced ultrasonography (US) combined with superb microvascular imaging (SMI) was useful for preoperative diagnosis. 18-Fluorodeoxyglucose positron emission tomography performed for systematic screening for lung cancer revealed an abnormal accumulation in the abdominal cavity. Transabdominal US showed a tumor with a mixture of hypoechoic and hyperechoic areas. Contrast-enhanced US combined with SMI revealed dendritic blood flow signals and no abnormal vascular network within the tumor. Macroscopic examination of the resected specimen revealed a white tumor with relatively clear boundaries. Microscopic examination revealed spindle cells with poor atypia proliferating in bundles with collagenous stromal cells. Immunohistochemistry showed nuclear localization of beta-catenin within the tumor cells. From these findings, we finally diagnosed intra-abdominal desmoid-type fibromatosis. Contrast-enhanced US combined with SMI is useful for diagnosing intra-abdominal desmoid-type fibromatosis.

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          Most cited references18

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          Prognostic factors influencing progression-free survival determined from a series of sporadic desmoid tumors: a wait-and-see policy according to tumor presentation.

          Desmoid tumors are mesenchymal fibroblastic/myofibroblastic proliferations with locoregional aggressiveness and high ability to recur after initial treatment. We present the results of the largest series of sporadic desmoid tumors ever published to determine the prognostic factors of these rare tumors. Four hundred twenty-six patients with a desmoid tumor at diagnosis were included, and the following parameters were studied: age, sex, delay between first symptoms and diagnosis, tumor size, tumor site, previous history of surgery or trauma in the area of the primary tumor, surgical margins, and context of abdominal wall desmoids in women of child-bearing age during or shortly after pregnancy. We performed univariate and multivariate analysis for progression-free survival (PFS). In univariate analysis, age, tumor size, tumor site, and surgical margins (R2 v R0/R1) had a significant impact on PFS. PFS curves were not significantly different for microscopic assessment of surgical resection quality (R0 v R1). In multivariate analysis, age, tumor size, and tumor site had independent values. Three prognostic groups for PFS were defined on the basis of the number of independent unfavorable prognostic factors (0 or 1, 2, and 3). This study clearly demonstrates that there are different prognostic subgroups of desmoid tumors that could benefit from different therapeutic strategies, including a wait-and-see policy.
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            Management of desmoid tumours: A nationwide survey of labelled reference centre networks in France.

            The optimal management of rare tumours (i.e. from accurate diagnosis to management in reference centres) is a public health challenge. In 2009, the French National Cancer Institute (INCa) identified and financially supported the two expert networks for pathological and clinical diagnosis and management of soft tissue tumours.
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              A to Z of desmoid tumors.

              The purpose of this article is to illustrate the common locations of desmoid tumors (deep fibromatosis), complications of intra- and extraabdominal desmoids, and treatment-related changes in their imaging appearance. Desmoids are locally aggressive fibrous tumors with a tendency to recur. Desmoids can be intraabdominal, in the abdominal wall, or extraabdominal. Complications, such as compression or invasion of adjacent structures, and abscess formation can occur. Treatment options include observation, local treatment (surgery, radiotherapy), or systemic therapy (conventional chemotherapy, molecular targeted agents).
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                Author and article information

                Contributors
                Journal
                Radiol Case Rep
                Radiol Case Rep
                Radiology Case Reports
                Elsevier
                1930-0433
                05 July 2024
                September 2024
                05 July 2024
                : 19
                : 9
                : 3988-3993
                Affiliations
                [a ]Center for Doctors’ Career Development, Kawasaki Medical School Hospital, Kurashiki, Japan
                [b ]Department of Pathology, Kawasaki Medical School Hospital, Kurashiki, Japan
                [c ]Division of Endoscopy and Ultrasonography, Department of Clinical Pathology and Laboratory Medicine, Kawasaki Medical School General Medical Center, Okayama, Japan
                [d ]Department of General Surgery, Kawasaki Medical School General Medical Center, Okayama, Japan
                [e ]Department of Diagnostic and Therapeutic Radiology, Kawasaki Medical School General Medical Center, Okayama, Japan
                [f ]Department of Pathology, Kawasaki Medical School General Medical Center, Okayama, Japan
                [g ]Department of Gastroenterology and Hepatology, HITO Medical Center, Shikokuchuou, Japan
                [h ]Department of Health Care Medicine, Kawasaki Medical School General Medical Center, Okayama, Japan
                [i ]Department of General Internal Medicine 2, Kawasaki Medical School General Medical Center, Okayama, Japan
                [j ]Division of Endoscopy and Ultrasonography, Department of Clinical Pathology and Laboratory Medicine, Kawasaki Medical School Hospital, Kurashiki, Japan
                Author notes
                [* ]Corresponding author. minorufu@ 123456med.kawasaki-m.ac.jp
                Article
                S1930-0433(24)00518-1
                10.1016/j.radcr.2024.06.026
                11269867
                39055107
                4dcfe00b-be6b-48d1-aeec-9341badac21e
                © 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.

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

                History
                : 9 April 2024
                : 28 May 2024
                : 8 June 2024
                Categories
                Case Report

                intra-abdominal desmoid-type fibromatosis,diagnosis,transabdominal ultrasonography,contrast-enhanced ultrasonography,superb microvascular imaging

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